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or severe substance use disorder, or no substance use disorder, can be diagnosed. Behav- ioral disturbance cannot be coded but should still be indicated in writing. ICD-10-CM ICD-9-CMWith use disorder, mildWith use disorder, moderate or severeWithout use disorder Alcohol (major neurocognitive disorder), nonamnestic- confabulatory type291.2 NA F10.27 F10.97 Alcohol (major neurocognitive disorder), amnestic- confabulatory type291.1 NA F10.26 F10.96 Alcohol (mild neurocognitive disorder)291.89 NA F10.288 F10.988 Inhalant (major neurocognitive disorder)292.82 F18.17 F18.27 F18.97 Inhalant (mild neurocognitive disorder)292.89 F18.188 F18.288 F18.988 Sedative, hypnotic, or anxiolytic (major neurocognitive disorder)292.82 NA F13.27 F13.97 Sedative, hypnotic, or anxiolytic (mild neurocognitive disorder)292.89 NA F13.288 F13.988 Other (or unknown) substance (major neurocognitive disorder)292.82 F19.17 F19.27 F19.97 Other (or unknown) substance (mild neurocognitive disorder)292.89 F19.188 F19.288 F19.988
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Substance/Medication-Induced Major or Mild Neurocognitive Disorder 629 Specify if: Persistent: Neurocognitive impairment continues to be significant after an extended period of abstinence. Recording Procedures ICD-9-CM. The name of the substance/medication-i nduced neurocogniti ve disorder be- gins with the specific substance/medication (e.g ., alcohol) that is presumed to be causing the neurocognitive symptoms. Th e diagnostic code is selected from the table included in the criteria set, which is based on the drug clas s. For substances that do not fit into any of the classes, the code for “other substance” should be used; and in cases in which a substance is judged to be an etiological factor but the sp ecific class of substance is unknown, the cat- egory “unknown substance” should be used. The name of the disorder (i.e., [specific su bstance]-induced major neurocognitive dis- order or [specific substance]-induced mild neurocognitive disorder) is followed by the type in the case of alcohol (i.e., nonamnestic-confabulatory type, amnestic-confabulatory type), followed by specification of duration (i .e., persistent). Unlike the recording procedures for ICD-10-CM, which combine the substanc e/medication-induced disorder and sub- stance use disorder into a single code, for ICD-9-CM a separate diagnostic code is given for the substance use disorder. For example, in th e case of persistent amnestic-confabulatory symptoms in a man with a severe alcohol use disorder, the diagnosis is 291.1 alcohol- induced major neurocognitive d isorder, amnestic-confabulatory type, persistent. An addi-
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tional diagnosis of 303.90 severe alcohol use diso rder is also given. If the substance/medi- cation-induced neurocognitive disorder occurs without a comorbid substance use disorder (e.g., after a sporadic heavy use of inhalants) , no accompanying substance use disorder is noted (e.g., 292.82 inhalant-induced mild neurocognitive disorder). ICD-10-CM. The name of the substance/medicati on-induced neurocognitive disorder begins with the specific substance (e.g., alcoho l) that is presumed to be causing the neuro- cognitive symptoms. The diagnostic code is sele cted from the table included in the criteria set, which is based on the drug class and pres ence or absence of a comorbid substance use disorder. For substances that do not fit into any of the cla sses, the code for “other sub- stance” should be used; and in cases in which a substance is judged to be an etiological fac- tor but the specific class of substance is unknown, the category “unknown substance” should be used. When recording the name of the disorder, the comorbid substa nce use disorder (if any) is listed first, followed by the word “with,” followe d by the name of the disorder (i.e., [specific substance]-induced major neurocognitive diso rder or [specific substance]-induced mild neurocognitive disorder), follow ed by the type in the case of alcohol (i.e., nonamnestic-con- fabulatory type, amnestic-confabulatory type), followed by specification of duration (i.e., persistent). For example, in the case of pers istent amnestic-confabulatory symptoms in a
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man with a severe alcohol use disorder, the di agnosis is F10.26 severe alcohol use disorder with alcohol-induced major neur ocognitive disorder, amnestic -confabulatory type, persis- tent. A separate diagnosis of the comorbid severe alcohol use disorder is not given. If the substance-induced neurocognitive disorder occurs without a comorbid substance use dis- order (e.g., after a sporadic heavy use of inha lants), no accompanying substance use disor- der is noted (e.g., F18.98 8 inhalant-induced mild neurocognitive disorder). Diagnostic Features Substance/medication-induced major or mild NCD is characterized by neurocognitive impairments that persist beyond the usual du ration of intoxication and acute withdrawal (Criterion B). Initially, these manifestations can reflect slow recovery of brain functions
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impairments that persist beyond the usual du ration of intoxication and acute withdrawal (Criterion B). Initially, these manifestations can reflect slow recovery of brain functions from a period of prolonged su bstance use, and improvements in neurocognitive as well as
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630 Neurocognitive Disorders brain imaging indicators may be seen over ma ny months. If the diso rder continues for an extended period, persistent should be specified. The give n substance and its use must be known to be capable of causing the observed impairments (Criterion C). While nonspecific decrements in a range of cognitive abilities can occur with nearly any substance of abuse and a variety of medications, some patterns occur more frequently with selected drug classes. For example, NCD due to sedative, hypno tic, or anxiolytic drugs (e.g., benzodiaz- epines, barbiturates) ma y show greater disturbances in memory than in other cognitive functions. NCD induced by alcohol frequently manifests with a co mbination of impair- ments in executive-function and memory and learning domains. Th e temporal course of the substance-induced NCD must be consistent with that of use of the given substance (Criterion D). In alcohol-induced amnestic confabulatory (Korsakoff ’s) NCD, the features include prominent amnesia (severe difficulty learning new information with rapid forget- ting) and a tendency to confabulate. These ma nifestations may co-occur with signs of thi- amine encephalopathy (Wernicke’s encephalopathy) with associated features such as nystagmus and ataxia. Ophthalmoplegia of Wernicke’s encephalopathy is typically charac- terized by a lateral gaze paralysis. In addition to or independent of the mo re common neurocognitive symptoms related to methamphetamine use (e.g., difficulties wi th learning and memory; executive func- tion), methamphetamine use can also be associat ed with evidence of vascular injury (e.g.,
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focal weakness, unilateral incoordination, as ymmetrical reflexes). The most common neu- rocognitive profile approximates that seen in vascular NCD. Associated Features Supporting Diagnosis Intermediate-duration NC D induced by drugs with central nervous system depressant effects may manifest with added symptoms of increased irritability, anxiety, sleep disturbance, and dysphoria. Intermediate-duration NCD induced by stimulant drugs may manifest with re- bound depression, hypersomnia, and apathy. In severe forms of substance/medication- induced major NCD (e.g., associ ated with long-term alcohol use), there may be prominent neuromotor features, such as incoordination, ataxia, and motor slowing. There may also be loss of emotional control, including aggres sive or inappropriat e affect, or apathy. Prevalence The prevalence of these conditions is not know n. Prevalence figures for substance abuse are available, and substance/medica tion-induced major or mild NCDs are more likely in those who are older, have longer use, and have other risk factors such as nutritional deficits. For alcohol abuse, the rate of mild NCD of intermediate duration is approximately 30%– 40% in the first 2 months of abstinence. Mild NCD may persist, particularly in those who do not achieve stable abstinence until after age 50 years. Major NCD is rare and may result from concomitant nutritional deficits, as in al cohol-induced amnestic confabulatory NCD. For individuals quitting cocaine, metham phetamine, opioids, phencyclidine, and sed- ative, hypnotics, or anxiolytics, substance/medication-induced mild NCD of intermediate duration may occur in one-third or more, and there is some evidence that these substances
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duration may occur in one-third or more, and there is some evidence that these substances may also be associated with persistent mild NCD. Major NCD associated with these sub- stances is rare, if it occurs at all. In the case of methamph etamine, cerebr ovascular disease can also occur, resulting in diffuse or focal brain injury that can be of mild or major neu- rocognitive levels. Solvent expo sure has been linked to both major and mild NCD of both intermediate and persistent duration. The presence of NCD induced by cannabis and various hallucinogens is controversial. With cannabis, intoxication is accompanied by various neur ocognitive disturbances, but these tend to clear with abstinence.
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Substance/Medication-Induced Major or Mild Neurocognitive Disorder 631 Development and Course Substance use disorders tend to commence du ring adolescence and peak in the 20s and 30s. Although longer history of severe substa nce use disorder is associated with greater likelihood of NCD, the relationships are not straightforward, with substantial and even complete recovery of neurocognitive func tions being common among individuals who achieve stable abstinence prior to age 50 ye ars. Substance/medication-induced major or mild NCD is most likely to become persistent in individuals who continue abuse of sub- stances past age 50 years, presumably becaus e of a combination of lessened neural plas- ticity and beginnings of other age-related br ain changes. Earlier commencement of abuse, particularly of alcohol, may lead to defects in later neural development (e.g., later stages of maturation of frontal circuitries), which may have effects on social cognition as well as other neurocognitive abilities. For alcohol-induced NCD, there may be an additive effect of aging and alcohol-induced brain injury. Risk and Prognostic Factors Risk factors for substance/medication-induced NCDs include older age, longer use, and persistent use past age 50 years. In additi on, for alcohol-induced NCD, long-term nutri- tional deficiencies, liver disease, vascular r isk factors, and cardiovascular and cerebrovas- cular disease may contribute to risk. Diagnostic Markers Magnetic resonance imaging (MRI) of individuals with chronic alcohol abuse frequently reveals cortical thinning, white matter loss, and enlargement of sulci and ventricles. While neuroimaging abnormalities are more common in those with NCDs, it is possible to ob-
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serve NCDs without neuroimaging abnormalitie s, and vice versa. Specialized techniques (e.g., diffusion tensor imaging) may reveal damage to specific white matter tracts. Mag- netic resonance spectroscopy may reveal reduction in N-acetylaspartate, and increase in markers of inflammation (e.g., myoinositol) or white matter injury (e.g., choline). Many of these brain imaging changes and neurocogniti ve manifestations reverse following suc- cessful abstinence. In individuals with meth amphetamine use disorder, MRI may also re- veal hyperintensities suggestive of microhem orrhages or larger areas of infarction. Functional Consequences of Substance/Medication- Induced Major or Mild Neurocognitive Disorder The functional consequences of substance/ medication-induced mild NCD are sometimes augmented by reduced cognitive efficiency an d difficulty concentrating beyond that seen in many other NCDs. In addition, at both major and mild levels, substance/medication- induced NCDs may have associated motor syndro mes that increase the level of functional impairment. Differential Diagnosis Individuals with substance use disorders, substance intoxica tion, and substance withdrawal are at increased risk for othe r conditions that may independently, or through a compounding effect, result in neurocognitive disturbance. These include hi story of traumatic brain injury and infections that can accompany substance use disorder (e.g., HIV, hepatitis C virus, syph- ilis). Therefore, presence of substance/medi cation-induced major or mild NCD should be differentiated from NCDs arising outside the co ntext of substance use, intoxication, and with- drawal, including these accompanying cond itions (e.g., traumatic brain injury).
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632 Neurocognitive Disorders Comorbidity Substance use disorders, substance intoxicati on, and substance withdrawal are highly co- morbid with other mental disorders. Comorb id posttraumatic stre ss disorder, psychotic disorders, depressive and bi polar disorders, and neurodev elopmental disorders can con- tribute to neurocognitive impairment in substance users. Traumatic brain injury occurs more frequently with substance use, complicati ng efforts to determine the etiology of NCD in such cases. Severe, long-term alcohol use disorder can be associated with major organ system disease, including cerebrovascular disease and cirrhosis. Amphetamine-induced NCD may be accompanied by major or mild vascular NCD, also secondary to amphet- amine use. Major or Mild Neurocognitive Disorder Due to HIV Infection Diagnostic Criteria A. The criteria are met for major or mild neurocognitive disorder. B. There is documented infection with human immunodeficiency virus (HIV). C. The neurocognitive disorder is not bette r explained by non-HIV conditions, including secondary brain diseases such as progre ssive multifocal leukoencephalopathy or cryptococcal meningitis. D. The neurocognitive disorder is not attribut able to another medical condition and is not better explained by a mental disorder. Coding note: For major neurocognitive disorder due to HIV infection, with behavioral dis- turbance, code first 042 (B20) HIV infection, followed by 294.11 (F02.81) major neurocog- nitive disorder due to HIV infection, with behavioral disturbance. For major neurocognitive disorder due to HIV infection, without behavioral disturbance, code first 042 (B20) HIV in-
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fection, followed by 294.10 (F02.80) major neurocognitive disorder due to HIV infection, without behavioral disturbance. For mild neurocognitive disorder due to HIV infection, code 331.83 (G31.84). (Note: Do not use the additional code for HIV infection. Behavioral disturbance cannot be coded but should still be indicated in writing.) Diagnostic Features HIV disease is caused by infection with hu man immunodeficiency virus type-1 (HIV-1), which is acquired through exposure to bodily fluids of an infected person through injection drug use, unprotected sexual contact, or acci dental or iatrogenic exposure (e.g., contami- nated blood supply, needle puncture injury to medical personnel). HIV infects several types of cells, most particularly immune cells. Over time, the infection can cause severe depletion of “T-helper” (CD4) lymphocytes, resulting in severe immunocompromise, often leading to opportunistic infections and neoplasms. This advanced form of HIV infection is termed acquired immune deficiency syndrome (AIDS). Diagnosis of HIV is confirmed by established laboratory methods such as enzyme-linked immunosorbent assay for HIV antibody with Western blot confirmation and/or polymera se chain reaction–based assays for HIV. Some individuals with HIV infection deve lop an NCD, which generally shows a “sub- cortical pattern” with prominently impaired executive function, slowing of processing speed, problems with more demanding attentio nal tasks, and difficulty in learning new information, but fewer problems with recall of learned information. In major NCD, slow- ing may be prominent. Language difficulties , such as aphasia, are uncommon, although reductions in fluency may be observed. HIV pathogenic proc esses can affect any part of
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the brain; therefore, other patterns are possible.
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Major or Mild Neurocognitive Disorder Due to HIV Infection 633 Associated Features Supporting Diagnosis Major or mild NCD due to HIV infection is usually more prevalent in individuals with prior episodes of severe immunosuppression, high viral loads in the cerebrospinal fluid, and indicators of advanced HIV disease such as anemia and hypoalbuminemia. Individ- uals with advanced NCD may experience prom inent neuromotor features such as severe incoordination, ataxia, and moto r slowing. There may be loss of emotional control, includ- ing aggressive or inappropriate affect or apathy. Prevalence Depending on stage of HIV disease, approx imately one-third to over one-half of HIV- infected individuals have at least mild neuroc ognitive disturbance, but some of these dis- turbances may not meet the full criteria for mild NCD. An estimated 25% of individuals with HIV will have signs and symptoms that meet criteria for mild NCD, and in fewer than 5% would criteria for major NCD be met. Development and Course An NCD due to HIV infection can resolve, improve, slowly worsen, or have a fluctuating course. Rapid progression to profound neur ocognitive impairment is uncommon in the context of currently available combination antiviral treatment; co nsequently, an abrupt change in mental status in an individual with HIV may prompt an evaluation of other medical sources for the cognitive change, incl uding secondary infections. Because HIV in- fection preferentially affects subcortical regi ons over the course of illness, including deep white matter, the progression of the disorder follows a “subcortical ” pattern. Since HIV can affect a variety of brain regions, and the illness can take on many different trajectories
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can affect a variety of brain regions, and the illness can take on many different trajectories depending on associated comorbidities and cons equences of HIV, the overall course of an NCD due to HIV infection has considerable heterogeneity. A subc ortical neurocognitive profile may interact with age over the life course, when psychomo tor slowing and motor impairments such as slowed gait may occur as a consequence of other age-related condi- tions so that the overall progression ma y appear more pronounced in later life. In developed countries, HIV disease is primar ily a condition of adults, with acquisition via risky behaviors (e.g., unprotected sex, inje ction drug use) beginning in late adolescence and peaking during young and middle adulth ood. In developing countries, particularly sub-Saharan Africa, where HIV testing and an tiretroviral treatments for pregnant women are not readily available, perinatal transmis sion is common. The NCD in such infants and children may present primarily as neurodevelopment al delay. As individuals treated for HIV survive into older age, additive and in teractive neurocognitive effects of HIV and aging, including other NCDs (e .g., due to Alzheimer’s dise ase, due to Parkinson’s dis- ease), are possible. Risk and Prognostic Factors Risk and prognostic factors for HIV infection. Risk factors for HIV infection include injec- tion drug use, unprotected sex, and unprotecte d blood supply and othe r iatrogenic factors. Risk and prognostic factors for major or mild neurocognitive disorder due to HIV in- fection. Paradoxically, NCD due to HIV infection ha s not declined significantly with the advent of combined antiretrov iral therapy, although the most severe presentations (con-
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sistent with the diagnosis of major NCD) ha ve decreased sharply. Contributory factors may include inadequate control of HIV in the central nervous system (CNS), the evolution of drug-resistant viral strains, the effects of chronic long-term systemic and brain inflam- mation, and the effects of comorb id factors such as aging, drug abuse, past history of CNS trauma, and co-infections, such as with the hepatitis C virus. Chronic exposure to antiret- roviral drugs also raises the po ssibility of neurotoxicity, alth ough this has not been defin- itively established.
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634 Neurocognitive Disorders Diagnostic Markers Serum HIV testing is required for the diagnosis. In addition, HIV charac terization of the cere- brospinal fluid may be helpful if it reveals a disp roportionately high viral load in cerebrospinal fluid versus in the plasma. Neuroimaging (i.e., magnetic resonance im aging [MRI]) may reveal reduction in total brain volume, cortical thi nning, reduction in white matter volume, and patchy areas of abnormal white matter (hyper intensities). MRI or lumbar puncture may be helpful to exclude a specific medical condition such as cryptococcus infection or herpes en- cephalitis that may contribute to CNS changes in the context of AIDS. Specialized techniques such as diffusion tensor imaging may reveal damage to specific white matter tracts. Functional Consequences of Major or Mild Neurocognitive Disorder Due to HIV Infection Functional consequences of ma jor or mild NCD due to HIV infection are variable across individuals. Thus, impaired executive abiliti es and slowed information processing may substantially interfere with the complex dise ase management decisions required for ad- herence to the combined antire troviral therapy regimen. The likelihood of comorbid dis- ease may further create functional challenges. Differential Diagnosis In the presence of comorbidities, such as other infections (e.g., hepati tis C virus, syphilis), drug abuse (e.g., methamphetamine abuse), or prior head injury or neurodevelopmental conditions, major or mild NCD due to HIV infe ction can be diagnosed provided there is ev- idence that infection with HIV has worsened any NCDs due to such preexisting or comorbid conditions. Among older adults, onset of neur ocognitive decline rela ted to cerebrovascular
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conditions. Among older adults, onset of neur ocognitive decline rela ted to cerebrovascular disease or neurodegeneration (e.g., major or mild NCD due to Alzheimer’s disease) may need to be differentiated. In general, stable, fluctuating (without progression) or improving neurocognitive status would favor an HIV et iology, whereas steady or stepwise deter- ioration would suggest neurodeg enerative or vascular etiolo gy. Because more severe im- munodeficiency can result in opportunistic in fections of the brain (e.g., toxoplasmosis; cryptococcosis) and neoplasia (e.g., CNS lymphoma), sudden onset of an NCD or sudden worsening of that disorder demands acti ve investigation of non-HIV etiologies. Comorbidity HIV disease is accompanied by chronic systemic and neuro-inflammation that can be as- sociated with cerebrovascular disease and metabolic syndrome. These complications can be part of the pathogenesis of major or mild NCD due to HIV infectio n. HIV frequently co- occurs with conditions such as substance use disorders when the substance has been in- jected and other sexually transmitted disorders. Major or Mild Neurocognitive Disorder Due to Prion Disease Diagnostic Criteria A. The criteria are met for major or mild neurocognitive disorder. B. There is insidious onset, and rapid progression of impairment is common. C. There are motor features of prion disease, such as myoclonus or ataxia, or biomarker evidence.
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Major or Mild Neurocognitive Disorder Due to Prion Disease 635 D. The neurocognitive disorder is not attribut able to another medical condition and is not better explained by another mental disorder. Coding note: For major neurocognitive disorder due to prion disease, with behavioral dis- turbance, code first 046.79 (A81.9) prion disease, followed by 294.11 (F02.81) major neurocognitive disorder due to prion disease, with behavioral disturbance. For major neu- rocognitive disorder due to prion disease, without behavioral disturbance, code first 046.79 (A81.9) prion disease, followed by 294.10 (F02.80) major neurocognitive disorder due to prion disease, without behavioral disturbance. For mild neurocognitive disorder due to prion disease, code 331.83 (G31.84). (Note: Do not use the additional code for prion disease. Behavioral disturbance cannot be coded but should still be indicated in writing.) Diagnostic Features The classification of major or mild neurocogni tive disorder (NCD) due to prion disease in- cludes NCDs due to a group of subacute sp ongiform encephalopathies (including Creutz- feldt-Jakob disease, variant Creutzfeldt-Jakob disease, kuru, Gerstmann-Sträussler- Scheinker syndrome, and fatal insomnia) ca used by transmissible agents known as prions. The most common type is sporadic Creutzfeld t-Jakob disease, typically referred to as Creutzfeldt-Jakob disease (CJD). Variant CJD is much rarer and is associated with trans-
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mission of bovine spongiform encephalopathy, also called “mad cow disease.” Typically, individuals with CJD present with neurocognitive deficits , ataxia, and abnormal move- ments such as myoclonus, chorea, or dystonia; a startle reflex is also common. Typically, the history reveals rapid progression to major NCD over as little as 6 months, and thus the disorder is typically seen only at the major le vel. However, many individuals with the dis- order may have atypical presentations, and the disease can be confirmed only by biopsy or at autopsy. Individuals with variant CJD may present with a greater preponderance of psychiatric symptoms, characterized a by lo w mood, withdrawal, and anxiety. Prion dis- ease is typically not diagnosed without at le ast one of the characteristic biomarker fea- tures: recognized lesions on magnetic resonance imaging with DWI (diffusion-weighted imaging) or FLAIR (flu id-attenuated inversion recovery), tau or 14-3-3 protein in cerebro- spinal fluid, characteristic triphasic waves on electroencephalogram, or, for rare familial forms, family history or genetic testing. Prevalence The annual incidence of sporadic CJD is a pproximately one or two cases per million peo- ple. Prevalence is unknown but ve ry low given the short survival. Development and Course Prion disease may develop at any age in adults—the peak age for the sporadic CJD is ap- proximately 67 years—although it has been report ed to occur in individuals spanning the teenage years to late life. Prodromal symptoms of prion disease may include fatigue, anx- iety, problems with appetite or sleeping, or difficulties with concentration. After several
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iety, problems with appetite or sleeping, or difficulties with concentration. After several weeks, these symptoms may be followed by incoordination, altered vision, or abnormal gait or other movements that may be myoclonic, choreoathetoid, or ballistic, along with a rapidly progressive dementia. The disease typi cally progresses very rapidly to the major level of impairment over several months. More rarely, it can progress over 2 years and ap- pear similar in its course to other NCDs.
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636 Neurocognitive Disorders Risk Factors and Prognosis Environmental. Cross-species transmission of prion infections, with agents that are closely related to the human form, has been demonstrated (e.g., the outbreak of bovine spongiform encephalopathy inducing variant CJD in the United Kingdom during the mid- 1990s). Transmission by corneal transplantation and by human growth factor injection has been documented, and anecdotal cases of tran smission to health care workers have been reported. Genetic and physiological. There is a genetic component in up to 15% of cases, associ- ated with an autosoma l dominant mutation. Diagnostic Markers Prion disease can be definitively confirmed only by biopsy or at autopsy. Although there are no distinctive findings on cerebrospinal fluid analysis across the prion diseases, reliable bio- markers are being developed and include 14-3-3 protein (particularly for sporadic CJD) as well as tau protein. Magnetic resonance brain imaging is currently considered the most sen- sitive diagnostic test when DWI is performed, with the most common finding being multi- focal gray matter hyperintensities in subcortical and cortical regions. In some individuals, the electroencephalogram reve als periodic sharp, often triphasic and synchronous dis- charges at a rate of 0.5–2 Hz at some point during the course of the disorder. Differential Diagnosis Other major neurocognitive disorders. Major NCD due to prion disease may appear similar in its course to other NCDs, but prio n diseases are typically distinguished by their rapid progression and prominent cerebellar and motor symptoms. Major or Mild Neurocognitive Disorder Due to Parkinson’s Disease Diagnostic Criteria A. The criteria are met for major or mild neurocognitive disorder.
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Diagnostic Criteria A. The criteria are met for major or mild neurocognitive disorder. B. The disturbance occurs in the setting of established Parkinson’s disease. C. There is insidious onset and gradual progression of impairment. D. The neurocognitive disorder is not attribut able to another medical condition and is not better explained by another mental disorder. Major or mild neurocognitive disorder probably due to Parkinson’s disease should be diagnosed if 1 and 2 are both met. Major or mild neurocognitive disorder possibly due to Parkinson’s disease should be diagnosed if 1 or 2 is met: 1. There is no evidence of mixed etiology (i.e., absence of other neurodegenerative or cerebrovascular disease or another neurological, mental, or systemic disease or con- dition likely contributing to cognitive decline). 2. The Parkinson’s disease clearly precedes the onset of the neurocognitive disorder. Coding note: For major neurocognitive disorder probably due to Parkinson’s disease, with behavioral disturbance, code first 332.0 (G20) Parkinson’s disease, followed by 294.11 (F02.81) major neurocognitive disorder probably due to Parkinson’s disease, with behavioral disturbance. For major neurocognitive disorder probably due to Parkinson’s disease, without behavioral disturbance, code first 332.0 (G20) Parkinson’s disease, fol-
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Major or Mild Neurocognitive Disorder Due to Parkinson’s Disease 637 lowed by 294.10 (F02.80) major neurocognitive disorder probably due to Parkinson’s dis- ease, without behavioral disturbance. For major neurocognitive disorder possibly due to Parkinson’s disease, code 331.9 (G31.9) major neurocognitive disorder possibly due to Parkinson’s disease. ( Note: Do not use the additional code for Parkinson’s disease. Behavioral disturbance cannot be coded but should still be indicated in writing.) For mild neurocognitive disorder due to Parkinson’s disease, code 331.83 (G31.84). (Note: Do not use the additional code for Parkinson’s disease. Behavioral disturbance cannot be coded but should still be indicated in writing.) Diagnostic Features The essential feature of major or mild neurocognitive disorder (NCD) due to Parkinson’s disease is cognitive decline following the onset of Parkinson’s disease. The disturbance must occur in the setting of established Parkinson’s disease (Criterion B), and deficits must have developed gradually (Criterion C). The NCD is viewed as probably due to Parkinson’s disease when there is no evidence of another disorder that might be contributing to the cognitive decline and when the Parkinson’s disease clearly precedes onset of the NCD. The NCD is considered possibly due to Parkinson’s disease either when there is no evidence of another disorder that might be contributing to the cognitive decline or when the Parkin- son’s disease precedes onset of the NCD, but not both. Associated Features Supporting Diagnosis Frequently present features include apathy , depressed mood, anxious mood, hallucina- tions, delusions, personality changes, rapid eye movement sleep behavior disorder, and excessive daytime sleepiness. Prevalence
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excessive daytime sleepiness. Prevalence The prevalence of Parkinson’s disease in the United States steadily increases with age from approximately 0.5% between ages 65 and 69 to 3% at age 85 years and older. Parkinson’s disease is more common in males than in females. Among individuals with Parkinson’s disease, as many as 75% will develop a major NC D sometime in the course of their disease. The prevalence of mild NCD in Parkinso n’s disease has been estimated at 27%. Development and Course Onset of Parkinson’s disease is typically betw een the sixth and ninth decades of life, with most expression in the early 60s. Mild NCD ofte n develops relatively early in the course of Parkinson’s disease, whereas major impairme nt typically does not occur until late. Risk and Prognostic Factors Environmental. Risk factors for Parkinson’s disease include exposure to herbicides and pesticides. Genetic and physiological. Potential risk factors for NC D among individuals with Par- kinson’s disease include older age at diseas e onset and increasing duration of disease. Diagnostic Markers Neuropsychological testing, with a focus on test s that do not rely on motor function, is crit- ical in detecting the core cogn itive deficits, particularly at the mild NCD phase. Structural neuroimaging and dopamine transporter scans, such as DaT scans, may differentiate Lewy body–related dementias (Parkinson’s and dementia wi th Lewy bodies) from non–
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638 Neurocognitive Disorders Lewy body–related dementias (e.g., Alzheimer’s disease) and can sometimes be helpful in the evaluation of major or mild NCD due to Parkinson’s disease. Differential Diagnosis Major or mild neurocognitive disorder with Lewy bodies. This distinction is based sub- stantially on the timing and sequence of mo tor and cognitive symptoms. For NCD to be at- tributed to Parkinson’s diseas e, the motor and other symptoms of Parkinson’s disease must be present well before (by convention, at leas t 1 year prior) cognit ive decline has reached the level of major NCD, whereas in major or mild NCD with Lewy bodies, cognitive symp- toms begin shortly before, or concurrent wi th, motor symptoms. For mild NCD, the timing is harder to establish because the diagnosis itse lf is less clear and th e two disorders exist on a continuum. Unless Parkinson’s disease has been established for some time prior to the onset of cognitive decline, or typical features of major or mild NCD with Lewy bodies are present, it is preferable to diagnose unspecified mild NCD. Major or mild neurocognitive disorder due to Alzheimer’s disease. The motor features are the key to distinguishing major or mild NCD due to Parkinson’s disease from major or mild NCD due to Alzheimer’s disease. However, the two disorders can co-occur. Major or mild vascular neurocognitive disorder. Major or mild vascular NCD may pre- sent with parkinsonian features such as psychomotor slowing that may occur as a conse- quence of subcortical small vessel disease. However, the parkinsonian features typically are not sufficient for a diagnosis of Parkinson’s disease, and the course of the NCD usually has a clear association with cerebrovascular changes.
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has a clear association with cerebrovascular changes. Neurocognitive disorder due to another medical condition (e.g., neurodegenerative disorders). When a diagnosis of major or mild NC D due to Parkinson’s disease is being considered, the distinction must also be made from other brain diso rders, such as progres- sive supranuclear palsy, corticobasal degeneration, multiple system atrophy, tumors, and hydrocephalus. Neuroleptic-induced parkinsonism. Neuroleptic-induced parkinsonism can occur in individuals with other NCDs, particularly when dopamine-blocking drugs are prescribed for the behavioral manifest ations of such disorders Other medical conditions. Delirium and NCDs due to side effects of dopamine-blocking drugs and other medical conditio ns (e.g., sedation or impair ed cognition, severe hypothy- roidism, B12 deficiency) must al so be ruled out. Comorbidity Parkinson’s disease may coexist with Alzheimer’ s disease and cerebrovascular disease, espe- cially in older individuals. The compounding of multiple pathological features may diminish the functional abilities of individuals with Pa rkinson’s disease. Moto r symptoms and frequent co-occurrence of depression or apathy can make functional impairment worse. Major or Mild Neurocognitive Disorder Due to Huntington’s Disease Diagnostic Criteria A. The criteria are met for major or mild neurocognitive disorder. B. There is insidious onset and gradual progression. C. There is clinically established Huntington’ s disease, or risk for Huntington’s disease based on family history or genetic testing.
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Major or Mild Neurocognitive Disorder Due to Huntington’s Disease 639 D. The neurocognitive disorder is not attribut able to another medical condition and is not better explained by another mental disorder. Coding note: For major neurocognitive disorder due to Huntington’s disease, with behav- ioral disturbance, code first 333.4 (G10) Huntington’s disease, followed by 294.11 (F02.81) major neurocognitive disorder due to Huntington’s disease, with behavioral dis- turbance. For major neurocognitive disorder due to Huntington’s disease, without behav- ioral disturbance, code first 333.4 (G10) Huntington’s disease, followed by 294.10 (F02.80) major neurocognitive disorder due to Huntington’s disease, without behavioral disturbance. For mild neurocognitive disorder due to Huntington’s disease, code 331.83 (G31.84). (Note: Do not use the additional code for Huntington’s disease. Behavioral disturbance cannot be coded but should still be indicated in writing.) Diagnostic Features Progressive cognitive impairment is a core feature of Huntington ’s disease, with early changes in executive function (i.e., processing speed, organization, and planni ng) rather than learn- ing and memory. Cognitive and associated beha vioral changes often precede the emergence of the typical motor abnormalities of bradykinesia (i.e., slowing of voluntary movement) and chorea (i.e., involuntary jerking movements). A diagnosis of definite Huntington’s dis- ease is given in the presence of unequivocal, extrapyramidal motor ab normalities in an in- dividual with either a family history of Huntington’s disease or genetic testing showing a CAG trinucleotide repeat expansion in the HTT gene, located on chromosome 4.
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Associated Features Supporting Diagnosis Depression, irritability, anxiety, obsessive -compulsive symptoms, and apathy are fre- quently, and psychosis more rarely, associat ed with Huntington’s disease and often pre- cede the onset of motor symptoms. Prevalence Neurocognitive deficits are an eventual ou tcome of Huntington’s disease; the worldwide prevalence is estimated to be 2.7 per 100,000. The prevalence of Huntington’s disease in North America, Europe, and Australia is 5.7 pe r 100,000, with a much lower prevalence of 0.40 per 100,000 in Asia. Development and Course The average age at diagnosis of Huntington’s disease is approximately 40 years, although this varies widely. Age at onset is inversely correlated with CAG expansion length. Juve- nile Huntington’s disease (ons et before age 20) may present more commonly with brady- kinesia, dystonia, and rigidity than with the choreic movements characteristic of the adult- onset disorder. The disease is gradually progre ssive, with median survival approximately 15 years after motor symptom diagnosis. Phenotypic expression of Huntington’s diseas e varies by presence of motor, cognitive, and psychiatric symptoms. Psychiatric and co gnitive abnormalities can predate the motor abnormality by at least 15 years. Initial symp toms requiring care often include irritabity, anxiety, or depressed mood. Other behavioral disturbances may include pronounced ap- athy, disinhibition, impulsivit y, and impaired insight, with apathy often becoming more progressive over time. Early movement symptoms may involve the appearance of fidget- iness of the extremitie s as well as mild apraxia (i.e., difficulty with purposeful movements),
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particularly with fine motor tasks. As the disorder progre sses, other motor problems in- clude impaired gait (ataxia ) and postural instability. Motor impairment eventually affects speech production ( dysarthria ) such that the speech becomes very difficult to understand,
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640 Neurocognitive Disorders which may result in significant distress resu lting from the communication barrier in the context of comparatively intact cognition. Advanced motor disease severely affects gait with progressive ataxia. Eventually individuals become nonambulatory. End-stage motor disease impairs motor control of eating and swallowing, typically a major contributor to the death of the individual from aspiration pneumonia. Risk and Prognostic Factors Genetic and physiological. The genetic basis of Huntington’s disease is a fully penetrant autosomal dominant expansion of th e CAG trincleotide, often called a CAG repeat in the huntingtin gene. A repeat length of 36 or more is invariably associated with Huntington’s disease, with longer repeat lengths associated with early age at onset. A CAG repeat length of 36 or more is invariably asso ciated with Huntington’s disease. Diagnostic Markers Genetic testing is the primary laboratory test for the determination of Huntington’s dis- ease, which is an autosomal dominant disord er with complete penetrance. The trinucleo- tide CAG is observed to have a repeat ex pansion in the gene that encodes huntingtin protein on chromosome 4. A di agnosis of Huntington’s disease is not made in the presence of the gene expansion alone, but the diagno sis is made only after symptoms become man- ifest. Some individuals with a positive family history request genetic testing in a presymp- tomatic stage. Associat ed features may also include neur oimaging changes; volume loss in the basal ganglia, particularly the caudate nu cleus and putamen, is well known to occur and progresses over the course of illness. Other structural and functional changes have been observed in brain imagin g but remain research measures. Functional Consequences of Major or Mild
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Functional Consequences of Major or Mild Neurocognitive Disorder Du e to Huntington’s Disease In the prodromal phase of illness and at early diagnosis, occupational decline is most com- mon, with most individuals repo rting some loss of ability to engage in their typical work. The emotional, behavioral, and cognitive aspects of Huntington’s disease, such as disin- hibition and personality changes, are highly associated with functional decline. Cognitive deficits that contribute most to functional decline may incl ude speed of processing, initi- ation, and attention rather th an memory impairment. Given that Huntington’s disease on- set occurs in productive years of life, it may have a very disruptive effect on performance in the work setting as well as social and fam ily life. As the disease progresses, disability from problems such as impair ed gait, dysarthria, and impulsive or irritable behaviors may substantially add to the level of impairment and daily care needs, over and above the care needs attributable to the cognitive decline. Severe choreic moveme nts may substantially interfere with provision of care such as bathing, dressing, and toileting. Differential Diagnosis Other mental disorders. Early symptoms of Huntington’s disease may include instabil- ity of mood, irritability, or compulsive behaviors that may suggest another mental disor- der. However, genetic testing or the develo pment of motor symptoms will distinguish the presence of Huntington’s disease. Other neurocognitive disorders. The early symptoms of Huntington’s disease, particu- larly symptoms of executive dysfunction an d impaired psychomotor speed, may resemble other neurocognitive disorders (NCDs), such as major or mild vascular NCD.
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Major or Mild Neurocognitive Disorder Due to Another Medical Condition 641 Other movement disorders. Huntington’s disease must also be differentiated from other disorders or conditions associated with chorea, such as Wilson’s disease, drug-induced tardive dyskinesia, Sydenham’s chorea, systemic lupus erythematosus, or senile chorea. Rarely, individuals may present with a course similar to that of Huntington’s disease but without positive genetic testin g; this is considered to be a Huntington’s disease pheno- copy that results from a variet y of potential genetic factors. Major or Mild Neurocognitive Disorder Due to Another Medical Condition Diagnostic Criteria A. The criteria are met for major or mild neurocognitive disorder. B. There is evidence from the history, physical examination, or laboratory findings that the neurocognitive disorder is the pathophysi ological consequence of another medical condition. C. The cognitive deficits are not better explained by another mental disorder or another specific neurocognitive disorder (e.g., Alzheimer’s disease, HIV infection). Coding note: For major neurocognitive disorder due to another medical condition, with behavioral disturbance, code first the other medical condition, followed by the major neu- rocognitive disorder due to another medical condition, with behavioral disturbance (e.g., 340 [G35] multiple sclerosis, 294.11 [F02.81] major neurocognitive disorder due to multi- ple sclerosis, with behavioral disturbance). For major neurocognitive disorder due to an- other medical condition, without behavioral disturbance, code first the other medical condition, followed by the major neurocognitive disorder due to another medical condition, without behavioral disturbance (e.g., 340 [G35] multiple sclerosis, 294.10 [F02.80] major
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neurocognitive disorder due to multiple sclerosis, without behavioral disturbance). For mild neurocognitive disorder due to another medical condition, code 331.83 (G31.84). (Note: Do not use the additional code for the other medical condition. Behavioral distur- bance cannot be coded but should still be indicated in writing.) Diagnostic Features A number of other medical co nditions can cause neurocognitive disorders (NCDs). These conditions include structural lesions (e.g., primary or secondary brain tumors, subdural hematoma, slowly progressive or normal-press ure hydrocephalus), hypoxia related to hy- poperfusion from heart failure, endocrine co nditions (e.g., hypothyroidism, hypercalce- mia, hypoglycemia), nutritional conditions (e.g., deficiencies of thiamine or niacin), other infectious conditions (e.g., neurosyphilis, cryptococcosis), im mune disorders (e.g., tempo- ral arteritis, systemic lupus erythematosus), hepatic or rena l failure, metabolic conditions (e.g., Kufs’ disease, adrenoleukodystrophy, metachromatic leukodystrophy, other storage diseases of adulthood and childhood), and ot her neurological conditions (e.g., epilepsy, multiple sclerosis). Unusual causes of central nervous system injury, such as electrical shock or intracranial radiation, are generally evident from the history. The temporal asso- ciation between the onset or exacerbation of the medical condition and the development of the cognitive deficit offers the greatest supp ort that the NCD is induced by the medical condition. Diagnostic certainty regarding this relationship may be increased if the neuro- cognitive deficits ameliorate partially or stab ilize in the context of treatment of the medical
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condition.
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642 Neurocognitive Disorders Development and Course Typically the course of the NCD progresses in a manner that is commensurate with progres- sion of the underlying medical disorder. In ci rcumstances where the medical disorder is treat- able (e.g., hypothyroidism), th e neurocognitive deficit may im prove or at least not progress. When the medical condition has a deteriorative course (e.g., secondary progressive multiple sclerosis), the neurocognitive deficits will progre ss along with the tempor al course of illness. Diagnostic Markers Associated physical examinatio n and laboratory findings and other clinical features de- pend on the nature and severity of the medical condition. Differential Diagnosis Other major or mild neurocognitive disorder. The presence of an attributable medical condition does not entirely exclude the possibility of another major or mild NCD. If cog- nitive deficits persist following successful treatment of an associated medical condition, then another etiology may be responsible for the cognitive decline. Major or Mild Neurocognitive Disorder Due to Multiple Etiologies Diagnostic Criteria A. The criteria are met for major or mild neurocognitive disorder. B. There is evidence from the history, physical examination, or laboratory findings that the neurocognitive disorder is the pathophysiological consequence of more than one etio- logical process, excluding substances (e.g., neurocognitive disorder due to Alzhei- mer’s disease with subsequent development of vascular neurocognitive disorder). Note: Please refer to the diagnostic criteria for the various neurocognitive disorders due to specific medical conditions for guidance on establishing the pa rticular etiologies. C. The cognitive deficits are not better ex plained by another mental disorder and do not occur exclusively during the course of a delirium.
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occur exclusively during the course of a delirium. Coding note: For major neurocognitive disorder due to multiple etiologies, with behavioral disturbance, code 294.11 (F02.81) ; for major neurocognitive disorder due to multiple etiolo- gies, without behavioral disturbance, code 294.10 (F02.80). All of the etiological medical conditions (with the exception of vascular disease) should be coded and listed separately immediately before major neurocognitive diso rder due to multiple etiologies (e.g., 331.0 [G30.9] Alzheimer’s disease; 331.82 [G31.83] Lewy body disease; 294.11 [F02.81] major neurocognitive disorder due to multiple etiologies, with behavioral disturbance). When a cerebrovascular etiology is contributin g to the neurocognitive disorder, the diagno- sis of vascular neurocognitive disorder should be listed in addition to major neurocognitive disorder due to multiple etiologies. For example, for a presentation of major neurocognitive disorder due to both Alzheimer’s disease and va scular disease, with behavioral disturbance, code the following: 331.0 (G30.9) Alzheimer’s disease; 294.11 (F02.81) major neurocogni- tive disorder due to multiple etiologies, with behavioral disturbance; 290.40 (F01.51) major vascular neurocognitive disorder, with behavioral disturbance. For mild neurocognitive disorder due to multiple etiologies, code 331.83 (G31.84). (Note: Do not use the additional codes for the etiologies. Behavioral disturbance cannot be coded but should still be indicated in writing.)
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Unspecified Neurocognitive Disorder 643 This category is included to cover the clinical presentation of a neurocognitive disorder (NCD) for which there is evidence that multiple medical conditions have played a probable role in the development of the NCD. In addition to evidence indicative of the presence of multiple med- ical conditions that are known to cause NCD (i .e., findings from the hi story and physical ex- amination, and laboratory findings), it may be he lpful to refer to the diag nostic criteria and text for the various medical etiologies (e.g., NCD du e to Parkinson’s disease) for more information on establishing the etiological connection for that particular medical condition. Unspecified Neurocognitive Disorder 799.59 (R41.9) This category applies to presentations in which symptoms characteristic of a neurocogni- tive disorder that cause clinically significant distress or impairment in social, occupational, or other important areas of functioning predominate but do not meet the full criteria for any of the disorders in the neurocognitive diso rders diagnostic class. The unspecified neuro- cognitive disorder category is used in situations in which the precise etiology cannot be determined with sufficient certainty to make an etiological attribution. Coding note: For unspecified major or mild neurocognitive disorder, code 799.59 (R41.9). (Note: Do not use additional codes for any presumed etiological medical conditions. Be- havioral disturbance cannot be coded but may be indicated in writing.)
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645Personality Disorders This chapter begins with a general definition of personality disorder that applies to each of the 10 specific personality disorders. A personality disorder is an enduring pattern of inner experience and behavior that deviates markedly from the expectations of the in- dividual’s culture, is pervasive and inflexible , has an onset in adolescence or early adult- hood, is stable over time, and lead s to distress or impairment. With any ongoing review process, especially one of this complexity, different view- points emerge, and an effort was made to accommodate them. Thus, personality disorders are included in both Sections II and III. The material in Section II represents an update of text associated with the same criteria foun d in DSM-IV-TR, whereas Section III includes the proposed research model for personality disorder diagnosis and conceptualization de- veloped by the DSM-5 Personality and Persona lity Disorders Work Group. As this field evolves, it is hoped that both versions will serve clinical practice and research initiatives, respectively. The following personality disorders are included in this chapter. •Paranoid personality disorder is a pattern of distrust and suspiciousness such that oth- ers’ motives are interp reted as malevolent. •Schizoid personality disorder is a pattern of detachment fr om social relationships and a restricted range of emotional expression. •Schizotypal personality disorder is a pattern of acute discomfort in close relationships, cognitive or perceptual distortions, and eccentricities of behavior. •Antisocial personality disorder is a pattern of disregard for, and violation of, the rights of others. •Borderline personality disorder is a pattern of instability in interpersonal relation- ships, self-image, and affects, and marked impulsivity.
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ships, self-image, and affects, and marked impulsivity. •Histrionic personality disorder is a pattern of excessive emotionality and attention seeking. •Narcissistic personality disorder is a pattern of grandiosity, need for admiration, and lack of empathy. •Avoidant personality disorder is a pattern of social inhibition, feelings of inadequacy, and hypersensitivity to negative evaluation. •Dependent personality disorder is a pattern of submissive and clinging behavior re- lated to an excessive need to be taken care of. •Obsessive-compulsive personality disorder is a pattern of preoccupation with order- liness, perfectionism, and control. •Personality change due to another medical condition is a persistent personality dis- turbance that is judged to be due to the direct physiological effects of a medical condi- tion (e.g., frontal lobe lesion). •Other specified personality disorder and unspecified personality disorder is a cate- gory provided for two situations: 1) the individual’s personality pattern meets the gen- eral criteria for a personality disorder, an d traits of several different personality disorders are present, but the criteria for any specific pers onality disorder are not met;
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646 Personality Disorders or 2) the individual’s personality pattern m eets the general criteria for a personality dis- order, but the individual is considered to have a personality disorder that is not in- cluded in the DSM-5 classification (e.g., passive-aggressive personality disorder). The personality disorders are grouped into thre e clusters based on descriptive similarities. Cluster A includes paranoid, schizoid, and schi zotypal personality diso rders. Individuals with these disorders often appear odd or eccentric. Cluster B includes antisocial, borderline, histri- onic, and narcissistic personality disorders. In dividuals with these disorders often appear dra- matic, emotional, or erratic. Cluster C in cludes avoidant, dependent, and obsessive- compulsive personality disorders. Individuals with these disorders often appear anxious or fearful. It should be noted that this clustering system, although useful in some research and ed- ucational situations, has serious limitations and has not been consistently validated. Moreover, individuals freque ntly present with co-occu rring personality disorders from different clusters. Prevalence estimates fo r the different clusters suggest 5.7% for dis- orders in Cluster A, 1.5% for disorders in Cl uster B, 6.0% for disorders in Cluster C, and 9.1% for any personality disorder, indicating frequent co-occurrence of disorders from dif- ferent clusters. Data from the 2001–2002 National Epidemiologic Survey on Alcohol and Related Conditions suggest that approximately 15% of U.S. adults have at least one per- sonality disorder. Dimensional Models for Personality Disorders The diagnostic approach used in this manual represents the categorical perspective that personality disorders are qualit atively distinct clinical synd romes. An alternative to the
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personality disorders are qualit atively distinct clinical synd romes. An alternative to the categorical approach is the dimensional perspective that personality disorders represent maladaptive variants of personality traits that merge imperceptibly into normality and into one another. See Section III for a full description of a dimensional model for person- ality disorders. The DSM-IV personality disord er clusters (i.e., odd-eccentric, dramatic- emotional, and anxious- fearful) may also be viewed as dimensions representing spectra of personality dysfunction on a continuum with other mental disorders. The alternative di- mensional models have much in common and together appear to cover the important ar- eas of personality dysfunction. Their integration, clinical utility, and relationship with the personality disorder diagnostic categories and various aspects of personality dysfunction are under active investigation. General Personality Disorder Criteria A. An enduring pattern of inner experience and behavior that deviates markedly from the expectations of the individual’s culture. This pattern is manifested in two (or more) of the following areas: 1. Cognition (i.e., ways of perceiving and interpreting self, other people, and events). 2. Affectivity (i.e., the range, intensity, lability, and appropriateness of emotional re- sponse). 3. Interpersonal functioning. 4. Impulse control. B. The enduring pattern is inflexible and per vasive across a broad range of personal and social situations. C. The enduring pattern leads to clinically signi ficant distress or impairment in social, oc- cupational, or other important areas of functioning.
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General Personality Disorder 647 D. The pattern is stable and of long duration, and its onset can be traced back at least to adolescence or early adulthood. E. The enduring pattern is not better explained as a manifestation or consequence of an- other mental disorder. F. The enduring pattern is not attributable to the physiological effects of a substance (e.g., a drug of abuse, a medication) or another medical condition (e.g., head trauma). Diagnostic Features Personality traits are enduring patterns of perceiving, relating to, and thinking about the en- vironment and oneself that are exhibited in a wide range of social and personal contexts. Only when personality traits are inflexible and maladaptive and cause significant func- tional impairment or subjective distress do th ey constitute personality disorders. The essen- tial feature of a personality disorder is an en during pattern of inner experience and behavior that deviates markedly from the expectations of the individual’s culture and is manifested in at least two of the following areas: cognition, affectivity, interpersonal functioning, or im- pulse control (Criterion A). This enduring pattern is inflexib le and pervasive across a broad range of personal and social sit uations (Criterion B) and leads to clinically significant dis- tress or impairment in social, occupational, or other important areas of functioning (Crite- rion C). The pattern is stable and of long duration, and its onset can be traced back at least to adolescence or early adulthood (Criterion D). The pattern is not better explained as a manifestation or consequence of another mental disorder (Criterion E) and is not attribut- able to the physiological effects of a substanc e (e.g., a drug of abuse, a medication, exposure to a toxin) or another medical condition (e.g., head trauma) (Criterion F). Specific diagnostic
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criteria are also provided for each of the pe rsonality disorders included in this chapter. The diagnosis of personality disorders requires an evaluation of the individual’s long- term patterns of functioning, and the partic ular personality features must be evident by early adulthood. The personality traits that define these disorders must also be distin- guished from characteristics th at emerge in response to sp ecific situational stressors or more transient mental states (e .g., bipolar, depressive, or an xiety disorders; substance in- toxication). The clinician should assess the stability of pers onality traits over time and across different situations. Although a single interview with the in dividual is sometimes sufficient for making the diagnosis, it is of ten necessary to conduct more than one inter- view and to space these over time. Assessment ca n also be complicated by the fact that the characteristics that define a personality di sorder may not be considered problematic by the individual (i.e., the traits are often ego-sy ntonic). To help overcome this difficulty, sup- plementary information from other informants may be helpful. Development and Course The features of a personality disorder usually become recogn izable during adolescence or early adult life. By definition, a personality disorder is an enduring pattern of thinking, feeling, and behaving that is relatively stable over time. Some types of personality disorder (notably, antisocial and borderli ne personality disorders) tend to become less evident or to remit with age, whereas this appears to be less true for some other types (e.g., obsessive- compulsive and schizotypal personality disorders). Personality disorder categories may be app lied with children or adolescents in those relatively unusual instances in which the individual’s particular maladaptive personality traits appear to be pervasive, persistent, an d unlikely to be limited to a particular devel-
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traits appear to be pervasive, persistent, an d unlikely to be limited to a particular devel- opmental stage or another mental disorder. It sh ould be recognized that the traits of a per-
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traits appear to be pervasive, persistent, an d unlikely to be limited to a particular devel- opmental stage or another mental disorder. It sh ould be recognized that the traits of a per- sonality disorder that appear in childhood will often not persist unchanged into adult life. For a personality disorder to be diagnosed in an individual younger than 18 years, the fea- tures must have been present fo r at least 1 year. The one exception to this is antisocial per-
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648 Personality Disorders sonality disorder, which cannot be diagnosed in individuals younger than 18 years. Al- though, by definition, a personality disorder requires an onset no later than early adulthood, individuals may not come to clinical attention until relatively late in life. A per- sonality disorder may be exacer bated following the loss of significant supporting persons (e.g., a spouse) or previously stabilizing social situations (e.g ., a job). However, the devel- opment of a change in person ality in middle adulthood or later life warrants a thorough evaluation to determine the possible presence of a personality change due to another med- ical condition or an unrecogn ized substance use disorder. Culture-Related Diagnostic Issues Judgments about personality functioning must take into account the in dividual’s ethnic, cul- tural, and social background. Personality disord ers should not be confused with problems as- sociated with acculturation following immigratio n or with the expressi on of habits, customs, or religious and political values professed by the individual’s culture of origin. It is useful for the clinician, especially when evaluating some one from a different background, to obtain ad- ditional information from informants who are fa miliar with the person’s cultural background. Gender-Related Diagnostic Issues Certain personality disorders (e.g., antisocial personality disorder) are diagnosed more frequently in males. Others (e.g., borderline, histrionic, and dependent personality disor- ders) are diagnosed more freque ntly in females. Although these differences in prevalence probably reflect real gender differences in the presence of such patterns, clinicians must be cautious not to overdiagnose or underdiagnos e certain personality disorders in females or in males because of social stereotypes about typical gender roles and behaviors. Differential Diagnosis
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in males because of social stereotypes about typical gender roles and behaviors. Differential Diagnosis Other mental disorders and personality traits. Many of the specific criteria for the per- sonality disorders describe features (e.g., su spiciousness, dependency, insensitivity) that are also characteristic of episo des of other mental disorders. A personality disorder should be diagnosed only when the defining characte ristics appeared before early adulthood, are typical of the individual’s long-term function ing, and do not occur exclusively during an episode of another mental diso rder. It may be particularly difficult (and not particularly useful) to distinguish personality disorders from persistent mental disorders such as per- sistent depressive disorder that have an ea rly onset and an enduring, relatively stable course. Some personality diso rders may have a “spectrum” relationship to other mental disorders (e.g., schizotypal personality disord er with schizophrenia; avoidant personality disorder with social anxiety disorder [social phobia]) based on phe nomenological or bio- logical similarities or familial aggregation. Personality disorders must be distinguished from personality traits that do not reach the threshold for a personality disorder. Person ality traits are diagnosed as a personality disorder only when they are inflexible, maladaptive, and persisting and cause significant functional impairment or subjective distress. Psychotic disorders. For the three personality disorders that may be related to the psy- chotic disorders (i.e., paranoid, schizoid, and schizotypal), there is an exclusion criterion stating that the pattern of behavior must no t have occurred exclusively during the course of schizophrenia, a bipolar or depressive disorder with psycho tic features, or another psy- chotic disorder. When an individual has a pers istent mental disorder (e.g., schizophrenia)
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that was preceded by a preexisting personalit y disorder, the personality disorder should also be recorded, followed by “premorbid” in parentheses. Anxiety and depressive disorders. The clinician must be cautious in diagnosing per-
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also be recorded, followed by “premorbid” in parentheses. Anxiety and depressive disorders. The clinician must be cautious in diagnosing per- sonality disorders during an episode of a depressive disorder or an anxiety disorder, be-
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Paranoid Personality Disorder 649 cause these conditions may have cross-sectio nal symptom features that mimic personality traits and may make it more difficult to evaluate retrospectively the individual’s long-term patterns of functioning. Posttraumatic stress disorder. When personality changes emerge and persist after an individual has been exposed to extreme stress, a diagnosis of posttraumatic stress disorder should be considered. Substance use disorders. When an individual has a substance use disorder, it is impor- tant not to make a personality disorder diag nosis based solely on behaviors that are con- sequences of substance intoxication or withdraw al or that are associated with activities in the service of sustaining substance use (e.g., antisocial behavior). Personality change due to another medical condition. When enduring changes in per- sonality arise as a result of the physiological effects of another medical condition (e.g., brain tumor), a diagnosis of personality chan ge due to another medical condition should be considered. Cluster A Personality Disorders Paranoid Personality Disorder Diagnostic Criteria 301.0 (F60.0) A. A pervasive distrust and suspiciousness of others such that their motives are inter- preted as malevolent, beginning by early adulthood and present in a variety of con- texts, as indicated by four (or more) of the following: 1. Suspects, without sufficient basis, that others are exploiting, harming, or deceiving him or her. 2. Is preoccupied with unjustified doubts about the loyalty or trustworthiness of friends or associates. 3. Is reluctant to confide in others because of unwarranted fear that the information will be used maliciously against him or her. 4. Reads hidden demeaning or threatening meanings into benign remarks or events.
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4. Reads hidden demeaning or threatening meanings into benign remarks or events. 5. Persistently bears grudges (i.e., is unforgiving of insults, injuries, or slights). 6. Perceives attacks on his or her character or reputation that are not apparent to oth- ers and is quick to react angrily or to counterattack. 7. Has recurrent suspicions, without justification, regarding fidelity of spouse or sexual partner. B. Does not occur exclusively during the course of schizophrenia, a bipolar disorder or depressive disorder with psychotic features, or another psychotic disorder and is not attributable to the physiological effects of another medical condition. Note: If criteria are met prior to the onset of schizophrenia, add “premorbid,” i.e., “paranoid personality disorder (premorbid).” Diagnostic Features The essential feature of parano id personality disorder is a pattern of pervasive distrust and suspiciousness of others such that their motives are interpreted as malevolent. This pattern begins by early adulthood and is present in a variety of contexts.
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650 Personality Disorders Individuals with this disorder assume that other people will exploit, harm, or deceive them, even if no evidence exists to support th is expectation (Criterion A1). They suspect on the basis of little or no eviden ce that others are plotting against them and may attack them suddenly, at any time and without reason. They often feel that they have been deeply and irreversibly injured by another person or persons even when there is no objective evidence for this. They are preoccupied with unjustifie d doubts about the loyalty or trustworthiness of their friends and associates, whose actions are minutely scrutinize d for evidence of hos- tile intentions (Criterion A2). Any perceived deviation from trustworthiness or loyalty serves to support their underlying assumptions. They are so amazed when a friend or as- sociate shows loyalty that they cannot trust or believe it. If they get into trouble, they ex- pect that friends and associates w ill either attack or ignore them. Individuals with paranoid personality disord er are reluctant to confide in or become close to others because they fear that the information they share will be used against them (Criterion A3). They may refuse to answer personal questions, saying that the information is “nobody’s business.” They read hidden meanings that are demeaning and threatening into benign remarks or events (Criterion A4). For example, an individual with this disor- der may misinterpret an honest mistake by a store clerk as a deliberate attempt to short- change, or view a casual humorous remark by a co-worker as a seri ous character attack. Compliments are often misinterpreted (e.g., a compliment on a new acquisition is mis- interpreted as a criticism for selfishness; a compliment on an accomplishment is misinter-
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interpreted as a criticism for selfishness; a compliment on an accomplishment is misinter- preted as an attempt to coerce more and better performance). They may view an offer of help as a criticism that they ar e not doing well enough on their own. Individuals with this disorder persistently bear grudges and are unwilling to forgive the insults, injuries, or slights that they th ink they have received (Criterion A5). Minor slights arouse major hostility, and the hostile f eelings persist for a long time. Because they are constantly vigilant to the harmful intentions of others, they very often feel that their character or reputation has been attacked or that they have been slighted in some other way. They are quick to counterattack and react with anger to perceived insults (Criterion A6). Individuals with this disorder may be pathologically jealous, often suspecting that their spouse or sexual partner is unfaithful without any adequate ju stification (Criterion A7). They may gather trivial and circumstantial “evidence” to support their jealous beliefs. They want to maintain complete control of intimate relationships to avoid being betrayed and may constantly question and challenge th e whereabouts, actions, intentions, and fi- delity of their spouse or partner. Paranoid personality disorder should not be diagnosed if the pattern of behavior oc- curs exclusively during the course of schizo phrenia, a bipolar disorder or depressive dis- order with psychotic features, or another psychoti c disorder, or if it is attributable to the physiological effects of a neurological (e.g., temporal lobe epilepsy) or another medical condition (Criterion B). Associated Features Supporting Diagnosis Individuals with paranoid personality disorder are generally difficult to get along with and often have problems with close relationsh ips. Their excessive suspiciousness and hos-
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and often have problems with close relationsh ips. Their excessive suspiciousness and hos- tility may be expressed in over t argumentativeness, in recurr ent complaining, or by quiet, apparently hostile aloofness. Because they ar e hypervigilant for potential threats, they may act in a guarded, secretive, or devious manner and appear to be “cold” and lacking in tender feelings. Although they may appear to be objective, rational , and unemotional, they
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may act in a guarded, secretive, or devious manner and appear to be “cold” and lacking in tender feelings. Although they may appear to be objective, rational , and unemotional, they more often display a labile range of affect, with hostile, stub born, and sarcastic expressions predominating. Their combative and suspicious nature may elicit a hostile response in others, which then serves to conf irm their original expectations. Because individuals with paranoid personality disorder lack trust in others, they have an excessive need to be self-sufficient and a strong sense of autonomy. They also need to
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Paranoid Personality Disorder 651 have a high degree of control over those around them. They are often rigid, critical of oth- ers, and unable to collaborate, although they have great difficulty accepting criticism them- selves. They may blame others for their own shortcomings. Because of their quickness to counterattack in response to the threats they perceive around them, they may be litigious and frequently become involved in legal disputes. Individuals with this disorder seek to confirm their preconceived nega tive notions regarding people or situations they encounter, attributing malevolent motivati ons to others that are projections of their own fears. They may exhibit thinly hidden, unrealistic grandios e fantasies, are often attuned to issues of power and rank, and tend to develop negative stereotypes of others, particularly those from population groups distinct from their own. Attracted by simplistic formulations of the world, they are often wary of ambiguous situations. They ma y be perceived as “fanatics” and form tightly knit “cults” or groups with others who share their paranoid belief systems. Particularly in response to stress, individu als with this disorder may experience very brief psychotic episodes (lasting minutes to hour s). In some instances, paranoid personal- ity disorder may appear as the premorbid ante cedent of delusional disorder or schizo- phrenia. Individuals with paranoid personal ity disorder may deve lop major depressive disorder and may be at increased risk fo r agoraphobia and obsessive-compulsive dis- order. Alcohol and other substance use disord ers frequently occur. The most common co- occurring personality disorders appear to be schizotypal, schizoid, narcissistic, avoidant, and borderline. Prevalence A prevalence estimate for paranoid personality based on a probability subsample from
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Prevalence A prevalence estimate for paranoid personality based on a probability subsample from Part II of the National Comorbidity Survey Replication suggests a prevalence of 2.3%, while the National Epidemiologic Survey on Alcohol and Related Conditions data suggest a prevalence of paranoid personality disorder of 4.4%. Development and Course Paranoid personality disorder may be first apparent in childhood and adolescence with solitariness, poor peer relationships, social anxiety, underachievement in school, hyper- sensitivity, peculiar thoughts and language, and idiosyncratic fantasies. These children may appear to be “odd” or “eccentric” and attr act teasing. In clinical samples, this disorder appears to be more commonly diagnosed in males. Risk and Prognostic Factors Genetic and physiological. There is some evidence for an increased prevalence of par- anoid personality disorder in relatives of probands with schizophrenia and for a more spe- cific familial relationship with delusional disorder, persecutory type. Culture-Related Diagnostic Issues Some behaviors that are influenced by sociocultural contexts or specific life circumstances may be erroneously labeled paranoid and may ev en be reinforced by the process of clinical evaluation. Members of minority groups, immi grants, political and economic refugees, or individuals of different ethnic backgrounds may display guarded or defensive behaviors because of unfamiliarity (e.g., language barriers or lack of knowledge of rules and regula- tions) or in response to the perceived neglect or indifference of the majority society. These behaviors can, in turn, generate anger and fr ustration in those who deal with these indi- viduals, thus setting up a vicious cycle of mutual mistrust, which should not be confused with paranoid personality diso rder. Some ethnic groups also display culturally related be- haviors that can be misinterpreted as paranoid.
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652 Personality Disorders Differential Diagnosis Other mental disorders with psychotic symptoms. Paranoid personality disorder can be distinguished from delusional disorder, pe rsecutory type; schizophrenia; and a bipolar or depressive disorder with psychotic features be cause these disorders are all characterized by a period of persistent psychotic symptoms (e.g., delusions and hallucinations). For an additional diagnosis of paranoid personality disorder to be given, the personal ity disorder must have been present before the onset of psychotic sy mptoms and must persist when the psychotic symptoms are in remission. When an individual has another persistent mental disorder (e.g., schizophrenia) that was preceded by paranoid personality disorder, paranoid personality dis- order should also be recorded, follo wed by “premorbid” in parentheses. Personality change due to another medical condition. Paranoid personality disorder must be distinguished from personality ch ange due to another medical condition, in which the traits that emerge ar e attributable to the direct effects of another medical condi- tion on the central nervous system. Substance use disorders. Paranoid personality disorder must be distinguished from symptoms that may develop in associat ion with persistent substance use. Paranoid traits associated with physical handicaps. The disorder must also be distin- guished from paranoid traits associated with the development of physical handicaps (e.g., a hearing impairment). Other personality disorders and personality traits. Other personality disorders may be confused with paranoid person ality disorder because they ha ve certain features in common. It is therefore important to distinguish among these disorders based on differences in their characteristic features. However, if an individu al has personality features that meet criteria for one or more personality disorders in additi on to paranoid personality disorder, all can be diagnosed. Paranoid personality disorder an d schizotypal personality disorder share the
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diagnosed. Paranoid personality disorder an d schizotypal personality disorder share the traits of suspiciousness, interpersonal aloofne ss, and paranoid ideati on, but schizotypal per- sonality disorder also includes symptoms such as magical thinking, unusual perceptual ex- periences, and odd thinking and speech. Indivi duals with behaviors th at meet criteria for schizoid personality disorder ar e often perceived as strange, ec centric, cold, and aloof, but they do not usually have prominent paranoid ideation. The tendency of individuals with paranoid personality disorder to react to minor stimuli with anger is also seen in borderline and histrionic personality disorders. However, these disord ers are not necessarily associ- ated with pervasive suspiciousness. People with avoidant personality disorder may also be reluctant to confide in others, but more from fear of being embarrassed or found inadequate than from fear of others’ malicious intent. Although antisocial behavior may be present in some individuals with paranoid personality diso rder, it is not usually motivated by a desire for personal gain or to exploit others as in an tisocial personality diso rder, but rather is more often attributable to a desire for revenge. In dividuals with narcissistic personality disorder may occasionally display suspiciousness, social withdrawal, or alienation, but this derives primarily from fears of having thei r imperfections or flaws revealed. Paranoid traits may be adaptive, particul arly in threatening environments. Paranoid personality disorder should be diagnosed only when these tr aits are inflexible, maladap- tive, and persisting and cause significant functional impairment or subjective distress. Schizoid Personality Disorder Diagnostic Criteria 301.20 (F60.1) A. A pervasive pattern of detachment from social relationships and a restricted range of
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A. A pervasive pattern of detachment from social relationships and a restricted range of expression of emotions in interpers onal settings, beginning by early adulthood and
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A. A pervasive pattern of detachment from social relationships and a restricted range of expression of emotions in interpers onal settings, beginning by early adulthood and present in a variety of contexts, as indicated by four (or more) of the following:
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Schizoid Personality Disorder 653 1. Neither desires nor enjoys close relationships, including being part of a family. 2. Almost always chooses solitary activities. 3. Has little, if any, interest in having sexual experiences with another person. 4. Takes pleasure in few, if any, activities. 5. Lacks close friends or confidants other than first-degree relatives. 6. Appears indifferent to the praise or criticism of others. 7. Shows emotional coldness, detachment, or flattened affectivity. B. Does not occur exclusively during the course of schizophrenia, a bipolar disorder or depressive disorder with psychotic features, another psychotic disorder, or autism spectrum disorder and is not attributable to the physiological effects of another medical condition. Note: If criteria are met prior to the onset of schizophrenia, add “premorbid,” i.e., “schiz- oid personality disorder (premorbid).” Diagnostic Features The essential feature of schizo id personality disorder is a pe rvasive pattern of detachment from social relationships and a restricted range of expression of emotions in interpersonal settings. This pattern begins by early adulth ood and is present in a variety of contexts. Individuals with schizoid personality disord er appear to lack a desire for intimacy, seem indifferent to opportunities to develop close relationships, and do not seem to derive much satisfaction from being part of a family or other social group (Criterion A1). They prefer spending time by themselves, rather than being with other people. They often ap- pear to be socially isolated or “loners” and al most always choose solitary activities or hob- bies that do not include interaction with others (Criterion A2). They prefer mechanical or abstract tasks, such as computer or mathemat ical games. They may have very little interest
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abstract tasks, such as computer or mathemat ical games. They may have very little interest in having sexual experiences with another person (Criterion A3) and take pleasure in few, if any, activities (Criterion A4). There is usually a reduced experience of pleasure from sen- sory, bodily, or interpersonal ex periences, such as walking on a beach at sunset or having sex. These individuals have no close friends or confidants, except possibly a first-degree relative (Criterion A5). Individuals with schizoid personality disord er often seem indifferent to the approval or criticism of others and do not appear to be bothered by what others may think of them (Criterion A6). They may be oblivious to the no rmal subtleties of social interaction and of- ten do not respond appropriately to social cues so that they seem socially inept or super- ficial and self-absorbed. They usually displa y a “bland” exterior without visible emotional reactivity and rarely reciprocate gestures or facial expressions, such as smiles or nods (Cri- terion A7). They claim that they rarely expe rience strong emotions such as anger and joy. They often display a constricted affect and ap pear cold and aloof. However, in those very unusual circumstances in which these indivi duals become at least temporarily comfort- able in revealing themselves, they may ackn owledge having painful feelings, particularly related to social interactions. Schizoid personality disorder should not be diagnosed if th e pattern of behavior occurs exclusively during the course of schizophrenia , a bipolar or depressive disorder with psy- chotic features, another psychotic disorder, or au tism spectrum disorder, or if it is attrib- utable to the physiological effects of a neurological (e.g., temporal lobe epilepsy) or another medical condition (Criterion B).
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medical condition (Criterion B). Associated Features Supporting Diagnosis Individuals with schizoid personality disord er may have particular difficulty expressing anger, even in response to direct provocation, which contributes to the impression that
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654 Personality Disorders they lack emotion. Their lives sometimes se em directionless, an d they may appear to “drift” in their goals. Such individuals ofte n react passively to adverse circumstances and have difficulty responding appropriately to im portant life events. Because of their lack of social skills and lack of desire for sexual experiences, individuals with this disorder have few friendships, date infreque ntly, and often do not marry. Occupational functioning may be impaired, particularly if interpersonal involvement is required, but individuals with this disorder may do well when they work un der conditions of soci al isolation. Particu- larly in response to stress, individuals with this disorder may experience very brief psy- chotic episodes (lasting minutes to hours) . In some instances, schizoid personality disorder may appear as the pr emorbid antecedent of delusional disorder or schizophre- nia. Individuals with this disorder may so metimes develop major depressive disorder. Schizoid personality disorder most often co-occurs with schi zotypal, paranoid, and avoid- ant personality disorders. Prevalence Schizoid personality disorder is uncommon in clinical settings. A prevalence estimate for schizoid personality based on a probability su bsample from Part II of the National Co- morbidity Survey Replication suggests a pr evalence of 4.9%. Data from the 2001–2002 National Epidemiologic Survey on Alcohol and Related Conditions suggest a prevalence of 3.1%. Development and Course Schizoid personality disorder may be first apparent in childhood and adolescence with solitariness, poor peer relationships, and underachievement in school, which mark these children or adolescents as different and make them subject to teasing. Risk and Prognostic Factors Genetic and physiological. Schizoid personality disorder may have increased preva-
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Genetic and physiological. Schizoid personality disorder may have increased preva- lence in the relatives of individuals with schizophrenia or schizotypa l personality disorder. Culture-Related Diagnostic Issues Individuals from a variety of cultural bac kgrounds sometimes exhibit defensive behaviors and interpersonal styles that may be erroneously labeled as “schizoid.” For example, those who have moved from rural to metropolitan environments may react with “emotional freezing” that may last for several months and manifest as solitary activities, constricted affect, and other deficits in communication. Immigrants from other countries are some- times mistakenly perceived as cold, hostile, or indifferent. Gender-Related Diagnostic Issues Schizoid personality disorder is diagnosed slightly more often in males and may cause more impairment in them. Differential Diagnosis Other mental disorders with psychotic symptoms. Schizoid persona lity disorder can be distinguished from delusional disorder, sc hizophrenia, and a bipolar or depressive dis- order with psychotic features because these d isorders are all characterized by a period of persistent psychotic symptoms (e .g., delusions and hallucinations). To give an additional diagnosis of schizoid personality disorder, the personality disorder must have been present before the onset of psychotic symptoms an d must persist when the psychotic symptoms
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Schizotypal Personality Disorder 655 are in remission. When an individual has a pe rsistent psychotic diso rder (e.g., schizophre- nia) that was preceded by schizoid person ality disorder, schizoid personality disorder should also be recorded, followe d by “premorbid” in parentheses. Autism spectrum disorder. There may be great difficulty di fferentiating individuals with schizoid personality disorder from those with milder forms of autism spectrum disorder, which may be differentiated by more severely impaired social inte raction and stereotyped behaviors and interests. Personality change due to another medical condition. Schizoid personality disorder must be distinguished from personality ch ange due to another medical condition, in which the traits that emerge ar e attributable to the effects of another medical condition on the central nervous system. Substance use disorders. Schizoid personality disorder must also be distinguished from symptoms that may develop in a ssociation with persistent substance use. Other personality disorder s and personality traits. Other personality disorders may be confused with schizoid personality disorder because they have certain features in com- mon. It is, therefore, import ant to distinguish among these disorders based on differences in their characteristic features. However, if an individual has personality features that meet criteria for one or more personality disorders in addition to schizoid personality dis- order, all can be diagnosed. Although characteri stics of social isolation and restricted af- fectivity are common to schizoid, schizotypal, and paranoid personality disorders, schizoid personality disorder can be distin guished from schizotypa l personality disorder by the lack of cognitive and perceptual dist ortions and from paranoid personality disorder by the lack of suspiciousness and paranoid ideation. The social isolation of schizoid per-
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sonality disorder can be distinguished from th at of avoidant personality disorder, which is attributable to fear of being embarrassed or found inadequate and excessive anticipation of rejection. In contrast, people with schizoid personality disorder have a more pervasive detachment and limited desire for social in timacy. Individuals with obsessive-compulsive personality disorder may also show an apparent social detachment stemming from devo- tion to work and discomfort wi th emotions, but they do have an underlying capacity for intimacy. Individuals who are “loners” may display pe rsonality traits that might be considered schizoid. Only when these trai ts are inflexible and maladaptive and cause significant func- tional impairment or subjective distress do they constitute schizoid personality disorder. Schizotypal Personality Disorder Diagnostic Criteria 301.22 (F21) A. A pervasive pattern of social and interpersonal deficits marked by acute discomfort with, and reduced capacity for, close relationships as well as by cognitive or perceptual distortions and eccentricities of behavior, beginning by early adulthood and present in a variety of contexts, as indicated by five (or more) of the following: 1. Ideas of reference (excluding delusions of reference). 2. Odd beliefs or magical thinking that influences behavior and is inconsistent with subcultural norms (e.g., superstitiousness, belief in clairvoyance, telepathy, or “sixth sense”; in children and adolescents, bizarre fantasies or preoccupations). 3. Unusual perceptual experiences, including bodily illusions. 4. Odd thinking and speech (e.g., vague, circumstantial, metaphorical, overelaborate, or stereotyped). 5. Suspiciousness or paranoid ideation.
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656 Personality Disorders 6. Inappropriate or constricted affect. 7. Behavior or appearance that is odd, eccentric, or peculiar. 8. Lack of close friends or confidants other than first-degree relatives. 9. Excessive social anxiety that does not diminish with familiarity and tends to be as- sociated with paranoid fears rather than negative judgments about self. B. Does not occur exclusively during the course of schizophrenia, a bipolar disorder or depressive disorder with psychotic features, another psychotic disorder, or autism spectrum disorder. Note: If criteria are met prior to the onset of schizophrenia, add “premorbid,” e.g., “schizo- typal personality disorder (premorbid).” Diagnostic Features The essential feature of schizo typal personality disorder is a pervasive pattern of social and interpersonal deficits ma rked by acute discomfort with, and reduced capacity for, close relationships as well as by cognitive or pe rceptual distortions and eccentricities of be- havior. This pattern begins by early adulthoo d and is present in a variety of contexts. Individuals with schizotypal personality disord er often have ideas of reference (i.e., in- correct interpretations of casual incidents and external events as having a particular and unusual meaning specifically for the person ) (Criterion A1). Th ese should be distin- guished from delusions of reference, in whic h the beliefs are held with delusional convic- tion. These individuals may be superstitiou s or preoccupied with paranormal phenomena that are outside the norms of their subculture (Criterion A2). They may feel that they have special powers to sense events before they happen or to read others’ thoughts. They may believe that they have magical control over others, which can be implemented directly
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believe that they have magical control over others, which can be implemented directly (e.g., believing that their spouse’s taking the do g out for a walk is the direct result of think- ing an hour earlier it should be done) or in directly through compli ance with magical rit- uals (e.g., walking past a specific object three times to avoid a certain harmful outcome). Perceptual alterations may be present (e.g., se nsing that another person is present or hear- ing a voice murmuring his or her name) (Criterion A3). Their speech may include unusual or idiosyncratic phrasing and construction. It is often loose, digressive, or vague, but with- out actual derailment or incoherence (Criteri on A4). Responses can be either overly con- crete or overly abstract, and words or conc epts are sometimes applied in unusual ways (e.g., the individual may state that he or she was not “talkable” at work). Individuals with this disorder are often suspicious and may have paranoid ideation (e.g., believing their colleagues at work ar e intent on undermining their reputation with the boss) (Criterion A5). They are usually not able to negotiate the fu ll range of affects and interpersonal cuing required fo r successful relationships and thus often appear to interact with others in an inappropriate, stiff, or constricted fashion (Criterion A6). These individ- uals are often considered to be odd or ecce ntric because of unusual mannerisms, an often unkempt manner of dress that does not quite “fit together,” and inattention to the usual social conventions (e.g., the in dividual may avoid eye contact, wear clothes that are ink stained and ill-fitting, and be unable to join in the give-and-take banter of co-workers)
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(Criterion A7). Individuals with schizotypal personality di sorder experience in terpersonal related- ness as problematic and are uncomfortable relating to other people. Although they may express unhappiness about their lack of relationships, their behavior suggests a decreased desire for intimate contacts. As a result, they usually have no or few close friends or con- fidants other than a first-degree relative (Cri terion A8). They are anxious in social situa- tions, particularly those invo lving unfamiliar people (Crite rion A9). They will interact
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fidants other than a first-degree relative (Cri terion A8). They are anxious in social situa- tions, particularly those invo lving unfamiliar people (Crite rion A9). They will interact with other individuals when they have to but prefer to keep to themselves because they feel that they are different and just do not “fit in.” Their social anxiety does not easily abate,
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Schizotypal Personality Disorder 657 even when they spend more time in the setti ng or become more fa miliar with the other people, because their anxiety tends to be assoc iated with suspiciousness regarding others’ motivations. For example, when attending a dinner party, the individual with schizotypal personality disorder will not become more rela xed as time goes on, but rather may become increasingly tense and suspicious. Schizotypal personality disorder should not be diagnosed if the pattern of behavior oc- curs exclusively during the course of schizo phrenia, a bipolar or de pressive disorder with psychotic features, another psychotic disorder, or autism spectrum disorder (Criterion B). Associated Features Supporting Diagnosis Individuals with schizotypal personality diso rder often seek treatment for the associated symptoms of anxiety or depression rather than for the personality disorder features per se. Particularly in response to stress, individual s with this disorder may experience transient psychotic episodes (lasting minutes to hours), although they usually are insufficient in du- ration to warrant an additional diagnosis such as brief psychotic disorder or schizophreni- form disorder. In some cases, clinically sig nificant psychotic symptoms may develop that meet criteria for brief psychotic disorder, sc hizophreniform disorder, delusional disorder, or schizophrenia. Over half may have a hist ory of at least one ma jor depressive episode. From 30% to 50% of individuals diagnosed with this disorder have a concurrent diagnosis of major depressive disorder when admitted to a clinical setting. There is considerable co- occurrence with schizoid, paranoid, avoi dant, and borderline personality disorders. Prevalence In community studies of schizotypal personality disorder, reported rates range from 0.6%
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in Norwegian samples to 4.6% in a U.S. commu nity sample. The prevalence of schizotypal personality disorder in clinical populations seems to be infrequent (0%–1.9%), with a higher estimated prevalence in the general population (3.9%) found in the National Epi- demiologic Survey on Alco hol and Related Conditions. Development and Course Schizotypal personality disorder has a relative ly stable course, with only a small propor- tion of individuals going on to develop sc hizophrenia or another psychotic disorder. Schizotypal personality disorder may be firs t apparent in childhood and adolescence with solitariness, poor peer relationships, social anxiety, underachievement in school, hyper- sensitivity, peculiar thoughts and language, an d bizarre fantasies. These children may ap- pear “odd” or “eccentric” and attract teasing. Risk and Prognostic Factors Genetic and physiological. Schizotypal personality disord er appears to aggregate fa- milially and is more prevalent among the firs t-degree biological relatives of individuals with schizophrenia than among the general po pulation. There may also be a modest in- crease in schizophrenia and other psychotic disorders in the relatives of probands with schizotypal personality disorder. Cultural-Related Diagnostic Issues Cognitive and perceptual distortions must be evaluated in the context of the individual’s cultural milieu. Pervasive cultur ally determined characteristic s, particularly those regard- ing religious beliefs and rituals, can appear to be schizotypal to the uninformed outsider (e.g., voodoo, speaking in tongues, life beyo nd death, shamanism, mind reading, sixth sense, evil eye, magical beliefs related to health and illness).
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658 Personality Disorders Gender-Related Diagnostic Issues Schizotypal personality disorder may be slightly more common in males. Differential Diagnosis Other mental disorders with psychotic symptoms. Schizotypal personality disorder can be distinguished from de lusional disorder, schizophrenia , and a bipolar or depressive disorder with psychotic features because thes e disorders are all characterized by a period of persistent psychotic symptoms (e.g., delu sions and hallucinations). To give an addi- tional diagnosis of schizotypal personality disorder, the personality disorder must have been present before the onset of psychoti c symptoms and persis t when the psychotic symptoms are in remission. Wh en an individual has a persist ent psychotic disorder (e.g., schizophrenia) that was preceded by schizo typal personality disorder, schizotypal per- sonality disorder should also be recorded, followed by “premorbid” in parentheses. Neurodevelopmental disorders. There may be great difficulty differentiating children with schizotypal personality disorder from the heterogeneous group of solitary, odd chil- dren whose behavior is characterized by mark ed social isolation, eccentricity, or peculiar- ities of language and whose diagnoses woul d probably include milder forms of autism spectrum disorder or language communicat ion disorders. Commun ication disorders may be differentiated by the primacy and severity of the disorder in language and by the char- acteristic features of impaired language found in a specialized language assessment. Milder forms of autism spectrum disorder are differentiated by the even greater lack of so- cial awareness and emotional reciprocity and stereotyped behaviors and interests. Personality change due to another medical condition. Schizotypal personality disor- der must be distinguished from personality change due to another medical condition, in
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der must be distinguished from personality change due to another medical condition, in which the traits that emerge ar e attributable to the effects of another medical condition on the central nervous system. Substance use disorders. Schizotypal personality disorder must also be distinguished from symptoms that may develop in a ssociation with persistent substance use. Other personality disorders and personality traits. Other personality disorders may be confused with schizotypal personality diso rder because they have certain features in common. It is, therefore, important to distin guish among these disorders based on differ- ences in their characteristic features. However, if an individual has personality features that meet criteria for one or more personality disorders in addition to schizotypal person- ality disorder, all can be diagnosed. Although paranoid and schizoid personality disor- ders may also be characterized by social de tachment and restricted affect, schizotypal personality disorder can be distinguished fr om these two diagnoses by the presence of cognitive or perceptual distortions and marked eccentricity or oddn ess. Close relation- ships are limited in both schizotypal person ality disorder and avoidant personality dis- order; however, in avoidant personality disorder an active desire for relationships is constrained by a fear of rejection, whereas in schizotypal personality disorder there is a lack of desire for relationships and persis tent detachment. Indivi duals with narcissistic personality disorder may also display suspic iousness, social withdrawal, or alienation, but in narcissistic personality disorder these qualities derive primarily from fears of hav- ing imperfections or flaws revealed. Individu als with borderline personality disorder may also have transient, psychotic-like symptoms, but these are usually more closely related to
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also have transient, psychotic-like symptoms, but these are usually more closely related to affective shifts in response to stress (e.g., intense anger, anxiety, disappointment) and are usually more dissociative (e.g., derealization, depersonalization). In contrast, individuals with schizotypal personality disorder are more likely to have enduring psychotic-like symp-
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usually more dissociative (e.g., derealization, depersonalization). In contrast, individuals with schizotypal personality disorder are more likely to have enduring psychotic-like symp- toms that may worsen under stress but are less likely to be invariably associated with pro- nounced affective symptoms. Although social isolation may occur in borderline personality
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Antisocial Personality Disorder 659 disorder, it is usually secondary to repeated interpersonal failures due to angry outbursts and frequent mood shifts, rather than a result of a persistent lack of social contacts and de- sire for intimacy. Furt hermore, individuals with schizoty pal personality disorder do not usually demonstrate the impulsiv e or manipulative behaviors of the individual with bor- derline personality diso rder. However, there is a high ra te of co-occurren ce between the two disorders, so that making such distinctions is not always feasible. Schizotypal features during adolescence may be reflective of transi ent emotional turmoil, rather than an endur- ing personality disorder. Cluster B Personality Disorders Antisocial Personality Disorder Diagnostic Criteria 301.7 (F60.2) A. A pervasive pattern of disregard for and violation of the rights of others, occurring since age 15 years, as indicated by three (or more) of the following: 1. Failure to conform to social norms with respect to lawful behaviors, as indicated by repeatedly performing acts that are grounds for arrest. 2. Deceitfulness, as indicated by repeated lying, use of aliases, or conning others for personal profit or pleasure. 3. Impulsivity or failure to plan ahead. 4. Irritability and aggressiveness, as indicated by repeated physical fights or assaults. 5. Reckless disregard for safety of self or others. 6. Consistent irresponsibility, as indicated by repeated failure to sustain consistent work behavior or honor financial obligations. 7. Lack of remorse, as indicated by being indifferent to or rationalizing having hurt, mistreated, or stolen from another. B. The individual is at least age 18 years.
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B. The individual is at least age 18 years. C. There is evidence of conduct disorder with onset before age 15 years. D. The occurrence of antisocial behavior is not exclusively during the course of schizo- phrenia or bipolar disorder. Diagnostic Features The essential feature of antisocial personality disorder is a pervasive pattern of disregard for, and violation of, the rights of others that begins in childhood or early adolescence and continues into adulthood. This pattern has also been referred to as psychopathy, sociopathy, or dyssocial personality disorder. Because deceit and manipulation are central features of an- tisocial personality disorder, it may be especially helpful to integrate information acquired from systematic clinical assessment with information collected from collateral sources. For this diagnosis to be given, the individual must be at least age 18 years (Criterion B) and must have had a history of some symptoms of conduct disorder before age 15 years (Criterion C). Conduct disorder involves a repe titive and persistent pattern of behavior in which the basic rights of othe rs or major age-appropriate societal norms or rules are vio- lated. The specific behaviors characteristic of conduct disorder fall into one of four cate- gories: aggression to people and animals, destruction of proper ty, deceitfulness or theft, or serious violation of rules.
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660 Personality Disorders The pattern of antisocial behavior continues into adulthood. Indi viduals with antiso- cial personality disorder fail to conform to social norms with respect to lawful behavior (Criterion A1). They may repeatedly perfor m acts that are grounds for arrest (whether they are arrested or not), such as destroying property, harassing ot hers, stealing, or pur- suing illegal occupations. Persons with this di sorder disregard the wishes, rights, or feel- ings of others. They are freq uently deceitful and manipulative in order to gain personal profit or pleasure (e.g., to obtain money, sex, or power) (Criterion A2). They may repeat- edly lie, use an alias, con others, or malinger. A pattern of impulsiv ity may be manifested by a failure to plan ahead (Criterion A3). Decisions are made on the spur of the moment, without forethought and without consideration fo r the consequences to self or others; this may lead to sudden changes of jobs, residences , or relationships. Individuals with antiso- cial personality disorder tend to be irrita ble and aggressive and may repeatedly get into physical fights or commit acts of physical assau lt (including spouse beating or child beat- ing) (Criterion A4). (Aggressive acts that are required to defend oneself or someone else are not considered to be evidence for this it em.) These individuals also display a reckless disregard for the safety of themselves or othe rs (Criterion A5). This may be evidenced in their driving behavior (i.e., recurrent speedi ng, driving while intoxicated, multiple acci- dents). They may engage in sexual behavior or substance use that has a high risk for harm- ful consequences. They may neglec t or fail to care for a child in a way that puts the child in danger.
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danger. Individuals with antisocial personality disord er also tend to be consistently and ex- tremely irresponsible (Criterion A6). Irrespon sible work behavior may be indicated by sig- nificant periods of unemployme nt despite available job opportunities, or by abandonment of several jobs without a realistic plan for ge tting another job. There may also be a pattern of repeated absences fr om work that are not explained by illness either in themselves or in their family. Financial irresponsibility is indicated by acts such as defaulting on debts, fail- ing to provide child support, or failing to support other dependents on a regular basis. In- dividuals with antisocial personality disorder show little remorse for the consequences of their acts (Criterion A7). They may be indifferent to, or provide a superficial rationaliza- tion for, having hurt, mistreated, or stolen from someone (e.g., “life’s unfair,” “losers de- serve to lose”). These individuals may blame the victims for being foolish, helpless, or deserving their fate (e.g., “he had it coming anyway”); they may minimize the harmful consequences of their actions; or they may simply indicate complete indifference. They generally fail to compensate or make amends for their behavior. They may believe that everyone is out to “help number one” and that one should stop at nothing to avoid being pushed around. The antisocial behavior must not occur excl usively during the course of schizophrenia or bipolar disorder (Criterion D). Associated Features Supporting Diagnosis Individuals with antisocial personality disord er frequently lack empathy and tend to be callous, cynical, and contempt uous of the feelings, rights, and sufferings of others. They
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may have an inflated and arrogant self-appraisa l (e.g., feel that ordinary work is beneath them or lack a realistic concern about their current problems or th eir future) and may be excessively opinionated, self-assured, or coc ky. They may display a glib, superficial charm and can be quite voluble and verbally facile (e.g., using technical terms or jargon that might impress someone who is unfamiliar with th e topic). Lack of empathy, inflated self-
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and can be quite voluble and verbally facile (e.g., using technical terms or jargon that might impress someone who is unfamiliar with th e topic). Lack of empathy, inflated self- appraisal, and superficial charm are features that have been commonly included in tradi- tional conceptions of psychopathy that may be particularly distinguishing of the disorder and more predictive of recidivism in prison or forensic settings, where criminal, delin- quent, or aggressive acts are likely to be no nspecific. These individuals may also be irre- sponsible and exploitative in their sexual re lationships. They may have a history of many
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Antisocial Personality Disorder 661 sexual partners and may never have sustained a monogamous relationship. They may be irresponsible as parents, as evidenced by maln utrition of a child, an illness in the child re- sulting from a lack of minimal hygiene, a ch ild’s dependence on neighbors or nonresident relatives for food or shelter, a failure to arrange for a caretaker for a young child when the individual is away from home, or repeated squandering of money required for household necessities. These individuals may receive di shonorable discharges from the armed ser- vices, may fail to be self-supporting, may be come impoverished or even homeless, or may spend many years in penal institutions. Indi viduals with antisocial personality disorder are more likely than people in the general po pulation to die prematurely by violent means (e.g., suicide, accidents, homicides). Individuals with antisocial personality disorder may also experience dysphoria, in- cluding complaints of tension, inability to tolerate boredom, and depressed mood. They may have associated anxiety disorders, depressive disorders, substa nce use disorders, so- matic symptom disorder, gambli ng disorder, and other disord ers of impulse control. In- dividuals with antisocial personality disorder also often have personality features that meet criteria for other personality disorders, particularly borderline, histrionic, and nar- cissistic personality disorders. The likelihood of developing antisocial personality disor- der in adult life is increased if the indivi dual experienced childhood onset of conduct disorder (before age 10 years) and accompanyi ng attention-deficit/hyperactivity disorder. Child abuse or neglect, unstable or erratic parenting, or inconsistent parental discipline may increase the likelihood that conduct disord er will evolve into antisocial personality disorder.
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may increase the likelihood that conduct disord er will evolve into antisocial personality disorder. Prevalence Twelve-month prevalence rates of antisocial personality diso rder, using criteria from pre- vious DSMs, are between 0.2% and 3.3%. The highest prevalence of antisocial personality disorder (greater than 70%) is among most se vere samples of males with alcohol use dis- order and from substance abuse clinics, prison s, or other forensic settings. Prevalence is higher in samples affected by adverse socioeconomic (i.e., poverty) or sociocultural (i.e., migration) factors. Development and Course Antisocial personality disorder has a chronic course but may become less evident or remit as the individual grows older, particularly by the fourth deca de of life. Although this re- mission tends to be particularly evident with respect to engaging in criminal behavior, there is likely to be a decrease in the full spectrum of antisocial behaviors and substance use. By definition, antisocial personalit y cannot be diagnosed before age 18 years. Risk and Prognostic Factors Genetic and physiological. Antisocial personality disorder is more common among the first-degree biological relatives of those with the disorder than in the general population. The risk to biological relatives of females with the disorder tends to be higher than the risk to biological relatives of males with the disorder. Biological relatives of individuals with this disorder are also at in creased risk for somatic symptom disorder and substance use disorders. Within a family that has a member with antisocial personality disorder, males more often have antisocial personality disord er and substance use disorders, whereas fe- males more often have somatic symptom disorder. However, in such families, there is an
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males more often have somatic symptom disorder. However, in such families, there is an increase in prevalence of al l of these disorders in both males and females compared with the general population. Adoption studies indi cate that both genetic and environmental factors contribute to the risk of developing antisocial personality disorder. Both adopted and biological children of parents with antisocial personality disorder have an increased
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662 Personality Disorders risk of developing antisocial personality disorder, somatic symptom disorder, and sub- stance use disorders. Adopted-away children resemble their biological parents more than their adoptive parents, but the adoptive family environm ent influences the risk of devel- oping a personality disorder and related psychopathology. Culture-Related Diagnostic Issues Antisocial personality disorder appears to be associated with low socioeconomic status and urban settings. Concerns have been raised that the diagnosis may at times be misap- plied to individuals in settings in which seemingly antisocial behavior may be part of a protective survival stra tegy. In assessing antisocial traits, it is helpful for the clinician to consider the social and economic co ntext in which the behaviors occur. Gender-Related Diagnostic Issues Antisocial personality disorder is much more common in males than in females. There has been some concern that antisocial persona lity disorder may be underdiagnosed in fe- males, particularly because of the emphasis on aggressive items in the definition of con- duct disorder. Differential Diagnosis The diagnosis of antisocial personality disorder is not given to individuals younger than 18 years and is given only if there is a histor y of some symptoms of conduct disorder be- fore age 15 years. For individuals older than 18 years, a diagnosis of conduct disorder is given only if the criteria for antiso cial personality disorder are not met. Substance use disorders. When antisocial behavior in an adult is associated with a substance use disorder, the diagnosis of antisoci al personality disorder is not made unless the signs of antisocial personality disorder were also present in childhood and have con- tinued into adulthood. When substance use and antisocial behavior both began in childhood and continued into adulthood, both a substance use disorder and antisocial personality
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and continued into adulthood, both a substance use disorder and antisocial personality disorder should be diagnosed if the criteria fo r both are met, even though some antisocial acts may be a consequence of the substance us e disorder (e.g., illegal selling of drugs, thefts to obtain money for drugs). Schizophrenia and bipolar disorders. Antisocial behavior that occurs exclusively dur- ing the course of schizophrenia or a bipolar disorder should not be diagnosed as antisocial personality disorder. Other personality disorders. Other personality disorders may be confused with antiso- cial personality disorder because they have certain features in common. It is therefore im- portant to distinguish among these disorders based on differences in their characteristic features. However, if an indivi dual has personality features that meet criteria for one or more personality disorders in addition to antisocial personality disorder, all can be diag- nosed. Individuals with antisocial personalit y disorder and narcissistic personality disor- der share a tendency to be tough-minded, glib , superficial, exploitative, and lack empathy. However, narcissistic personality disorder does not include ch aracteristics of impulsivity, aggression, and deceit. In addition, individuals with antisocial personality disorder may not be as needy of the admiration and envy of others, and persons with narcissistic per- sonality disorder usually lack the history of conduct disorder in childhood or criminal behavior in adulthood. Indivi duals with antisocial personality disorder and histrionic personality disorder share a tendency to be impulsive, superficial, excitement seeking, reckless, seductive, and manipulative, but pe rsons with histrionic personality disorder tend to be more exaggerated in their emotions and do not characteristically engage in an- tisocial behaviors. Individuals with histrion ic and borderline pers onality disorders are
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Borderline Personality Disorder 663 manipulative to gain nurturance, whereas thos e with antisocial personality disorder are manipulative to gain profit, po wer, or some other material gratification. Individuals with antisocial personality disorder tend to be less emotionally unstable and more aggressive than those with borderline personality disorder. Although antisocial behavior may be present in some individuals with paranoid pe rsonality disorder, it is not usually moti- vated by a desire for personal gain or to exploit others as in antisocial personality disorder, but rather is more often attributable to a desire for revenge. Criminal behavior not associated with a personality disorder. Antisocial personality disorder must be distinguished from criminal behavior undertaken for gain that is not ac- companied by the personality features characteristic of this disorder. Only when antisocial personality traits are inflexible, maladaptive, and persistent and cause significant func- tional impairment or subjective distress do th ey constitute antisocial personality disorder. Borderline Personality Disorder Diagnostic Criteria 301.83 (F60.3) A pervasive pattern of instability of interpersonal relationships, self-image, and affects, and marked impulsivity, beginning by early adulthood and present in a variety of contexts, as indicated by five (or more) of the following: 1. Frantic efforts to avoid real or imagined abandonment. ( Note: Do not include suicidal or self-mutilating behavior covered in Criterion 5.) 2. A pattern of unstable and intense interpersonal relationships characterized by alternat- ing between extremes of idealization and devaluation. 3. Identity disturbance: markedly and persistently unstable self-image or sense of self. 4. Impulsivity in at least two areas that are potentially self-damaging (e.g., spending, sex,
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substance abuse, reckless driving, binge eating). ( Note: Do not include suicidal or self- mutilating behavior covered in Criterion 5.) 5. Recurrent suicidal behavior, gestures, or threats, or self-mutilating behavior. 6. Affective instability due to a marked reactivity of mood (e.g., intense episodic dysphoria, irritability, or anxiety usually lasting a few hours and only rarely more than a few days). 7. Chronic feelings of emptiness. 8. Inappropriate, intense anger or difficulty controlling anger (e.g., frequent displays of temper, constant anger, recurrent physical fights). 9. Transient, stress-related paranoid ideation or severe dissociative symptoms. Diagnostic Features The essential feature of borderline personality disorder is a pervasive pattern of instability of interpersonal relationships, self-image, an d affects, and marked impulsivity that begins by early adulthood and is present in a variety of contexts. Individuals with borderline pe rsonality disorder make fran tic efforts to avoid real or imagined abandonment (Criterion 1). The perception of impending separation or rejection, or the loss of external structure, can lead to profound changes in sel f-image, affect, cognition, and behavior. These individuals are very sensitive to environmental circumstances. They ex- perience intense abandonment fears and inappropriate anger even when faced with a real- istic time-limited separation or when there are unavoidable changes in plans (e.g., sudden despair in reaction to a clinician’s announcing the end of the hour; panic or fury when some- one important to them is just a few minutes la te or must cancel an appointment). They may believe that this “abandonment” implies they are “bad.” These abandonment fears are re- lated to an intolerance of being alone and a need to have other people with them. Their frantic
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664 Personality Disorders efforts to avoid abandonment may include impulsive actions such as self-mutilating or sui- cidal behaviors, which are described separately in Criterion 5. Individuals with borderline personality disord er have a pattern of unstable and intense relationships (Criterion 2). They may idealize potential caregivers or lovers at the first or second meeting, demand to spend a lot of time together, and share the most intimate details early in a relationship. Howeve r, they may switch quickly from idealizing other people to devaluing them, feeling that the other person does not care enough, does not give enough, or is not “there” enough. These individuals can empathize with and nurture other people, but only with the expectation th at the other person will “be ther e” in return to meet their own needs on demand. These indi viduals are prone to sudden and dramatic shifts in their view of others, who may alternatively be seen as beneficent supports or as cruelly punitive. Such shifts often reflect disillusionment with a caregiver whose nurturing qualities had been idealized or whose rejectio n or abandonment is expected. There may be an identity disturbance charac terized by markedly and persistently un- stable self-image or sense of self (Criterion 3). There are sudden and dramatic shifts in self- image, characterized by shifting goals, values, and vocational aspirations. There may be sudden changes in opinions and plans about ca reer, sexual identity, values, and types of friends. These individuals may suddenly chan ge from the role of a needy supplicant for help to that of a righteous av enger of past mistreat ment. Although they usually have a self- image that is based on being bad or evil, individuals with this disorder may at times have
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image that is based on being bad or evil, individuals with this disorder may at times have feelings that they do not exist at all. Such experiences usually occur in situations in which the individual feels a lack of a meaningful relationship, nurturing, and support. These in- dividuals may show worse performance in unstructured work or school situations. Individuals with borderline pe rsonality disorder display impulsivity in at least two areas that are potentially self-damaging (Criterion 4). They may gamble, spend money irrespon- sibly, binge eat, abuse substanc es, engage in unsafe sex, or drive recklessly. Individuals with this disorder display recurrent suicidal behavior, gestures, or threats, or self-mutilat- ing behavior (Criterion 5). Completed suicide occurs in 8%–10% of such individuals, and self-mutilative acts (e.g., cutting or burnin g) and suicide threats and attempts are very common. Recurrent suicidality is often the reason that these individuals present for help. These self-destructive acts are usually precipit ated by threats of separation or rejection or by expectations that the individual assumes increased responsibility. Self-mutilation may occur during dissociative experiences and often brings relief by reaffirming the ability to feel or by expiating the individual’s sense of being evil. Individuals with borderline personality disorder may display affective instability that is due to a marked reactivity of mood (e.g., intense episodic dysphoria, irritability, or anx- iety usually lasting a few hours and only rarely more than a few days) (Criterion 6). The basic dysphoric mood of those with borderline personality disorder is often disrupted by periods of anger, panic, or de spair and is rarely relieved by periods of well-being or satis-
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faction. These episodes may reflect the indivi dual’s extreme reactivity to interpersonal stresses. Individuals with borderline personalit y disorder may be troubled by chronic feel- ings of emptiness (Criterion 7). Easily bored, they may constantly seek something to do. Individuals with this disorder frequently expr ess inappropriate, intense anger or have dif- ficulty controlling their anger (Criterion 8) . They may display extreme sarcasm, enduring
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Individuals with this disorder frequently expr ess inappropriate, intense anger or have dif- ficulty controlling their anger (Criterion 8) . They may display extreme sarcasm, enduring bitterness, or verbal outbursts. The anger is often elicited when a caregiver or lover is seen as neglectful, withholding, uncaring, or abandoning. Such expressions of anger are often followed by shame and guilt and contribute to the feeling they have of being evil. During periods of extreme stress , transient paranoid ideation or dissociative symptoms (e.g., de- personalization) may occur (Criterion 9), but th ese are generally of insufficient severity or duration to warrant an addition al diagnosis. These episodes occur most frequently in re- sponse to a real or imagined abandonment. Symptoms tend to be transient, lasting min- utes or hours. The real or perceived return of the caregiver’s nurturance may result in a remission of symptoms.
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Borderline Personality Disorder 665 Associated Features Supporting Diagnosis Individuals with borderline personality disorder may have a pattern of undermining themselves at the moment a goal is about to be realized (e.g., dropping out of school just before graduation; regressing se verely after a discussion of ho w well therapy is going; de- stroying a good relationship just when it is clear that the relationship could last). Some in- dividuals develop psychotic-like symptoms (e.g ., hallucinations, body-image distortions, ideas of reference, hypnagogic phenomena) du ring times of stress. Individuals with this disorder may feel more secure with transition al objects (i.e., a pet or inanimate possession) than in interpersonal relationships. Prematur e death from suicide may occur in individu- als with this disorder, especially in those with co-occurring depressive disorders or sub- stance use disorders. Physical handicaps may result from self-inflicted abuse behaviors or failed suicide attempts. Recurrent job losses, in terrupted education, and separation or di- vorce are common. Physical and sexual abuse, neglect, hostile conflict, and early parental loss are more common in the childhood histor ies of those with borderline personality dis- order. Common co-occurring disorders include depressive and bipolar disorders, sub- stance use disorders, eating disorders (not ably bulimia nervosa), posttraumatic stress disorder, and attention-defici t/hyperactivity disorder. Bo rderline personality disorder also frequently co-occurs with the other personality disorders. Prevalence The median population prevalence of border line personality disorder is estimated to be 1.6% but may be as high as 5.9%. The preval ence of borderline personality disorder is
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about 6% in primary care sett ings, about 10% among individual s seen in outpatient mental health clinics, and about 20% among psychiat ric inpatients. The prevalence of borderline personality disorder may decrease in older age groups. Development and Course There is considerable variability in the course of borderline personality disorder. The most common pattern is one of chronic instability in early adulthoo d, with episodes of serious affective and impulsive dyscontrol and high levels of use of health and mental health re- sources. The impairment from the disorder and the risk of suicide are greatest in the young-adult years and gradually wane with advancing age. Although the tendency to- ward intense emotions, impulsivity, and intens ity in relationships is often lifelong, indi- viduals who engage in therapeutic interv ention often show improvement beginning sometime during the first year. During their 30s and 40s, the majority of individuals with this disorder attain greater stability in their relationships and vocational functioning. Fol- low-up studies of individuals identified thr ough outpatient mental health clinics indicate that after about 10 years, as many as half of the individuals no longer have a pattern of be- havior that meets full criteria for borderline personality disorder. Risk and Prognostic Factors Genetic and physiological. Borderline personality disorder is about five times more common among first-degree biological relatives of those with the disorder than in the gen- eral population. There is also an increased familial risk for substance use disorders, anti- social personality disorder, and depressive or bipolar disorders. Culture-Related Diagnostic Issues The pattern of behavior seen in borderline personality disorder has been identified in many settings around the world. Adolescents and youn g adults with identity problems (especially when accompanied by substance use) may transiently display behaviors that misleadingly
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666 Personality Disorders give the impression of borderline personality disorder. Such situations are characterized by emotional instability, “existential” dilemmas, uncertainty, anxiety- provoking choices, con- flicts about sexual orientation, and competing social pressures to decide on careers. Gender-Related Diagnostic Issues Borderline personality disord er is diagnosed predominantly (about 75%) in females. Differential Diagnosis Depressive and bipolar disorders. Borderline personality disorder often co-occurs with depressive or bipolar disorders, and when criteria for both are met, both may be diagnosed. Because the cross-sectional presentation of bo rderline personality disorder can be mimicked by an episode of depressive or bipolar disord er, the clinician should avoid giving an addi- tional diagnosis of borderline personality diso rder based only on cr oss-sectional presenta- tion without having documented that the pattern of behavior had an early onset and a long- standing course. Other personality disorders. Other personality disorders ma y be confused with border- line personality disorder because they have certain features in common. It is therefore im- portant to distinguish among these disorders based on differences in their characteristic features. However, if an individual has person ality features that meet criteria for one or more personality disorders in addition to bord erline personality disorder, all can be diag- nosed. Although histrionic personality disorder can also be characterized by attention seek- ing, manipulative behavior, an d rapidly shifting emotions, borderline personality disorder is distinguished by self-destructiveness, an gry disruptions in close relationships, and chronic feelings of deep emptiness and loneline ss. Paranoid ideas or illusions may be pres- ent in both borderline personality disorder an d schizotypal personality disorder, but these symptoms are more transient, in terpersonally reactive, and responsive to external structur-
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