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posed. However, consistent co-occurrence in twins appears only in severe cases. Course modifiers. Individuals with ADHD and with developmental coordination dis- order demonstrate more impair ment than individuals with ADHD without developmen- tal coordination disorder. Culture-Related Diagnostic Issues Developmental coordination disorder occurs across cultures, races, and socioeconomic conditions. By definition, “acti vities of daily living” implie s cultural differences necessi- tating consideration of the context in whic h the individual child is living as well as whether he or she has had appropriate opportun ities to learn and practice such activities. Functional Consequences of Developmental Coordination Disorder Developmental coordination disorder leads to impaired functional performance in activ- ities of daily living (Criterion B), and the impairment is increased with co-occurring con- ditions. Consequences of developmental coordination disorder include reduced participation in team play and sports; poor self-esteem and sense of self-worth ; emotional or behavior problems; impair ed academic achievement; p oor physical fitness; and re- duced physical activity and obesity. Differential Diagnosis Motor impairments due to another medical condition. Problems in coordination may be associated with visual function impairme nt and specific neurological disorders (e.g., cerebral palsy, progressive lesions of the cerebellum, neuromuscula r disorders). In such cases, there are additional findin gs on neurological examination. Intellectual disability (intell ectual developmental disorder). If intellectual disability is present, motor competences may be impaired in accordance with the intellectual disabil-
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Stereotypic Movement Disorder 77 ity. However, if the motor difficulties are in excess of what could be accounted for by the intellectual disability, and crit eria for developmental coordination disorder are met, de- velopmental coordination disorder can be diagnosed as well. Attention-deficit/hyperactivity disorder. Individuals with ADHD may fall, bump into objects, or knock things over. Careful observat ion across different contexts is required to ascertain if lack of motor competence is at tributable to distract ibility and impulsiveness rather than to developmental coordination disorder. If criteria for both ADHD and devel- opmental coordination disorder are met, both diagnoses can be given. Autism spectrum disorder. Individuals with autism spectrum disorder may be uninter- ested in participating in task s requiring complex coordination skills, such as ball sports, which will affect test performance and function but not reflect core motor competence. Co- occurrence of developmental coordination disorder and autism spectr um disorder is com- mon. If criteria for both disorders are met, both diagnoses can be given. Joint hypermobility syndrome. Individuals with syndrome s causing hyperextensible joints (found on ph ysical examination; often with a complaint of pain) may present with symptoms similar to those of deve lopmental coordination disorder. Comorbidity Disorders that commonly co-occur with deve lopmental coordination disorder include speech and language disorder; specific learning disorder (especially reading and writing); problems of inattention, incl uding ADHD (the most frequent coexisting condition, with about 50% co-occurrence); autism spectrum disord er; disruptive and emotional behavior problems; and joint hypermobility syndrome. Different clusters of co-occurrence may be present (e.g., a cluster with severe reading di sorders, fine motor problems, and handwriting
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problems; another cluster with impaired movement control and motor planning). Presence of other disorders does not exclude develo pmental coordination disorder but may make testing more difficult and may independently interfere with th e execution of activities of daily living, thus requiring examiner judgment in ascribing impairment to motor skills. Stereotypic Movement Disorder Diagnostic Criteria 307.3 (F98.4) A. Repetitive, seemingly driven, and apparently purposeless motor behavior (e.g., hand shaking or waving, body rocking, head banging, self-biting, hitting own body). B. The repetitive motor behavior interferes wit h social, academic, or other activities and may result in self-injury. C. Onset is in the early developmental period. D. The repetitive motor behavior is not attributable to the physiological effects of a sub- stance or neurological condition and is no t better explained by another neurodevel- opmental or mental disorder (e.g., trichotillomania [hair-pulling disorder], obsessive- compulsive disorder). Specify if: With self-injurious behavior (or behavior that would result in an injury if preventive measures were not used) Without self-injurious behavior Specify if: Associated with a known medical or genetic condition, neurodevelopmental dis- order, or environmental factor (e.g., Lesch-Nyhan syndrome, intellectual disability [intellectual developmental disorder], intrauterine alcohol exposure)
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78 Neurodevelopmental Disorders Coding note: Use additional code to identify the associated medical or genetic condition, or neurodevelopmental disorder. Specify current severity: Mild: Symptoms are easily suppressed by sensory stimulus or distraction. Moderate: Symptoms require explicit protective measures and behavioral modification. Severe: Continuous monitoring and protective m easures are required to prevent seri- ous injury. Recording Procedures For stereotypic movement diso rder that is associated with a known medical or genetic condition, neurodevelopmental disorder, or environmental factor, record stereotypic movement disorder associated with (name of condition, disorder, or factor) (e.g., stereo- typic movement disorder associat ed with Lesch-Nyhan syndrome). Specifiers The severity of non-self-injurious stereotypi c movements ranges from mild presentations that are easily suppressed by a sensory stimul us or distraction to continuous movements that markedly interfere with all activities of da ily living. Self-injurious behaviors range in se- verity along various dimensions, including the frequency, impact on adaptive functioning, and severity of bodily injury (from mild bruising or erythema from hitting hand against body, to lacerations or amputa tion of digits, to retinal detachment from head banging). Diagnostic Features The essential feature of stereotypic movement disorder is repetitive, seemingly driven, and apparently purposeless motor behavior (Criterion A). These behaviors are often rhythmical movements of the head, hands, or body without obvious adaptive function. The movements may or may not respond to efforts to stop them. Among typically devel- oping children, the repetitive movements may be stopped when attention is directed to them or when the child is distracted from performing them. Among children with neuro- developmental disorders, the behaviors are typically less responsive to such efforts. In
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developmental disorders, the behaviors are typically less responsive to such efforts. In other cases, the individual demonstrates self-restraining beha viors (e.g., sitting on hands, wrapping arms in clothing, finding a protective device). The repertoire of behaviors is variable; each individual presents with his or her own in- dividually patterned, “signat ure” behavior. Examples of no n-self-injurious stereotypic movements include, but are not limited to, bo dy rocking, bilateral flapping or rotating hand movements, flicking or fl uttering fingers in front of the face, arm waving or flapping, and head nodding. Stereotyped self-injurious behaviors include, but are not limited to, re- petitive head banging, face slapping, eye poki ng, and biting of hands, lips, or other body parts. Eye poking is particularly concerning; it occurs more freq uently among children with visual impairment. Multiple movements may be combined (e.g., cocking the head, rocking the torso, waving a small string repetitively in front of the face). Stereotypic movements may oc cur many times during a da y, lasting a few seconds to several minutes or longer. Frequency can vary from many occurrences in a single day to several weeks elapsing between episodes. The behaviors vary in context, occurring when the individual is engrossed in other activities , when excited, stressed, fatigued, or bored. Criterion A requires that the movements be “apparently” purposeless. However, some functions may be served by the movements. For example, stereotypic movements might reduce anxiety in response to external stressors. Criterion B states that the stereotypic mo vements interfere with social, academic, or
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Criterion B states that the stereotypic mo vements interfere with social, academic, or other activities and, in some children, may resu lt in self-injury (or would if protective mea- sures were not used). If self-injury is present, it should be coded using the specifier. Onset
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Stereotypic Movement Disorder 79 of stereotypic movements is in the early deve lopmental period (Criterion C). Criterion D states that the repetitive, stereotyped behavior in stereotypic movement disorder is not at- tributable to the physiological effects of a su bstance or neurological condition and is not better explained by another ne urodevelopmental or mental disorder. The presence of stereotypic movements may indicate an unde tected neurodevelopmental problem, espe- cially in children ages 1–3 years. Prevalence Simple stereotypic movements (e.g., rocking) ar e common in young typically developing chil- dren. Complex stereoty pic movements are much less common (occurring in approximately 3%–4%). Between 4% and 16% of individuals with intellectual disability (intellectual develop- mental disorder) engage in stereotypy and self-injury. The risk is greater in individuals with severe intellectual disability. Among individual s with intellectual disability living in res- idential facilities, 10%–15% may have stereo typic movement disord er with self-injury. Development and Course Stereotypic movements typically begin within the first 3 years of life. Simple stereotypic move- ments are common in infancy and may be involve d in acquisition of motor mastery. In chil- dren who develop complex motor stereotypies, approximately 80% exhibit symptoms before 24 months of age, 12% between 24 and 35 months, and 8% at 36 months or older. In most typ- ically developing children, these movements resolve over time or can be suppressed. Onset of complex motor stereotypies may be in infancy or later in the develo pmental period. Among
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individuals with intellectual disability, the ster eotyped, self-injurious behaviors may persist for years, even though the typography or pattern of self-injury may change. Risk and Prognostic Factors Environmental. Social isolation is a risk factor for self-stimulation that may progress to stereotypic movements with repetitive self-inj ury. Environmental stress may also trigger stereotypic behavior. Fear may alter physiological state, resulting in increased frequency of stereotypic behaviors. Genetic and physiological. Lower cognitive functioning is link ed to greater risk for stereo- typic behaviors and poorer response to interv entions. Stereotypic movements are more fre- quent among individuals with moderate-to-se vere/profound intellectual disability, who by virtue of a particular syndrome (e.g., Rett syndrome) or environmental factor (e.g., an environ- ment with relatively insufficient stimulation) se em to be at higher risk for stereotypies. Repet- itive self-injurious behavior ma y be a behavioral phenotype in neurogenetic syndromes. For example, in Lesch-Nyhan syndrome, there are bo th stereotypic dystonic movements and self- mutilation of fingers, lip biti ng, and other forms of self-injury unless the individual is re- strained, and in Rett syndrome and Cornelia de Lange syndrome, self-i njury may result from the hand-to-mouth st ereotypies. Stereotypic behaviors may result from a painful medical con- dition (e.g., middle ear infection, de ntal problems, gastro esophageal reflux). Culture-Related Diagnostic Issues Stereotypic movement disorder, wi th or without self-injury, occurs in all races and cultures. Cultural attitudes toward unusual behaviors may result in delayed diagnosis. Overall cultural
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Cultural attitudes toward unusual behaviors may result in delayed diagnosis. Overall cultural tolerance and attitudes toward stereotypic movement vary and mu st be considered. Differential Diagnosis Normal development. Simple stereotypic movements ar e common in infancy and early childhood. Rocking may occur in the transition from sleep to awake, a behavior that usu-
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80 Neurodevelopmental Disorders ally resolves with age. Complex stereotypi es are less common in typically developing children and can usually be suppressed by dist raction or sensory stimulation. The indi- vidual’s daily routine is rarely affected, and the movements generally do not cause the child distress. The diagno sis would not be appropriat e in these circumstances. Autism spectrum disorder. Stereotypic movements may be a presenting symptom of autism spectrum disorder and should be con sidered when repetitive movements and be- haviors are being evaluated. Deficits of soci al communication and re ciprocity manifesting in autism spectrum disorder are generally ab sent in stereotypic mo vement disorder, and thus social interaction, social communication , and rigid repetitive behaviors and interests are distinguishing features. Wh en autism spectrum disorder is present, stereotypic move- ment disorder is diagnosed only when there is self-injury or when the stereotypic behav- iors are sufficiently severe to become a focus of treatment. Tic disorders. Typically, stereotypies have an earlier age at onset (before 3 years) than do tics, which have a mean age at onset of 5–7 years. They are consistent and fixed in their pattern or topography compared with tics, which are variable in their presentation. Ste- reotypies may involve arms, hands, or the entire body, while tics commonly involve eyes, face, head, and shoulders. Stereotypies are more fixed, rhythmic, and prolonged in dura- tion than tics, which, generally, are brief, ra pid, random, and fluctuating. Tics and stereo- typic movements are both reduced by distraction. Obsessive-compulsive and related disorders. Stereotypic movement disorder is dis-
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Obsessive-compulsive and related disorders. Stereotypic movement disorder is dis- tinguished from obsessive-co mpulsive disorder (OCD) by the absence of obsessions, as well as by the nature of the repetitive behaviors. In OCD the individual feels driven to per- form repetitive behaviors in response to an ob session or according to rules that must be ap- plied rigidly, whereas in stereotypic movement disorder the behaviors are seemingly driven but apparently purposeless. Trichot illomania (hair-pulling disorder) and excoria- tion (skin-picking) disorder are characterize d by body-focused repetitive behaviors (i.e., hair pulling and skin picking) that may be seemingly driven but th at are not apparently purposeless, and that may not be patterned or rhythmical. Furthermore, onset in tricho- tillomania and excoriation disord er is not typically in the early developmental period, but rather around puberty or later. Other neurological and medical conditions. The diagnosis of stereotypic movements requires the exclusion of habits, mannerisms, pa roxysmal dyskinesias, and benign he- reditary chorea. A neurological history and examination are required to assess features suggestive of other disorders, such as myoclo nus, dystonia, tics, and chorea. Involuntary movements associated with a neurological co ndition may be distinguished by their signs and symptoms. For example, repetitive, stereotypic movements in tardive dyskinesia can be distinguished by a history of chronic neuroleptic use and characteristic oral or facial dyskinesia or irregular trunk or limb movements. These type s of movements do not result in self-injury. A diagnosis of stereotypic mo vement disorder is not appropriate for repet-
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itive skin picking or scratching associated with amphetamine intoxi cation or abuse (e.g., patients are diagnosed with substance/medication-induced obsessi ve-compulsive and re- lated disorder) and repetitive choreoathetoid movements associated with other neurolog- ical disorders. Comorbidity Stereotypic movement d isorder may occur as a primary diagnosis or secondary to another disorder. For example, stereotypies are a common manifestation of a variety of neuro- genetic disorders, such as Lesch-Nyhan syndrome, Rett syndrome, fragile X syndrome, Cornelia de Lange syndrome, and Smith-Magenis syndrome. When stereotypic move-
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genetic disorders, such as Lesch-Nyhan syndrome, Rett syndrome, fragile X syndrome, Cornelia de Lange syndrome, and Smith-Magenis syndrome. When stereotypic move- ment disorder co-occurs with another me dical condition, both should be coded.
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Tic Disorders 81 Tic Disorders Diagnostic Criteria Note: A tic is a sudden, rapid, recurrent, nonrhythmic motor movement or vocalization. Tourette’s Disorder 307.23 (F95.2) A. Both multiple motor and one or more vocal tics have been present at some time during the illness, although not necessarily concurrently. B. The tics may wax and wane in frequency but have persisted for more than 1 year since first tic onset. C. Onset is before age 18 years. D. The disturbance is not attributable to the physiological effects of a substance (e.g., co- caine) or another medical condition (e.g., Huntington’s disease, postviral encephalitis). Persistent (Chronic) Motor or Vocal Tic Disorder 307.22 (F95.1) A. Single or multiple motor or vocal tics have been present during the illness, but not both motor and vocal. B. The tics may wax and wane in frequency but have persisted for more than 1 year since first tic onset. C. Onset is before age 18 years. D. The disturbance is not attributable to the physiological effects of a substance (e.g., co- caine) or another medical condition (e.g., Huntington’s disease, postviral encephalitis). E. Criteria have never been met for Tourette’s disorder. Specify if: With motor tics only With vocal tics only Provisional Tic Disorder 307.21 (F95.0) A. Single or multiple motor and/or vocal tics. B. The tics have been present for less than 1 year since first tic onset. C. Onset is before age 18 years.
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C. Onset is before age 18 years. D. The disturbance is not attributable to the physiological effects of a substance (e.g., co- caine) or another medical condition (e.g., Huntington’s disease, postviral encephalitis). E. Criteria have never been met for Tourette’s disorder or persistent (chronic) motor or vocal tic disorder. Specifiers The “motor tics only” or “vocal tics only” spec ifier is only required for persistent (chronic) motor or vocal tic disorder. Diagnostic Features Tic disorders comprise four diagnostic categori es: Tourette’s disorder, persistent (chronic) motor or vocal tic disorder, pr ovisional tic disorder, and the other specified and unspecified tic disorders. Diagnosis for any tic disorder is based on the presence of motor and/or vocal tics (Criterion A), dura tion of tic symptoms (C riterion B), age at onse t (Criterion C), and ab- sence of any known cause such as another medical condition or substance use (Criterion D). The tic disorders are hierarchical in order (i .e., Tourette’s disorder, followed by persistent [chronic] motor or vocal tic disorder, followed by provisional tic disorder, followed by the
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82 Neurodevelopmental Disorders other specified and unspecified tic disorders), such that once a tic disorder at one level of the hierarchy is diagnosed, a lower hierarchy diagnosis cannot be made (Criterion E). Tics are sudden, rapid, recurrent, nonrhythmic motor movements or vocalizations. An individual may have various tic symptoms over time, but at any point in time, the tic rep- ertoire recurs in a characteristic fashion. Although tics can include almost any muscle group or vocalization, certain tic symptoms, such as eye blinking or throat clearing, are common across patient populations. Tics are generall y experienced as involun tary but can be vol- untarily suppressed for varying lengths of time. Tics can be either simple or complex. Simple motor tics are of short duration (i.e., milli- seconds) and can include eye blinking, should er shrugging, and extension of the extrem- ities. Simple vocal tics include throat clea ring, sniffing, and grunting often caused by contraction of the diaphragm or muscles of the oropharynx. Complex motor tics are of lon- ger duration (i.e., seconds) and often include a combination of simple tics such as simul- taneous head turning and shoulder shrugging . Complex tics can appear purposeful, such as a tic-like sexual or obscene gesture ( copropraxia ) or a tic-like imitation of someone else’s movements ( echopraxia). Similarly, complex vocal tics include repeating one’s own sounds or words ( palilalia ), repeating the last-heard word or phrase ( echolalia ), or uttering socially unacceptable words, including obscenities, or ethnic, racial, or religious slurs ( coprolalia ).
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Importantly, coprolalia is an abrupt, sharp ba rk or grunt utterance and lacks the prosody of similar inappropriate speech observed in human interactions. The presence of motor and/or vocal tics varies across the four tic disorders (Criterion A). For Tourette’s disorder, both motor and vo cal tics must be present, whereas for per- sistent (chronic) motor or vocal tic disorder, on ly motor or only vocal tics are present. For provisional tic disorder, motor and/or vocal tics may be presen t. For other specified or un- specified tic disorders, the movement disorder symptoms are best characterized as tics but are atypical in presentation or age at onset, or have a known etiology. The 1-year minimum duration criterion (Cri terion B) assures that individuals diag- nosed with either Tourette’s disorder or pe rsistent (chronic) moto r or vocal tic disorder have had persistent symptoms. Tics wax and wa ne in severity, and some individuals may have tic-free periods of weeks to months; ho wever, an individual who has had tic symp- toms of greater than 1 year’s duration since fi rst tic onset would be considered to have per- sistent symptoms regardless of duration of ti c-free periods. For an individual with motor and/or vocal tics of less than 1 year since firs t tic onset, a provisional tic disorder diagnosis can be considered. There is no duration specif ication for other specif ied and unspecified tic disorders. The onset of tics mu st occur prior to age 18 year s (Criterion C). Tic disorders typically begin in the prepubertal period, with an average age at onset between 4 and 6 years, and with the incidence of new-onset tic disorders decreasing in the teen years. New
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years, and with the incidence of new-onset tic disorders decreasing in the teen years. New onset of tic symptoms in adulthood is exceedin gly rare and is often associated with expo- sures to drugs (e.g., excessive cocaine use) or is a result of a central nervous system insult (e.g., postviral encephalitis). Although tic onse t is uncommon in teenagers and adults, it is not uncommon for adolescents an d adults to present for an initial diagnostic assessment and, when carefully evaluated, provide a histor y of milder symptoms dating back to child- hood. New-onset abnormal move ments suggestive of tics out side of the us ual age range should result in evaluation for other move ment disorders or for specific etiologies.
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hood. New-onset abnormal move ments suggestive of tics out side of the us ual age range should result in evaluation for other move ment disorders or for specific etiologies. Tic symptoms cannot be attributable to the physiologica l effects of a substance or an- other medical condition (Criterion D). When there is strong eviden ce from the history, physical examination, and/or laboratory resu lts to suggest a plausible, proximal, and probable cause for a tic disorder, a diagnosis of other specified tic disorder should be used. Having previously met diagnostic criteria fo r Tourette’s disorder negates a possible di- agnosis of persistent (chronic) motor or vocal ti c disorder (Criterion E). Similarly, a previ- ous diagnosis of persistent (chronic) motor or vocal tic disorder negates a diagnosis of provisional tic disorder or ot her specified or unspecified tic disorder (Criterion E).
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Tic Disorders 83 Prevalence Tics are common in childhood but transient in most cases. The estimated prevalence of Tourette’s disorder ranges from 3 to 8 per 1,000 in school-age children. Males are more commonly affected than females, with the rati o varying from 2:1 to 4:1. A national survey in the United States estimated 3 per 1,000 for the prevalence of clinically identified cases. The frequency of identified cases was lower among African Americans and Hispanic Americans, which may be related to differences in access to care. Development and Course Onset of tics is typically between ages 4 and 6 years. Peak severity occurs between ages 10 and 12 years, with a decline in severity during adolescence. Many adults with tic disorders experience diminished sympto ms. A small percentage of in dividuals will have persis- tently severe or worsening symptoms in adulthood. Tic symptoms manifest similarly in all age groups and across the lifespan. Tics wax and wane in severity and change in affected muscle groups an d vocalizations over time. As children get older, they begin to report th eir tics being associated with a premonitory urge—a somatic sensation that precedes the tic—and a feeling of tension reduction follow- ing the expression of the tic. Tics associated with a premonitory urge may be experienced as not completely “involuntary” in that the ur ge and the tic can be resisted. An individual may also feel the need to perform a tic in a specific way or repeat it until he or she achieves the feeling that the tic has been done “just right.” The vulnerability toward developing co-occurring conditions changes as individuals pass through the age of risk for various co-o ccurring conditions. For example, prepubertal
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children with tic disorders are more likely to experience attention-deficit/hyperactivity disorder (ADHD), obsessive-co mpulsive disorder (OCD), and separation anxiety disorder than are teenagers and adults, who are more li kely to experience the new onset of major depressive disorder, substance use disorder, or bipolar disorder. Risk and Prognostic Factors Temperamental. Tics are worsened by anxiety, excitement, and exhaustion and are better during calm, focused activities. Individuals ma y have fewer tics when engaged in schoolwork or tasks at work than when relaxing at home after school or in the evening. Stressful/exciting events (e.g., taking a test, participating in exciting activities) often make tics worse. Environmental. Observing a gesture or sound in another person may result in an indi- vidual with a tic disorder making a simila r gesture or sound, which may be incorrectly perceived by others as purposeful. This can be a particular problem when the individual is interacting with authority figures (e .g., teachers, supe rvisors, police). Genetic and physiological. Genetic and environmental factors influence tic symptom expression and severity. Important risk allele s for Tourette’s disorder and rare genetic variants in families with tic disorders have been identified. Obstetrical complications, older paternal age, lower birth weight, and maternal smoking during pregnancy are as- sociated with worse tic severity. Culture-Related Diagnostic Issues Tic disorders do not appear to vary in clinical characteristics, course , or etiology by race, ethnicity, and culture. However, race, ethnici ty, and culture may impa ct how tic disorders are perceived and managed in the family an d community, as well as influencing patterns of help seeking, and choices of treatment.
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84 Neurodevelopmental Disorders Gender-Related Diagnostic Issues Males are more commonly affected than females, but there are no gender differences in the kinds of tics, age at onset, or course. Wome n with persistent tic disorders may be more likely to experience anxiety and depression. Functional Consequenc es of Tic Disorders Many individuals with mild to moderate tic se verity experience no distress or impairment in functioning and may even be unaware of their tics. Individuals with more severe symp- toms generally have more impairment in daily living, but ev en individuals with moderate or even severe tic disorders may function we ll. The presence of a co-occurring condition, such as ADHD or OCD, can have greater impa ct on functioning. Less commonly, tics dis- rupt functioning in daily activities and result in social isolation, interpersonal conflict, peer victimization, inab ility to work or to go to school, and lower quality of life. The indi- vidual also may experience substantial psycho logical distress. Rare complications of Tou- rette’s disorder include physical injury, such as eye injury (from hitting oneself in the face), and orthopedic and neurological injury (e.g., disc disease related to forceful head and neck movements). Differential Diagnosis Abnormal movements that may accompany other medical conditions and stereotypic movement disorder. Motor stereotypies are defined as involuntary rhythmic, repetitive, predictable movements that appe ar purposeful but serve no obvious adaptive function or purpose and stop with distraction. Examples include repetitive hand waving/rotating, arm flapping, and finger wiggling. Motor stereo typies can be differentiated from tics based on the former’s earlier age at onset (younger than 3 years), prolonged duration (seconds to
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minutes), constant repetitive fixed form and location, exacer bation when engrossed in ac- tivities, lack of a premonitory urge, and ce ssation with distraction (e.g., name called or touched). Chorea represents rapid, random, continua l, abrupt, irregular, unpredictable, nonstereotyped actions that are usually bilatera l and affect all parts of the body (i.e., face, trunk, and limbs). The timing, direction, and dist ribution of movements vary from mo- ment to moment, and movements usually worsen during attempted voluntary action. Dys- tonia is the simultaneous sustained contracture of both agonist and antagonist muscles, resulting in a distorted posture or movement of parts of the bo dy. Dystonic postures are of- ten triggered by attempts at voluntary movements and are not seen during sleep. Substance-induced and paroxysmal dyskinesias. Paroxysmal dyskinesias usually oc- cur as dystonic or choreoathetoid movement s that are precipitated by voluntary move- ment or exertion and less commonly arise from normal background activity. Myoclonus. Myoclonus is characterized by a sudden unidirectional movement that is often nonrhythmic. It may be worsened by movement and occur du ring sleep. Myoclonus is differentiated from tics by its rapidity, la ck of suppressibility, and absence of a premon- itory urge. Obsessive-compulsive and related disorders. Differentiating obsessive-compulsive behaviors from tics may be difficult. Clues fa voring an obsessive-compulsive behavior in- clude a cognitive-based drive (e.g., fear of co ntamination) and the n eed to perform the ac-
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tion in a particular fashion a certain number of times, equally on both sides of the body, or until a “just right” feeling is achieved. Impulse-control proble ms and other repetitive be- haviors, including persistent hair pulling, skin picking, and nail biting, appear more goal directed and complex than tics.
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Other Specified Tic Disorder 85 Comorbidity Many medical and psychiatric conditions have been described as co-occurring with tic disor- ders, with ADHD and obsessive-compulsive and related disorders be ing particularly com- mon. The obsessive-compulsive symptoms observed in tic disorder tend to be characterized by more aggressive symmetry and order symptoms and poorer response to pharmacotherapy with selective serotonin reuptake inhibitors. Children with ADHD ma y demonstrate disrup- tive behavior, social immaturi ty, and learning difficulties th at may interfere with academic progress and interpersonal relationships and lead to greater impairment than that caused by a tic disorder. Individuals with tic disorders can also have other movement disorders and other mental disorders, such as depressive, bipolar, or substance use disorders. Other Specified Tic Disorder 307.20 (F95.8) This category applies to presentations in which symptoms characteristic of a tic disorder that cause clinically significant distress or impairment in social, occupational, or other im- portant areas of functioning predominate but do not meet the full criteria for a tic disorder or any of the disorders in the neurodevelopmental disorders diagnostic class. The other specified tic disorder category is used in situations in which the clinician chooses to com- municate the specific reason that the presentation does not meet the criteria for a tic disor- der or any specific neurodevelopmental disorder. This is done by recording “other specified tic disorder” followed by the specific reason (e.g., “with onset after age 18 years”). Unspecified Tic Disorder 307.20 (F95.9) This category applies to presentations in whic h symptoms characteristic of a tic disorder that cause clinically significant distress or impairment in social, occupational, or other im- portant areas of functioning predominate but do not meet the full criteria for a tic disorder
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or for any of the disorders in the neurodevelopmental disorders diagnostic class. The un- specified tic disorder category is used in situations in which the clinician chooses not to specify the reason that the criteria are not met for a tic disorder or for a specific neurode- velopmental disorder, and includes presentations in which there is insufficient information to make a more specific diagnosis.
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86 Neurodevelopmental Disorders Other Neurodevelopmental Disorders Other Specified Neurodevelopmental Disorder 315.8 (F88) This category applies to presentations in which symptoms characteristic of a neurodevel- opmental disorder that cause 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 neurodevelopmental disorders diagnostic clas s. The other specified neurodevelopmental disorder category is used in situations in which the clinician chooses to communicate the specific reason that the presentation does not meet the criteria for any specific neurode- velopmental disorder. This is done by recording “other specified neurodevelopmental dis- order” followed by the specific reason (e.g., “neurodevelopmental disorder associated with prenatal alcohol exposure”). An example of a presentation that can be specified using the “other specified” desig- nation is the following: Neurodevelopmental disord er associated with pren atal alcohol exposure: Neu- rodevelopmental disorder associated with prenatal alcohol exposure is characterized by a range of developmental disabilities following exposure to alcohol in utero. Unspecified Neurodevelopmental Disorder 315.9 (F89) This category applies to presentations in which symptoms characteristic of a neurodevel- opmental disorder that cause 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 neurodevelopmental disorders diagnostic class. The unspecified neurodevelopmental dis- order category is used in situations in which the clinician chooses not to specify the reason that the criteria are not met for a specific neurodevelopmental disorder, and includes pre- sentations in which there is insufficient information to make a more specific diagnosis (e.g., in emergency room settings).
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87Schizophrenia Spectrum and Other Psychotic Disorders Schizophrenia spectrum and other psychotic disorders include schizophrenia, other psychotic disorders, and schizotypal (per sonality) disorder. They are defined by ab- normalities in one or more of the following fi ve domains: delusions, hallucinations, disor- ganized thinking (speech), grossly disorganiz ed or abnormal moto r behavior (including catatonia), and negative symptoms. Key Features That Define the Psychotic Disorders Delusions Delusions are fixed beliefs that are not amenable to change in light of conflicting evidence. Their content may include a variety of themes (e.g., persecutory, referential, somatic, reli- gious, grandiose). Persecutory delusions (i.e., belief that one is going to be harmed, harassed, and so forth by an individual, organizat ion, or other group) are most common. Referential delusions (i.e., belief that certain gestures, comm ents, environmental cues, and so forth are directed at oneself) are also common. Grandiose delusions (i.e., when an individual believes that he or she has exceptional abilities, wealth, or fame) and erotomanic delusions (i.e., when an individual believes falsely that another person is in love with him or her) are also seen. Nihilistic delusions involve the conviction that a ma jor catastrophe will occur, and somatic delusions focus on preoccupations regarding health and organ function. Delusions are deemed bizarre if they are clearly implausible and not understandable to same-culture peers and do not derive from or dinary life experiences. An example of a bi- zarre delusion is the belief that an outside forc e has removed his or her internal organs and
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replaced them with someone else’s organs wi thout leaving any wounds or scars. An ex- ample of a nonbizarre delusion is the belief that one is under surveilla nce by the police, de- spite a lack of convincing evidence. Delusion s that express a loss of control over mind or body are generally considered to be bizarre; these include th e belief that one’s thoughts have been “removed” by some outside force (thought withdrawal ), that alien thoughts have been put into one’s mind ( thought insertion ), or that one’s body or actions are being acted on or manipulated by so me outside force ( delusions of control ). The distinction between a de- lusion and a strongly held idea is sometimes di fficult to make and depends in part on the degree of conviction with which the belief is held despite clear or reasonable contradictory evidence regarding its veracity. Hallucinations Hallucinations are perception-like experiences that occur without an external stimulus. They are vivid and clear, with the full force and impact of normal perceptions, and not under voluntary control. They may occur in any sensory modality, but auditory halluci- nations are the most common in schizophrenia and related disorders. Auditory hallucina- tions are usually experienced as voices, whethe r familiar or unfamiliar, that are perceived as distinct from the individual’s own though ts. The hallucinations must occur in the con- text of a clear sensorium; those that occur while falling asleep ( hypnagogic ) or waking up
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88 Schizophrenia Spectrum and Other Psychotic Disorders (hypnopompic ) are considered to be within the rang e of normal experience. Hallucinations may be a normal part of religious expe rience in certain cultural contexts. Disorganized Thinking (Speech) Disorganized thinking (formal thought disorder ) is typically inferred from the individual’s speech. The individual may switch from one topic to another ( derailment or loose associa- tions ). Answers to questions may be oblique ly related or completely unrelated ( tangential- ity). Rarely, speech may be so severely disorgan ized that it is nearly incomprehensible and resembles receptive aphasia in its linguistic disorganization ( incoherence or “word salad”). Because mildly disorganized speech is common and nonspecific, the symptom must be se- vere enough to substantially impair effective communication. The severity of the impair- ment may be difficult to evaluate if the person making the diagnosis comes from a different linguistic background than that of the person being examin ed. Less severe dis- organized thinking or speech may occur du ring the prodromal and residual periods of schizophrenia. Grossly Disorganized or Abnormal Motor Behavior (Including Catatonia) Grossly disorganized or abnormal motor behavior may manifest itself in a variety of ways, ranging from childlike “silliness” to unpred ictable agitation. Problems may be noted in any form of goal-directed behavior, leading to difficulties in performing activities of daily living. Catatonic behavior is a marked decrease in reactivity to the environment. This ranges from resistance to instructions ( negativism ); to maintaining a rigid, inappropriate or bi- zarre posture; to a complete lack of verbal and motor responses ( mutism and stupor ). It can
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also include purposeless and excessive motor activity without obvious cause ( catatonic excitement ). Other features are repeated stereo typed movements, staring, grimacing, mutism, and the echoing of speech. Although catatonia has historically been associated with schizophrenia, catatonic symptoms are nonspecific and may occur in other mental disorders (e.g., bipolar or depressive disorder s with catatonia) and in medical conditions (catatonic disorder due to another medical condition). Negative Symptoms Negative symptoms account for a substantial portion of the morbidity associated with schizophrenia but are less prominent in other psychotic disorders. Two negative symp- toms are particularly prominent in schizophrenia: diminished emotional expression and avolition. Diminished emotional expression includes reductions in the expression of emo- tions in the face, eye contact, intonation of speech (prosody), and movements of the hand, head, and face that normally give an emotional emphasis to speech. Avolition is a decrease in motivated self-initiated purposeful activities. The individual may sit for long periods of time and show little interest in participating in work or so cial activities. Other negative symptoms include alogia, anhedonia, and asociality. Alogia is manifested by diminished speech output. Anhedonia is the decreased ability to ex perience pleasure from positive stimuli or a degradation in the recollection of pleasure previously experienced. Asociality refers to the apparent lack of interest in so cial interactions and may be associated with avo- lition, but it can also be a ma nifestation of limited opportunities for social interactions. Disorders in This Chapter This chapter is organized along a gradient of psychopathology. Clinicians should first con- sider conditions that do not re ach full criteria for a psychotic disorder or are limited to one
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Schizophrenia Spectrum and Other Psychotic Disorders 89 domain of psychopathology. Then they should consider time-limited conditions. Finally, the diagnosis of a schizophrenia spectrum diso rder requires the exclusion of another con- dition that may give rise to psychosis. Schizotypal personality disorder is noted within this chapter as it is considered within the schizophrenia spectrum, although its full description is found in the chapter “Person- ality Disorders.” The diagnosis schizotypal pers onality disorder captures a pervasive pat- tern of social and interpersonal deficits, including reduced capacity for close relationships; cognitive or perceptual distor tions; and eccentricities of behavior, usually beginning by early adulthood but in some cases first becoming apparent in childhood and adolescence. Abnormalities of beliefs, thinki ng, and perception are below the threshold for the diagno- sis of a psychotic disorder. Two conditions are defined by abnormalities limited to one domain of psychosis: delu- sions or catatonia. Delusional disorder is characterized by at least 1 month of delusions but no other psychotic symptoms. Catatonia is desc ribed later in the chapter and further in this discussion. Brief psychotic disorder lasts more than 1 day and remits by 1 month. Schizophreni- form disorder is characterized by a symptomatic presentation equivalent to that of schizo- phrenia except for its duration (less than 6 months) and the absence of a requirement for a decline in functioning. Schizophrenia lasts for at least 6 months an d includes at least 1 month of active-phase symptoms. In schizoaffective disorder, a mood episode and the active-phase symptoms of schizophrenia occur together an d were preceded or are followe d by at least 2 weeks of de-
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lusions or hallucinations with out prominent mood symptoms. Psychotic disorders may be induced by anot her condition. In substance/medication- induced psychotic disorder, the psychotic symp toms are judged to be a physiological con- sequence of a drug of abuse, a medication, or toxin exposure and cease after removal of the agent. In psychotic disorder due to anothe r medical condition, the psychotic symptoms are judged to be a direct physiological consequence of another medical condition. Catatonia can occur in severa l disorders, including neurod evelopmental, psychotic, bi- polar, depressive, and other mental disorders. This chapter also includes the diagnoses catatonia associated with anot her mental disorder (catatonia specifier), catatonic disorder due to another medical condition, and unspecifie d catatonia, and the diagnostic criteria for all three conditions are described together. Other specified and unspecified schizophrenia spectrum and other psychotic disor- ders are included for classifyin g psychotic presentations that do not meet the criteria for any of the specific psychotic disorders, or psychotic symptomatology about which there is inadequate or contra dictory information. Clinician-Rated Assessment of Symptoms and Related Clinical Phenomena in Psychosis Psychotic disorders are heterogeneous, and th e severity of symptoms can predict impor- tant aspects of the illness, such as the degree of cognitive or neur obiological deficits. To move the field forward, a detailed framework fo r the assessment of severity is included in Section III “Assessment Measures,” which may help with treatment planning, prognostic decision making, and research on patho physiological mechanisms. Section III “Assess- ment Measures” also contains dimensional as sessments of the primary symptoms of psy- chosis, including hallucinations, delusions, disorganized speech (except for substance/
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chosis, including hallucinations, delusions, disorganized speech (except for substance/ medication-induced psychotic disorder and psychotic disorder due to another medical condition), abnormal psychomotor behavior, and negative symptoms, as well as dimen- sional assessments of depression and mania. Th e severity of mood symptoms in psychosis
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condition), abnormal psychomotor behavior, and negative symptoms, as well as dimen- sional assessments of depression and mania. Th e severity of mood symptoms in psychosis has prognostic value and guides treatment. There is growing evidence that schizoaffective
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90 Schizophrenia Spectrum and Other Psychotic Disorders disorder is not a distinct nosological catego ry. Thus, dimensional assessments of depres- sion and mania for all psychotic disorders alert clinicians to mood pathology and the need to treat where appropriate. The Section III sc ale also includes a dimensional assessment of cognitive impairment. Many individuals with psychotic disorders have impairments in a range of cognitive domains that predict functi onal status. Clinical neuropsychological as- sessment can help guide diagnosis and treatm ent, but brief assessments without formal neuropsychological assessment can provide us eful information that can be sufficient for diagnostic purposes. Formal neuropsychological testing, when conducted, should be ad- ministered and scored by personnel trained in the use of testing instruments. If a formal neuropsychological assessment is not conducted, the clinician should use the best avail- able information to make a judgment. Furthe r research on these assessments is necessary in order to determine their c linical utility; thus, the assessments available in Section III should serve as a prototype to stimulate such research. Schizotypal (Personality) Disorder Criteria and text for schizotypal personality disorder can be found in the chapter “Person- ality Disorders.” Because this disorder is cons idered part of the schizophrenia spectrum of disorders, and is labeled in this section of ICD-9 and ICD-10 as schizotypal disorder, it is listed in this chapter and disc ussed in detail in the DSM-5 chapter “Personality Disorders.” Delusional Disorder Diagnostic Criteria 297.1 (F22) A. The presence of one (or more) delusions with a duration of 1 month or longer. B. Criterion A for schizophrenia has never been met.
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B. Criterion A for schizophrenia has never been met. Note: Hallucinations, if present, are not prominent and are related to the delusional theme (e.g., the sensation of being infested with insects associated with delusions of infestation). C. Apart from the impact of the delusion(s) or its ramifications, functioning is not markedly impaired, and behavior is not obviously bizarre or odd. D. If manic or major depressive episodes have occurred, these have been brief relative to the duration of the delusional periods. E. The disturbance is not attributable to the physiological effects of a substance or an- other medical condition and is not better ex plained by another mental disorder, such as body dysmorphic disorder or obsessive-compulsive disorder. Specify whether: Erotomanic type: This subtype applies when the central theme of the delusion is that another person is in love with the individual. Grandiose type: This subtype applies when the central theme of the delusion is the conviction of having some great (but unrecognized) talent or insight or having made some important discovery. Jealous type: This subtype applies when the central theme of the individual’s delusion is that his or her spouse or lover is unfaithful. Persecutory type: This subtype applies when the central theme of the delusion in- volves the individual’s belief that he or she is being conspired against, cheated, spied on, followed, poisoned or drugged, maliciously maligned, harassed, or obstructed in the pursuit of long-term goals. Somatic type: This subtype applies when the central theme of the delusion involves bodily functions or sensations.
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Delusional Disorder 91 Mixed type: This subtype applies when no one delusional theme predominates. Unspecified type: This subtype applies when the dominant delusional belief cannot be clearly determined or is not described in the specific types (e.g., referential delu- sions without a prominent persecutory or grandiose component). Specify if: With bizarre content: Delusions are deemed bizarre if they are clearly implausible, not understandable, and not derived from ordinary li fe experiences (e.g., an individual’s be- lief that a stranger has removed his or her internal organs and replaced them with some- one else’s organs without leaving any wounds or scars). Specify if: The following course specifiers are only to be used after a 1-year duration of the disorder: First episode, currentl y in acute episode: First manifestation of the disorder meet- ing the defining diagnostic symptom and time criteria. An acute episode is a time pe- riod in which the symptom criteria are fulfilled. First episode, currently in partial remission: Partial remission is a time period dur- ing which an improvement after a previous episode is maintained and in which the de- fining criteria of the disorder are only partially fulfilled. First episode, currently in full remission: Full remission is a period of time after a previous episode during which no disorder-specific symptoms are present. Multiple episodes, curr ently in acute episode Multiple episodes, currently in partial remission Multiple episodes, currently in full remission Continuous: Symptoms fulfilling the diagnostic symptom criteria of the disorder are remaining for the majority of the illness course, with subthreshold symptom periods be- ing very brief relative to the overall course. Unspecified Specify current severity: Severity is rated by a quantitative assessment of the primary symptoms of psychosis,
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Specify current severity: Severity is rated by a quantitative assessment of the primary symptoms of psychosis, including delusions, hallucinations, disorganized speech, abnormal psychomotor be- havior, and negative symptoms. Each of these symptoms may be rated for its current severity (most severe in the last 7 days) on a 5-point scale ranging from 0 (not present) to 4 (present and severe). (See Clinician-Rated Dimensions of Psychosis Symptom Severity in the chapter “Assessment Measures.”) Note: Diagnosis of delusional disorder can be made without using this severity specifier. Subtypes In erotomanic type, the central theme of the delusion is that another person is in love with the individual. The person about whom this conviction is held is usually of higher status (e.g., a famous individual or a superior at work) but can be a complete stranger. Efforts to contact the object of the delusion are common. In grandiose type, the central theme of the de- lusion is the conviction of having some great talent or insight or of having made some im- portant discovery. Less commo nly, the individual may have the delusion of having a special relationship with a prominent individu al or of being a prominent person (in which case the actual individual may be regarded as an impostor). Grandiose delusions may have a religious content. In jealous type, the central theme of the delusion is that of an un- faithful partner. This belief is arrived at with out due cause and is based on incorrect infer- ences supported by small bits of “evidence” (e.g., disarrayed clothing). The individual with the delusion usually conf ronts the spouse or lover and at tempts to intervene in the
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imagined infidelity. In persecutory type, the central theme of the delusion involves the in-
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92 Schizophrenia Spectrum and Other Psychotic Disorders dividual’s belief of being conspired against, cheated, spied on, followed, poisoned, mali- ciously maligned, harassed, or obstructed in the pursuit of long-term goals. Small slights may be exaggerated and become the focus of a delusional system. The affected individual may engage in repeated attempts to obtain sat isfaction by legal or legislative action. Indi- viduals with persecutory delusions are often resentful and angry and may resort to vio- lence against those they believe are hurting them. In somatic type, the central theme of the delusion involves bodily functi ons or sensations. Somatic de lusions can occur in several forms. Most common is the belief that the indi vidual emits a foul odor ; that there is an in- festation of insects on or in the skin; that ther e is an internal parasite; that certain parts of the body are misshapen or ugly; or that parts of the body are not functioning. Diagnostic Features The essential feature of delusional disorder is the presence of one or more delusions that persist for at least 1 month (Criterion A). A diagnosis of delusional disorder is not given if the individual has ever had a symptom presen tation that met Criterion A for schizophre- nia (Criterion B). Apart from the direct impact of the delusions, impairments in psychoso- cial functioning may be more circumscribed th an those seen in other psychotic disorders such as schizophrenia, and behavior is not obviously bizarre or odd (Criterion C). If mood episodes occur concurrently with the delusions , the total duration of these mood episodes is brief relative to the total duration of the delusional periods (Criterion D). The delusions
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are not attributable to the physiological effects of a su bstance (e.g., cocaine) or another medical condition (e.g., Alzheimer’s disease) and are not better explained by another men- tal disorder, such as body dy smorphic disorder or obsessive- compulsive disorder (Crite- rion E). In addition to the five symptom domain areas identified in the diagnostic criteria, the assessment of cognition, depression, and mani a symptom domains is vital for making crit- ically important distinctions between the va rious schizophrenia spectrum and other psy- chotic disorders. Associated Features Supporting Diagnosis Social, marital, or work problems can result from the delusional beliefs of delusional dis- order. Individuals with delusional disorder ma y be able to factually describe that others view their beliefs as irrational but are unable to accept this themselves (i.e., there may be “factual insight” but no true insight). Many individuals de velop irritable or dysphoric mood, which can usually be understood as a re action to their delusional beliefs. Anger and violent behavior can occur with persecutory, jealous, and erotomanic types. The individ- ual may engage in litigious or antagonistic behavior (e.g., sending hundreds of letters of protest to the government). Legal difficulties can occur, particularly in jealous and eroto- manic types. Prevalence The lifetime prevalence of delusional disorder has been estimated at around 0.2%, and the most frequent subtype is persecutory. Delu sional disorder, jealous type, is probably more common in males than in females, but there ar e no major gender differences in the overall frequency of delu sional disorder. Development and Course On average, global function is generally better than that observed in schizophrenia. Al-
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On average, global function is generally better than that observed in schizophrenia. Al- though the diagnosis is generally stable, a pr oportion of individuals go on to develop
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Delusional Disorder 93 schizophrenia. Delusional disorder has a significant familial relationship with both schizophrenia and schizotypal personality diso rder. Although it can occur in younger age groups, the condition may be more prevalent in older individuals. Culture-Related Diagnostic Issues An individual’s cultural and religious backgr ound must be taken into account in evaluat- ing the possible presence of delusional di sorder. The content of delusions also varies across cultural contexts. Functional Consequences of Delusional Disorder The functional impairment is usually more ci rcumscribed than that seen with other psy- chotic disorders, although in some cases, the impairment may be substantial and include poor occupational functioning and social isola tion. When poor psychosocial functioning is present, delusional beliefs themselves ofte n play a significant role. A common character- istic of individuals with delusional disorder is the apparent normality of their behavior and appearance when their delusional idea s are not being discussed or acted on. Differential Diagnosis Obsessive-compulsive and related disorders. If an individual wi th obsessive-compul- sive disorder is completely convinced that his or her obsessive-comp ulsive disorder beliefs are true, then the diagnosis of obsessive-compulsive disorder, with absent insight/delu- sional beliefs specifier, should be given rath er than a diagnosis of delusional disorder. Similarly, if an individual with body dysm orphic disorder is completely convinced that his or her body dysmorphic disorder beliefs are true, then the diagnosis of body dysmor- phic disorder, with absent insight/delusional beliefs specifier, should be given rather than a diagnosis of delusional disorder. Delirium, major neurocognitive disorder, psychotic disorder due to another medical con-
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Delirium, major neurocognitive disorder, psychotic disorder due to another medical con- dition, and substance/medicati on-induced psychotic disorder. Individuals with these disorders may present with symptoms that su ggest delusional disorder. For example, sim- ple persecutory delusions in the context of major neurocognitive di sorder would be di- agnosed as major neurocognitive disorder, with behavioral disturbance. A substance/ medication-induced psychotic disorder cross-sectionally may be identical in symptom- atology to delusional disorder but can be di stinguished by the chronological relationship of substance use to the onset and remission of the delusional beliefs. Schizophrenia and schizophreniform disorder. Delusional disorder can be distinguished from schizophrenia and schizophreniform disord er by the absence of the other character- istic symptoms of the active phase of schizophrenia. Depressive and bipolar disorders and schizoaffective disorder. These disorders may be distinguished from delusional disorder by the temporal relationship between the mood disturbance and the delusions and by the severi ty of the mood symptoms. If delusions oc- cur exclusively during mood episodes, the diag nosis is depressive or bipolar disorder with psychotic features. Mood sympto ms that meet full criteria for a mood episode can be su- perimposed on delusional disorder. Delusional di sorder can be diagnose d only if the total duration of all mood episodes remains brief rela tive to the total duration of the delusional disturbance. If not, then a diagnosis of othe r specified or unspecified schizophrenia spec- trum and other psychotic diso rder accompanied by other sp ecified depressi ve disorder, unspecified depressive disorder, other specif ied bipolar and related disorder, or unspeci-
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fied bipolar and related disorder is appropriate.
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94 Schizophrenia Spectrum and Other Psychotic Disorders Brief Psychotic Disorder Diagnostic Criteria 298.8 (F23) A. Presence of one (or more) of the following symptoms. At least one of these must be (1), (2), or (3): 1. Delusions. 2. Hallucinations. 3. Disorganized speech (e.g., frequent derailment or incoherence). 4. Grossly disorganized or catatonic behavior. Note: Do not include a symptom if it is a culturally sanctioned response. B. Duration of an episode of the disturbance is at least 1 day but less than 1 month, with eventual full return to premorbid level of functioning. C. The disturbance is not better explained by major depressive or bipolar disorder with psychotic features or another psychotic disorder such as schizophrenia or catatonia, and is not attributable to the physiological effects of a substance (e.g., a drug of abuse, a medication) or another medical condition. Specify if: With marked stressor(s) (brief reactive psychosis): If symptoms occur in response to events that, singly or together, would be markedly stressful to almost anyone in similar circumstances in the individual’s culture. Without marked stressor(s): If symptoms do not occur in response to events that, singly or together, would be markedly stressful to almost anyone in similar circum- stances in the individual’s culture. With postpartum onset: If onset is during pregnancy or within 4 weeks postpartum. Specify if: With catatonia (refer to the criteria for catatonia associated with another mental dis- order, pp. 119–120, for definition) Coding note: Use additional code 293.89 (F06.1) catatonia associated with brief
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psychotic disorder to indicate the presence of the comorbid catatonia. Specify current severity: Severity is rated by a quantitative assessment of the primary symptoms of psychosis, including delusions, hallucinations, disorganized speech, abnormal psychomotor be- havior, and negative symptoms. Each of these symptoms may be rated for its current severity (most severe in the last 7 days) on a 5-point scale ranging from 0 (not present) to 4 (present and severe). (See Clinician-Rated Dimensions of Psychosis Symptom Severity in the chapter “Assessment Measures.”) Note: Diagnosis of brief psychotic disorder can be made without using this severity specifier. Diagnostic Features The essential feature of brief psychotic disorder is a disturba nce that involves the sudden onset of at least one of the following positive psychotic symptoms: delusions, hallucina- tions, disorganized speech (e.g., frequent de railment or incoherence), or grossly abnormal psychomotor behavior, includin g catatonia (Criterion A). Sudden onset is defined as change from a nonpsychotic state to a clearly psychotic state within 2 weeks, usually with- out a prodrome. An episode of the disturbance lasts at least 1 day but less than 1 month, and the individual eventually has a full return to the premorbid level of functioning (Cri-
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Brief Psychotic Disorder 95 terion B). The disturbance is not better explai ned by a depressive or bipolar disorder with psychotic features, by schizoaffective disorder , or by schizophrenia and is not attributable to the physiological effects of a substance (e .g., a hallucinogen) or another medical condi- tion (e.g., subdural hematoma) (Criterion C). In addition to the five symptom domain areas identified in the diagnostic criteria, the assessment of cognition, depression, and mani a symptom domains is vital for making crit- ically important distinctions between the va rious schizophrenia spectrum and other psy- chotic disorders. Associated Features Supporting Diagnosis Individuals with brief psychoti c disorder typically experience emotional turmoil or over- whelming confusion. They may have rapid sh ifts from one intense affect to another. Although the disturbance is brief, the level of impairment may be severe, and supervision may be required to ensure that nutritional and hygienic needs are met and that the indi- vidual is protected from the consequences of poor judgment, cognitive impairment, or act- ing on the basis of delusions. There appears to be an increased risk of suicidal behavior, particularly during the acute episode. Prevalence In the United States, brief psychotic disorder may account for 9% of cases of first-onset psychosis. Psychotic disturbances that meet Crit eria A and C, but not Criterion B, for brief psychotic disorder (i.e., duration of active symptoms is 1–6 months as opposed to remis- sion within 1 month) are more common in developing countries than in developed coun- tries. Brief psychotic diso rder is twofold more common in females than in males. Development and Course Brief psychotic disorder may appear in adolescence or early adulthood, and onset can oc-
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Brief psychotic disorder may appear in adolescence or early adulthood, and onset can oc- cur across the lifespan, with the average age at onset being the mid 30s. By definition, a diagnosis of brief psychotic disorder requir es a full remission of all symptoms and an eventual full return to the premorbid level of functioning within 1 mont h of the onset of the disturbance. In some individuals, the duration of psychotic symptoms may be quite brief (e.g., a few days). Risk and Prognostic Factors Temperamental. Preexisting personality disorders an d traits (e.g., schizotypal person- ality disorder; borderline personality disorder; or traits in the psychoticism domain, such as perceptual dysregulation, and the negative affectivity domain, such as suspiciousness) may predispose the individual to the development of the disorder. Culture-Related Diagnostic Issues It is important to distinguish symptoms of br ief psychotic disorder from culturally sanc- tioned response patterns. For example, in so me religious ceremonies, an individual may report hearing voices, but these do not genera lly persist and are not perceived as abnormal by most members of the indivi dual’s community. In addition , cultural and religious back- ground must be taken into account when considering whether be liefs are delusional. Functional Consequences of Brief Psychotic Disorder Despite high rates of relapse, for most indivi duals, outcome is excellent in terms of social functioning and symptomatology.
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96 Schizophrenia Spectrum and Other Psychotic Disorders Differential Diagnosis Other medical conditions. A variety of medical disorders can manifest with psychotic symptoms of short duration. Psychotic disorder due to another medica l condition or a de- lirium is diagnosed when there is evidence from the history, physical examination, or lab- oratory tests that the delusions or hallucinati ons are the direct physiological consequence of a specific medical condition (e.g., Cushing’s syndrome, brain tumor) (see “Psychotic Disorder Due to Another Medical Co ndition” later in this chapter). Substance-related disorders. Substance/medication-induced psychotic disorder, sub- stance-induced delirium, and substance intoxication are distinguished from brief psychotic disorder by the fact that a substance (e.g., a drug of abuse, a medication, exposure to a toxin) is judged to be etiologically related to the psychotic symp toms (see “Substance/Medication- Induced Psychotic Disorder” later in this chapte r). Laboratory tests, such as a urine drug screen or a blood alcohol level, may be helpful in making this determination, as may a care- ful history of substance use with attention to temporal relationships between substance in- take and onset of the symp toms and to the nature of the substance being used. Depressive and bipolar disorders. The diagnosis of brief psychotic disorder cannot be made if the psychotic symptoms are better explained by a mood episode (i.e., the psychotic symptoms occur exclusively during a full major depressive, manic, or mixed episode). Other psychotic disorders. If the psychotic symptoms pers ist for 1 month or longer, the diagnosis is either schizophr eniform disorder, delusional disorder, depressive disorder with psychotic features, bipolar disorder with psychotic fe atures, or other specified or un-
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with psychotic features, bipolar disorder with psychotic fe atures, or other specified or un- specified schizophrenia spectrum and other psychotic disorder, depending on the other symptoms in the presentation. The differentia l diagnosis between brief psychotic disorder and schizophreniform disorder is difficult wh en the psychotic symptoms have remitted be- fore 1 month in response to successful trea tment with medication. Careful attention should be given to the possibility that a recurrent diso rder (e.g., bipolar disorder, recurrent acute ex- acerbations of schizophrenia) may be respon sible for any recurring psychotic episodes. Malingering and factitious disorders. An episode of factitious disorder, with predomi- nantly psychological signs and symptoms, may have the appearance of brief psychotic disorder, but in such cases there is evidence that the symptoms are intentionally produced. When malingering involves apparently psyc hotic symptoms, there is usually evidence that the illness is being feigned for an understandable goal. Personality disorders. In certain individual s with personality disorders, psychosocial stressors may precipitate brief periods of psychotic symptoms. These symptoms are usu- ally transient and do not warrant a separate diagnosis. If psychotic symptoms persist for at least 1 day, an additional diagnosis of br ief psychotic disorder may be appropriate. Schizophreniform Disorder Diagnostic Criteria 295.40 (F20.81) A. Two (or more) of the following, each present for a significant portion of time during a 1-month period (or less if successfully treated). At least one of these must be (1), (2), or (3): 1. Delusions. 2. Hallucinations. 3. Disorganized speech (e.g., frequent derailment or incoherence). 4. Grossly disorganized or catatonic behavior.
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4. Grossly disorganized or catatonic behavior. 5. Negative symptoms (i.e., diminished emotional expression or avolition).
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Schizophreniform Disorder 97 B. An episode of the disorder lasts at least 1 month but less than 6 months. When the diagnosis must be made without waiting for recovery, it should be qualified as “provi- sional.” C. Schizoaffective disorder and depressive or bipolar disorder with psychotic features have been ruled out because either 1) no major depressive or manic episodes have occurred concurrently with the active-phase symptoms, or 2) if mood episodes have occurred dur- ing active-phase symptoms, they have been present for a minority of the total duration of the active and residual periods of the illness. D. The disturbance is not attributable to the physiological effects of a substance (e.g., a drug of abuse, a medication) or another medical condition. Specify if: With good prognostic features: This specifier requires the presence of at least two of the following features: onset of prominent psychotic symptoms within 4 weeks of the first noticeable change in usual behavior or f unctioning; confusion or perplexity; good premorbid social and occupational functioning; and absence of blunted or flat affect. Without good prognostic features: This specifier is applied if two or more of the above features have not been present. Specify if: With catatonia (refer to the criteria for catatonia associated with another mental disor- der, pp. 119–120, for definition). Coding note: Use additional code 293.89 (F06.1) catatonia associated with schizo- phreniform disorder to indicate the presence of the comorbid catatonia. Specify current severity: Severity is rated by a quantitative assessment of the primary symptoms of psychosis, including delusions, hallucinations, disorganized speech, abnormal psychomotor be-
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including delusions, hallucinations, disorganized speech, abnormal psychomotor be- havior, and negative symptoms. Each of these symptoms may be rated for its current severity (most severe in the last 7 days) on a 5-point scale ranging from 0 (not present) to 4 (present and severe). (See Clinician-Rated Dimensions of Psychosis Symptom Severity in the chapter “Assessment Measures.”) Note: Diagnosis of schizophreniform disorder can be made without using this severity specifier. Note: For additional information on Associated Features Supp orting Diagnosis, Develop- ment and Course (age-related factors), Culture-Related Diag nostic Issues, Gender-Related Diagnostic Issues, Differential Diagnosis, and Comorbidity, see the corresponding sec- tions in schizophrenia. Diagnostic Features The characteristic symptoms of schizophreniform disorder are identical to those of schizo- phrenia (Criterion A). Schizophreniform disorder is distinguish ed by its difference in du- ration: the total duration of the illness, includ ing prodromal, active, and residual phases, is at least 1 month but less than 6 months (Crite rion B). The duration requirement for schizo- phreniform disorder is intermediate between th at for brief psychotic disorder, which lasts more than 1 day and remits by 1 month, and schizophrenia, which lasts for at least 6 months. The diagnosis of schizophreniform disorder is made under two conditions. 1) when an ep- isode of illness lasts between 1 and 6 months and the individual has already recovered, and 2) when an individual is symptomatic for less than the 6 months’ duration required for the diagnosis of schizophrenia but has not yet recovered. In this case, the diagnosis should
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the diagnosis of schizophrenia but has not yet recovered. In this case, the diagnosis should be noted as “schizophreniform disorder (provisional)” because it is uncertain if the indi- vidual will recover from the disturbance within the 6-month period. If the disturbance per- sists beyond 6 months, the diagnosis should be changed to schizophrenia.
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98 Schizophrenia Spectrum and Other Psychotic Disorders Another distinguishing feature of schizophren iform disorder is the lack of a criterion requiring impaired social and occupational functioning. While such impairments may po- tentially be present, they are not necessary for a diagnosis of schizophreniform disorder. In addition to the five symptom domain areas identified in the diagnostic criteria, the assessment of cognition, depression, and mani a symptom domains is vital for making crit- ically important distinctions between the va rious schizophrenia spectrum and other psy- chotic disorders. Associated Features Supporting Diagnosis As with schizophrenia, currently there are no laboratory or psychometric tests for schizo- phreniform disorder. There are multiple brain regions where ne uroimaging, neuropa- thological, and neurophy siological research has indicated abnormalities, but none are diagnostic. Prevalence Incidence of schizophreniform disorder across sociocultural settings is likely similar to that observed in schizophrenia. In the United States and other developed countries, the in- cidence is low, possibly fivefold less than th at of schizophrenia. In developing countries, the incidence may be higher, especially for th e specifier “with good prognostic features”; in some of these settings schizophreniform disorder may be as common as schizophrenia. Development and Course The development of schizophreniform disorder is similar to that of schizophrenia. About one-third of individuals with an initial di agnosis of schizophreniform disorder (provi- sional) recover within the 6-mo nth period and schizophreniform disorder is their final di- agnosis. The majority of the remaining two-th irds of individuals will eventually receive a diagnosis of schizophrenia or schizoaffective disorder. Risk and Prognostic Factors Genetic and physiological. Relatives of individuals with schizophreniform disorder
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Genetic and physiological. Relatives of individuals with schizophreniform disorder have an increased risk for schizophrenia. Functional Consequences of Schizophreniform Disorder For the majority of individuals with schizo phreniform disorder who eventually receive a diagnosis of schizophrenia or schizoaffective disorder, the functional consequences are similar to the consequences of those disorder s. Most individuals ex perience dysfunction in several areas of daily functioning, such as sc hool or work, interperso nal relationships, and self-care. Individuals who recover from schi zophreniform disorder ha ve better functional outcomes. Differential Diagnosis Other mental disorders and medical conditions. A wide variety of mental and medical conditions can manifest with psychotic sympto ms that must be considered in the differ- ential diagnosis of schizophreniform disord er. These include psychotic disorder due to another medical condition or its treatment; delirium or major neurocognitive disorder; substance/medication-induced psychotic diso rder or delirium; depressive or bipolar disorder with psychotic features; schizoaffectiv e disorder; other specified or unspecified bi- polar and related disorder; depressive or bipola r disorder with catatonic features; schizophre-
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Schizophrenia 99 nia; brief psychotic disorder; delusional diso rder; other specified or unspecified schizo- phrenia spectrum and other psychotic diso rder; schizotypal, schizoid, or paranoid personality disorders; autism spectrum disorder; disorders presenting in childhood with disorganized speech; attention-deficit/hype ractivity disorder; ob sessive-compulsive dis- order; posttraumatic stress diso rder; and traumatic brain injury. Since the diagnostic criteria for schizophreniform disorder and schizophrenia differ primarily in duration of illness, the discussion of the differential diagnosis of schizophre- nia also applies to schizophreniform disorder. Brief psychotic disorder. Schizophreniform disorder differs in duration from brief psy- chotic disorder, which has a duration of less than 1 month. Schizophrenia Diagnostic Criteria 295.90 (F20.9) A. Two (or more) of the following, each present for a significant portion of time during a 1-month period (or less if successfully treated). At least one of these must be (1), (2), or (3): 1. Delusions. 2. Hallucinations. 3. Disorganized speech (e.g., frequent derailment or incoherence). 4. Grossly disorganized or catatonic behavior. 5. Negative symptoms (i.e., diminished emotional expression or avolition). B. For a significant portion of the time since the onset of the disturbance, level of function- ing in one or more major areas, such as work, interpersonal relations, or self-care, is markedly below the level achieved prior to the onset (or when the onset is in childhood or adolescence, there is failure to achieve expected level of interpersonal, academic, or occupational functioning).
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or occupational functioning). C. Continuous signs of the disturbance persist for at least 6 months. This 6-month period must include at least 1 month of symptoms (or less if successfully treated) that meet Cri- terion A (i.e., active-phase symptoms) and may include periods of prodromal or residual symptoms. During these prodromal or residual periods, the signs of the disturbance may be manifested by only negative symptoms or by two or more symptoms listed in Criterion A present in an attenuated form (e.g., odd beliefs, unusual perceptual experiences). D. Schizoaffective disorder and depressive or bipolar disorder with psychotic features have been ruled out because either 1) no major depressive or manic episodes have occurred concurrently with the active-phas e symptoms, or 2) if mood episodes have occurred during active-phase symptoms, they have been present for a minority of the total duration of the active and residual periods of the illness. E. The disturbance is not attributable to the physiological effects of a substance (e.g., a drug of abuse, a medication) or another medical condition. F. If there is a history of autism spectrum disorder or a communication disorder of child- hood onset, the additional diagnosis of schizophrenia is made only if prominent delu- sions or hallucinations, in addition to the other required symptoms of schizophrenia, are also present for at least 1 month (or less if successfully treated). Specify if: The following course specifiers are only to be used after a 1-year duration of the disorder and if they are not in contradiction to the diagnostic course criteria. First episode, currentl y in acute episode: First manifestation of the disorder meet- ing the defining diagnostic symptom and time criteria. An acute episode is a time pe- riod in which the symptom criteria are fulfilled.
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100 Schizophrenia Spectrum and Other Psychotic Disorders First episode, currently in partial remission: Partial remission is a period of time during which an improvement after a previous episode is maintained and in which the defining criteria of the disorder are only partially fulfilled. First episode, currently in full remission: Full remission is a period of time after a previous episode during which no disorder-specific symptoms are present. Multiple episodes, currently in acute episode: Multiple episodes may be deter- mined after a minimum of two episodes (i.e., after a first episode, a remission and a minimum of one relapse). Multiple episodes, currently in partial remission Multiple episodes, currently in full remission Continuous: Symptoms fulfilling the diagnostic symptom criteria of the disorder are remaining for the majority of the illness course, with subthreshold symptom periods be- ing very brief relative to the overall course. Unspecified Specify if: With catatonia (refer to the criteria for catatonia associated with another mental disorder, pp. 119–120, for definition). Coding note: Use additional code 293.89 (F06.1) catatonia associated with schizophrenia to indicate the presence of the comorbid catatonia. Specify current severity: Severity is rated by a quantitative assessment of the primary symptoms of psychosis, including delusions, hallucinations, disorganized speech, abnormal psychomotor be- havior, and negative symptoms. Each of these symptoms may be rated for its current severity (most severe in the last 7 days) on a 5-point scale ranging from 0 (not present) to 4 (present and severe). (See Clinician-Rated Dimensions of Psychosis Symptom Severity in the chapter “Assessment Measures.”)
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Severity in the chapter “Assessment Measures.”) Note: Diagnosis of schizophrenia can be made without using this severity specifier. Diagnostic Features The characteristic symptoms of schizophrenia involve a range of cogn itive, behavioral, and emotional dysfunctions, but no single sympto m is pathognomonic of the disorder. The di- agnosis involves the recognition of a constellation of sign s and symptoms associated with impaired occupational or social functioning. Individuals with the disorder will vary sub- stantially on most features, as schizophrenia is a heterogeneous clinical syndrome. At least two Criterion A symptoms must be present for a significant portion of time during a 1-month period or longer. At least one of these symptoms must be the clear pres- ence of delusions (Criterion A1), hallucinati ons (Criterion A2), or disorganized speech (Criterion A3). Grossly disorganized or ca tatonic behavior (Criterion A4) and negative symptoms (Criterion A5) may also be present. In those situations in which the active- phase symptoms remit within a mo nth in response to treatment, Criterion A is still met if the clinician estimates that they would have persisted in the absence of treatment. Schizophrenia involves impairment in one or more major areas of functioning (Crite- rion B). If the disturbance begins in childhood or adolescence, the expected level of func- tion is not attained. Co mparing the individual with unaffected siblings may be helpful. The dysfunction persists for a substantial period du ring the course of the disorder and does not appear to be a direct result of any single feature. Avolition (i.e., reduced drive to pursue goal-directed behavior; Criterion A5) is linked to the social dysfunction described under Criterion B. There is also strong evidence for a relationship betw een cognitive impairment
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Criterion B. There is also strong evidence for a relationship betw een cognitive impairment (see the section “Associated Features Supporting Diagnosis” for this disorder) and func- tional impairment in individuals with schizophrenia.
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Schizophrenia 101 Some signs of the disturbance must persist for a continuous period of at least 6 months (Criterion C). Prodromal symptoms often prec ede the active phase, and residual symp- toms may follow it, characterized by mild or subthreshold forms of hallucinations or delusions. Individuals may express a variety of unusual or odd beliefs that are not of de- lusional proportions (e.g., ideas of reference or magical thinking); they may have unusual perceptual experiences (e.g., sensing the pres ence of an unseen person); their speech may be generally understandable but vague; and their behavior may be unusual but not grossly disorganized (e.g., mumbling in public). Negative symptoms are common in the pro- dromal and residual phases and can be seve re. Individuals who had been socially active may become withdrawn from previous routines . Such behaviors are often the first sign of a disorder. Mood symptoms and full mood episodes ar e common in schizophrenia and may be con- current with active-phase symptomatology. However, as distinct from a psychotic mood dis- order, a schizophrenia diagnosis requires the presence of delusions or hallucinations in the absence of mood episodes. In addition, mood ep isodes, taken in total, should be present for only a minority of the total duration of th e active and residual periods of the illness. In addition to the five symptom domain areas identified in the diagnostic criteria, the assessment of cognition, depression, and mani a symptom domains is vital for making crit- ically important distinctions between the va rious schizophrenia spectrum and other psy- chotic disorders. Associated Features Supporting Diagnosis Individuals with schizophrenia may display inappropriate affect (e.g., laughing in the ab- sence of an appropriate stimulus); a dysphoric mood that can take the form of depression,
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anxiety, or anger; a disturbed sleep pattern (e.g., daytime sleeping and nighttime activity); and a lack of interest in eating or food refu sal. Depersonalization, derealization, and so- matic concerns may occur and sometimes reac h delusional proportions. Anxiety and pho- bias are common. Cognitive deficits in schi zophrenia are common and are strongly linked to vocational and functional impairments. Thes e deficits can include decrements in declar- ative memory, working memory, language function, and other executive functions, as well as slower processing speed. Ab normalities in sensory processing and inhibitory capacity, as well as reductions in attention, are also found. Some individuals with schizophrenia show social cognition deficits, including deficits in the ability to infer the intentions of other people (theory of mind), and may attend to and then interpret irrelevant events or stimuli as meaningful, perhaps leading to th e generation of explanatory delusions. These impairments frequently persist during symptomatic remission. Some individuals with psychosis may lack insi ght or awareness of their disorder (i.e., anosognosia). This lack of “insight” includes unawareness of symptoms of schizophrenia and may be present throughout the entire cour se of the illness. Unawareness of illness is typically a symptom of schizophrenia itself rath er than a coping strategy. It is comparable to the lack of awareness of neurological deficits following brain damage, termed anoso- gnosia. This symptom is the most common predicto r of non-adherence to treatment, and it predicts higher relapse rates, increased numb er of involuntary trea tments, poorer psycho- social functioning, aggression, and a poorer course of illness. Hostility and aggression can be associated with schizophrenia, although spontaneous
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Hostility and aggression can be associated with schizophrenia, although spontaneous or random assault is uncommon. Aggression is more frequent for younger males and for individuals with a past history of violence, non-adherenc e with treatment, substance abuse, and impulsivity. It should be noted that the vast majority of persons with schizo-
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individuals with a past history of violence, non-adherenc e with treatment, substance abuse, and impulsivity. It should be noted that the vast majority of persons with schizo- phrenia are not aggressive and are more frequently victimized than are individuals in the general population. Currently, there are no radiological, laborato ry, or psychometric tests for the disorder. Differences are evident in multiple brain regions between groups of healthy individuals
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102 Schizophrenia Spectrum and Other Psychotic Disorders and persons with schizophrenia, including ev idence from neuroimaging, neuropatholog- ical, and neurophysiological studies. Differences are also evident in cellular architecture, white matter connectivity, and gray matter volume in a vari ety of regions such as the pre- frontal and temporal cortices. Reduced overall brain volume has been observed, as well as increased brain volume reduction with age. Brain volume reductions with age are more pronounced in individuals with schizophrenia than in healthy individuals. Finally, indi- viduals with schizophrenia appear to differ from individuals withou t the disorder in eye- tracking and electrophysiological indices. Neurological soft signs common in individuals with schizophrenia include impairments in motor coordination, sensory integration, and motor sequencing of complex movements; left-right confusion; and disinhibition of associated mo vements. In addition, minor phys- ical anomalies of the face and limbs may occur. Prevalence The lifetime prevalence of schizophrenia appears to be approximately 0.3%–0.7%, al- though there is reported variation by race/ethnicity, across countries, and by geographic origin for immigrants and children of immigr ants. The sex ratio differs across samples and populations: for example, an emphasis on nega tive symptoms and longer duration of dis- order (associated with poorer outcome) show s higher incidence rates for males, whereas definitions allowing for the inclusion of more mood symptoms and brief presentations (associated with better outcome) show equivalent risks for both sexes. Development and Course The psychotic features of schizophrenia typically emerge between the late teens and the mid-30s; onset prior to adolescence is rare. The peak age at onset for the first psychotic ep-
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isode is in the early- to mid-20s for males and in the late-20s for females. The onset may be abrupt or insidious, but the majority of individuals manifest a slow and gradual develop- ment of a variety of clinically significant signs and symptoms. Half of these individuals complain of depressive symptoms. Earlier age at onset has traditionally been seen as a pre- dictor of worse prognosis. However, the effect of age at onset is likely related to gender, with males having worse premorbid adjustment, lower educational achievement, more prominent negative symptoms and cognitiv e impairment, and in general a worse out- come. Impaired cognition is common, and altera tions in cognition are present during de- velopment and precede the emergence of psycho sis, taking the form of stable cognitive impairments during adulthood. Cognitive impairments may persist when other symptoms are in remission and contribute to the disability of the disease. The predictors of course and outcome are la rgely unexplained, and course and outcome may not be reliably predicted. The course appears to be favorable in about 20% of those with schizophrenia, and a small number of indi viduals are reported to recover completely. However, most individuals with schizophrenia still require formal or informal daily living supports, and many remain chronically ill, with exacerbations and remissions of active symptoms, while others have a course of progressive deterioration. Psychotic symptoms tend to diminish over the life course, perhaps in association with normal age-related declines in dopamine activity. Negative symptoms are more closely re- lated to prognosis than are positive symptoms and tend to be the most persistent. Further- more, cognitive deficits associated with the illness may not improve over the course of the illness. The essential features of schizophrenia are th e same in childhood, but it is more diffi-
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The essential features of schizophrenia are th e same in childhood, but it is more diffi- cult to make the diagnosis. In children, delu sions and hallucinations may be less elaborate than in adults, and visual hallucinations are more common and should be distinguished
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cult to make the diagnosis. In children, delu sions and hallucinations may be less elaborate than in adults, and visual hallucinations are more common and should be distinguished from normal fantasy play. Disorganized speec h occurs in many disorders with childhood onset (e.g., autism spectrum disorder), as does disorganized behavior (e.g., attention-deficit/
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Schizophrenia 103 hyperactivity disord er). These symptoms should not be attributed to schizophrenia with- out due consideration of the more common disorders of childhood. Childhood-onset cases tend to resemble poor-outcome adult cases, with gradual onset and prominent negative symptoms. Children who later receive the diag nosis of schizophrenia are more likely to have experienced nonspecific emotional-behavioral distur bances and psychopathology, intellectual and language alterati ons, and subtle motor delays. Late-onset cases (i.e., onset after age 40 ye ars) are overrepresent ed by females, who may have married. Often, the course is ch aracterized by a predom inance of psychotic symptoms with preservation of affect and soci al functioning. Such late-onset cases can still meet the diagnostic crit eria for schizophrenia, but it is not yet clear whether this is the same condition as schizophrenia diagnosed prio r to mid-life (e.g., prior to age 55 years). Risk and Prognostic Factors Environmental. Season of birth has been linked to the incidence of schizophrenia, in- cluding late winter/early spring in some locations and summer for the deficit form of the disease. The incidence of schizophrenia and related disorders is high er for children grow- ing up in an urban environment an d for some minority ethnic groups. Genetic and physiological. There is a strong contribution for genetic factors in deter- mining risk for schizophrenia, although most individuals who have been diagnosed with schizophrenia have no family history of psyc hosis. Liability is conferred by a spectrum of risk alleles, common and rare, with each allele contributing only a small fraction to the to- tal population variance. The risk alleles identi fied to date are also associated with other mental disorders, including bipolar disorder, depression, an d autism spectrum disorder.
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mental disorders, including bipolar disorder, depression, an d autism spectrum disorder. Pregnancy and birth complications with hypoxia and greater paternal age are associated with a higher risk of schizophrenia for the developing fetus. In addition, other prenatal and perinatal adversities, including stress, in fection, malnutrition, maternal diabetes, and other medical conditions, have been linked wi th schizophrenia. However, the vast major- ity of offspring with these risk factors do not deve lop schizophrenia. Culture-Related Diagnostic Issues Cultural and socioeconomic factors must be considered, particularly when the individual and the clinician do not share the same cult ural and socioeconomic background. Ideas that appear to be delusional in one culture (e.g., witchcraft) may be commonly held in another. In some cultures, visual or auditory halluci nations with a religious content (e.g., hearing God’s voice) are a normal part of religious experience. In addition, the assessment of dis- organized speech may be made difficult by lin guistic variation in narrative styles across cultures. The assessment of affect requires sens itivity to differences in styles of emotional expression, eye contact, and body language, wh ich vary across cultures. If the assessment is conducted in a language that is differen t from the individual’s primary language, care must be taken to ensure that alogia is not rela ted to linguistic barriers. In certain cultures, distress may take the form of hallucinations or pseudo -hallucinations and overvalued ideas that may present clinically similar to true psychosis but are normative to the pa- tient’s subgroup. Gender-Related Diagnostic Issues A number of features distinguish the clinical expression of schizophrenia in females and males. The general incidence of schizophrenia tends to be slightly lower in females, par-
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males. The general incidence of schizophrenia tends to be slightly lower in females, par- ticularly among treated cases. The age at onset is later in females, with a second mid-life peak as described earlier (see the section “D evelopment and Course” for this disorder). Symptoms tend to be more affect-laden am ong females, and there are more psychotic
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peak as described earlier (see the section “D evelopment and Course” for this disorder). Symptoms tend to be more affect-laden am ong females, and there are more psychotic symptoms, as well as a greater propensity for psychotic symptoms to worsen in later life.
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104 Schizophrenia Spectrum and Other Psychotic Disorders Other symptom differences include less freque nt negative symptoms and disorganization. Finally, social functioning tends to remain better preserved in females. There are, how- ever, frequent exceptions to these general caveats. Suicide Risk Approximately 5%–6% of individuals with schizophrenia die by suicide, about 20% attempt suicide on one or more occasions, and many mo re have significant suicidal ideation. Suicidal behavior is sometimes in response to command hallucinations to harm oneself or others. Suicide risk remains high over the whole lifespan for males an d females, although it may be especially high for younger males with comorb id substance use. Other risk factors include having depressive symptoms or feelings of hopelessness and being unemployed, and the risk is higher, also, in the period after a psychotic episode or hospital discharge. Functional Consequences of Schizophrenia Schizophrenia is associated with significant social and occupational dysfunction. Making educational progress and maintaining employment are frequently impaired by avolition or other disorder manifestatio ns, even when the cognitive sk ills are sufficient for the tasks at hand. Most individuals are employed at a lower level than their parents, and most, par- ticularly men, do not marry or have limited social contacts outside of their family. Differential Diagnosis Major depressive or bipolar disorder with psychotic or catatonic features. The distinc- tion between schizophrenia and major depre ssive or bipolar disorder with psychotic features or with catatonia depends on the te mporal relationship between the mood distur- bance and the psychosis, and on the severity of the depressive or manic symptoms. If de- lusions or hallucinations occur exclusively during a major depressive or manic episode, the diagnosis is depressive or bipolar disorder with ps ychotic features.
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the diagnosis is depressive or bipolar disorder with ps ychotic features. Schizoaffective disorder. A diagnosis of schizoaffective disorder requires that a major depressive or manic episode occur concurrently with the active-phase symptoms and that the mood symptoms be present for a majority of the total duration of the active periods. Schizophreniform disorder an d brief psychotic disorder. These disorders are of shorter duration than schizophrenia as specified in Cr iterion C, which requires 6 months of symp- toms. In schizophreniform disorder, the disturbance is present less than 6 months, and in brief psychotic disorder, symp toms are present at least 1 day but less than 1 month. Delusional disorder. Delusional disorder can be distinguished from schizophrenia by the absence of the other symptoms characteristic of schizophrenia (e.g., delusions, prom- inent auditory or visual halluc inations, disorganized speech, grossly disorganized or cata- tonic behavior, negative symptoms). Schizotypal personality disorder. Schizotypal personality disorder may be distinguished from schizophrenia by subthres hold symptoms that are associ ated with persistent person- ality features. Obsessive-compulsive disorder and body dysmorphic disorder. Individuals with obsessive-compulsive disorder and body dysmorphic disorder may present with poor or absent insight, and the preoccupations may reach delusional proportions. But these disorders are distinguished from schizophren ia by their prominent obsessions, compul- sions, preoccupations with appearance or bo dy odor, hoarding, or body-focused repeti- tive behaviors. Posttraumatic stress disorder. Posttraumatic stress disorder may include flashbacks that have a hallucinatory quality, and hypervigilanc e may reach paranoid proportions. But a trau-
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Schizoaffective Disorder 105 matic event and characteristic symptom features relating to reliving or reacting to the event are required to make the diagnosis. Autism spectrum disorder or communication disorders. These disorders may also have symptoms resembling a psychotic episode but are distinguished by their respective defi- cits in social interaction with repetitive an d restricted behaviors and other cognitive and communication deficits. An individual with autism spectrum disord er or communication disorder must have symptoms that meet fu ll criteria for schizophrenia, with prominent hallucinations or delusions for at least 1 mont h, in order to be diagnosed with schizophre- nia as a comorbid condition. Other mental disorders associated with a psychotic episode. The diagnosis of schizo- phrenia is made only when the psychotic episode is persistent and not attributable to the physiological effects of a subs tance or another medical condit ion. Individuals with a de- lirium or major or minor neurocognitive disorder may present with psychotic symptoms, but these would have a temporal relationship to the onset of cognit ive changes consistent with those disorders. Individuals with subs tance/medication-induced psychotic disorder may present with symptoms char acteristic of Criterion A for schizophrenia, but the sub- stance/medication-induced psyc hotic disorder can usually be distinguished by the chron- ological relationship of subs tance use to the onset and remi ssion of the psychosis in the absence of substance use. Comorbidity Rates of comorbidity with substance-relate d disorders are high in schizophrenia. Over half of individuals with schizophrenia have tobacco use disorder and smoke cigarettes regularly. Comorbidity with anxiety disorder s is increasingly recognized in schizophre- nia. Rates of obsessive-compulsive disorder and panic disorder are elevated in individuals
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nia. Rates of obsessive-compulsive disorder and panic disorder are elevated in individuals with schizophrenia compared with the general population. Schizotypal or paranoid per- sonality disorder may sometimes pr ecede the onset of schizophrenia. Life expectancy is reduced in individual s with schizophrenia because of associated medical conditions. Weight gain, diabetes, metabolic syndrome, and cardiovascular and pulmonary disease are more common in schi zophrenia than in the general population. Poor engagement in health maintenance behavi ors (e.g., cancer screening, exercise) in- creases the risk of chronic disease, but other disorder factors, including medications, life- style, cigarette smoking, and diet, may also play a role. A shared vulnerability for psychosis and medical disorders may explain so me of the medical comorbidity of schizo- phrenia. Schizoaffective Disorder Diagnostic Criteria A. An uninterrupted period of illness during which there is a major mood episode (major depressive or manic) concurrent with Criterion A of schizophrenia. Note: The major depressive episode must include Criterion A1: Depressed mood. B. Delusions or hallucinations for 2 or more weeks in the absence of a major mood epi- sode (depressive or manic) during the lifetime duration of the illness. C. Symptoms that meet criteria for a major mood episode are present for the majority of the total duration of the active and residual portions of the illness. D. The disturbance is not attributable to the effects of a substance (e.g., a drug of abuse, a medication) or another medical condition.
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106 Schizophrenia Spectrum and Other Psychotic Disorders Specify whether: 295.70 (F25.0) Bipolar type: This subtype applies if a manic episode is part of the pre- sentation. Major depressive episodes may also occur. 295.70 (F25.1) Depressive type: This subtype applies if only major depressive epi- sodes are part of the presentation. Specify if: With catatonia (refer to the criteria for catatonia associated with another mental disorder, pp. 119–120, for definition). Coding note: Use additional code 293.89 (F06.1) catatonia associated with schizoaffective disorder to indicate the presence of the comorbid catatonia. Specify if: The following course specifiers are only to be used after a 1-year duration of the disorder and if they are not in contradiction to the diagnostic course criteria. First episode, currentl y in acute episode: First manifestation of the disorder meet- ing the defining diagnostic symptom and time criteria. An acute episode is a time pe- riod in which the symptom criteria are fulfilled. First episode, currently in partial remission: Partial remission is a time period dur- ing which an improvement after a previous episode is maintained and in which the de- fining criteria of the disorder are only partially fulfilled. First episode, currently in full remission: Full remission is a period of time after a previous episode during which no disorder-specific symptoms are present. Multiple episodes, currently in acute episode: Multiple episodes may be deter- mined after a minimum of two episodes (i.e., after a first episode, a remission and a minimum of one relapse). Multiple episodes, currently in partial remission
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minimum of one relapse). Multiple episodes, currently in partial remission Multiple episodes, currently in full remission Continuous: Symptoms fulfilling the diagnostic symptom criteria of the disorder are remaining for the majority of the illness course, with subthreshold symptom periods be- ing very brief relative to the overall course. Unspecified Specify current severity: Severity is rated by a quantitative assessment of the primary symptoms of psychosis, including delusions, hallucinations, disorganized speech, abnormal psychomotor be- havior, and negative symptoms. Each of these symptoms may be rated for its current severity (most severe in the last 7 days) on a 5-point scale ranging from 0 (not present) to 4 (present and severe). (See Clinician-Rated Dimensions of Psychosis Symptom Severity in the chapter “Assessment Measures.”) Note: Diagnosis of schizoaffective disorder can be made without using this severity specifier. Note: For additional information on Developmen t and Course (age-related factors), Risk and Prognostic Factors (environmental risk factors), Culture-Related Diagnostic Issues, and Gender-Related Diagnostic Issues, see th e corresponding sections in schizophrenia, bipolar I and II disorders, an d major depressive disorder in their respective chapters. Diagnostic Features The diagnosis of schizoaffective disorder is based on the assessment of an uninterrupted period of illness during which the individual co ntinues to display active or residual symp- toms of psychotic illness. The diagnosis is usually, but not necessarily, made during the period of psychotic illness. At some time during the period, Criterion A for schizophrenia
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Schizoaffective Disorder 107 has to be met. Criteria B (soc ial dysfunction) and F (exclusion of autism sp ectrum disorder or other communication disorder of childhood onset) for schizophrenia do not have to be met. In addition to meeting Criterion A for schizophrenia, there is a major mood episode (major depressive or manic) (Criterion A for schizoaffective disorder). Because loss of in- terest or pleasure is common in schizophrenia, to meet Criterion A for schizoaffective dis- order, the major depressive episode must in clude pervasive depressed mood (i.e., the presence of markedly diminished interest or pleasure is not sufficie nt). Episodes of de- pression or mania are present for the majority of the total duration of the illness (i.e., after Criterion A has been met) (Criterion C for schi zoaffective disorder). To separate schizoaf- fective disorder from a depressive or bipola r disorder with psychotic features, delusions or hallucinations must be present for at least 2 weeks in the absence of a major mood epi- sode (depressive or manic) at some point duri ng the lifetime duration of the illness (Cri- terion B for schizoaffective disorder). The symptoms must not be attributable to the effects of a substance or another medical condition (Criterion D for schi zoaffective disorder). Criterion C for schizoaf fective disorder specifies that mood symptoms meeting criteria for a major mood episode must be present for the majority of the total duration of the ac- tive and residual portion of the illness. Criter ion C requires the asses sment of mood symp- toms for the entire course of a psychotic illnes s, which differs from th e criterion in DSM-IV,
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which required only an assessment of the curr ent period of illness. If the mood symptoms are present for only a relative ly brief period, the diagnosis is schizophrenia, not schizoaf- fective disorder. When deciding whether an individual’s presentation meets Criterion C, the clinician should review the total duration of psychotic illness (i.e., both active and re- sidual symptoms) and determine when significa nt mood symptoms (untreated or in need of treatment with antidepressant and/or mo od-stabilizing medication) accompanied the psychotic symptoms. This determination requir es sufficient histor ical information and clinical judgment. For example, an individual with a 4-year history of active and residual symptoms of schizophrenia develops depressi ve and manic episodes that, taken together, do not occupy more than 1 year during the 4- year history of psycho tic illness. This presen- tation would not meet Criterion C. In addition to the five symptom domain areas identified in the diagnostic criteria, the assessment of cognition, depression, and mani a symptom domains is vital for making crit- ically important distinctions between the va rious schizophrenia spectrum and other psy- chotic disorders. Associated Features Supporting Diagnosis Occupational functioning is frequently impair ed, but this is not a defining criterion (in contrast to schizophrenia). Restricted social contact and difficulties with self-care are as- sociated with schizoaffective disorder, but negative symptoms may be less severe and less persistent than those seen in schizophrenia. Anosognosia (i.e., poor insight) is also com- mon in schizoaffective disorder, but the deficits in insight may be less severe and perva- sive than those in schizophrenia. Individual s with schizoaffective disorder may be at increased risk for later developing episodes of major depressive disorder or bipolar disor-
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increased risk for later developing episodes of major depressive disorder or bipolar disor- der if mood symptoms contin ue following the remission of symptoms meeting Criterion A for schizophrenia. There may be associated al cohol and other substance-related disorders. There are no tests or biological measures that can assist in making the diagnosis of schizoaffective disorder. Whether schizoaffe ctive disorder differs from schizophrenia
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There are no tests or biological measures that can assist in making the diagnosis of schizoaffective disorder. Whether schizoaffe ctive disorder differs from schizophrenia with regard to associated features such as structural or function al brain abnormalities, cognitive deficits, or geneti c risk factors is not clear. Prevalence Schizoaffective disorder appe ars to be about one-third as common as schizophrenia. Life- time prevalence of schizoaffective disorder is estimated to be 0.3%. The incidence of
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108 Schizophrenia Spectrum and Other Psychotic Disorders schizoaffective disorder is higher in females than in males, mainly due to an increased in- cidence of the depressive type among females. Development and Course The typical age at onset of schizoaffective di sorder is early adulthood, although onset can occur anywhere from adolescence to late in lif e. A significant number of individuals diag- nosed with another psychotic illness initially will receive the diagnosis schizoaffective dis- order later when the pattern of mood epis odes has become more apparent. With the current diagnostic Criterion C, it is expected that the diagnosis for some individuals will convert from schizoaffective d isorder to another disorder as mood symptoms become less prominent. The prognosis for schizoaffective di sorder is somewhat better than the prog- nosis for schizophrenia but worse than the prognosis for mood disorders. Schizoaffective disorder may occur in a variety of temporal patterns. The following is a typical pattern: An individual may have pronounced auditory hallucinations and per- secutory delusions for 2 months before the onset of a prom inent major depressive episode. The psychotic symptoms and the full major depr essive episode are then present for 3 months. Then, the individual recovers completely fr om the major depressive episode, but the psy- chotic symptoms persist for another month be fore they too disappear. During this period of illness, the individual’s symptoms concurre ntly met criteria for a major depressive ep- isode and Criterion A for schizophrenia, and during this same period of illness, auditory hallucinations and delusions were present both before and after the depressive phase. The total period of illness lasted for about 6 mo nths, with psychotic sy mptoms alone present during the initial 2 months, both depressive and psychotic symptoms present during the
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during the initial 2 months, both depressive and psychotic symptoms present during the next 3 months, and psychotic symptoms alone pr esent during the last month. In this in- stance, the duration of the depressive episode was not brief relative to the total duration of the psychotic disturbance, and thus the presen tation qualifies for a diagnosis of schizoaf- fective disorder. The expression of psychotic symptoms across the lifespan is variable. Depressive or manic symptoms can occur before the onset of psychosis, during acute psychotic episodes, during residual periods, and after cessation of psychosis. For example, an individual might present with prominent mood symptoms during the prodromal stage of schizo- phrenia. This pattern is not necessarily indicative of schizoaffective disorder, since it is the co-occurrence of psychotic and mood symptoms that is diagnostic. For an individual with symptoms that clearly meet the criteria for sc hizoaffective disorder but who on further fol- low-up only presents with residual psychoti c symptoms (such as subthreshold psychosis and/or prominent negative symptoms), the diagnosis may be changed to schizophrenia, as the total proportion of psychotic illness compared with mood symptoms becomes more prominent. Schizoaffective diso rder, bipolar type, may be more common in young adults, whereas schizoaffective disorder, depressive type, may be more common in older adults. Risk and Prognostic Factors Genetic and physiological. Among individuals with schizophrenia, there may be an in- creased risk for schizoaffective disorder in first-degree relatives. The risk for schizoaffec- tive disorder may be increased among indivi duals who have a first- degree relative with schizophrenia, bipolar disorder, or schizoaffective disorder. Culture-Related Diagnostic Issues
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Culture-Related Diagnostic Issues Cultural and socioeconomic factors must be considered, particularly when the individual and the clinician do not share the same cultur al and economic backgr ound. Ideas that ap- pear to be delusional in one culture (e.g., witchcraft) may be commonly held in another.
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and the clinician do not share the same cultur al and economic backgr ound. Ideas that ap- pear to be delusional in one culture (e.g., witchcraft) may be commonly held in another. There is also some evidence in the literature for the overdiagnosis of schizophrenia com-
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Schizoaffective Disorder 109 pared with schizoaffective disorder in Afri can American and Hispanic populations, so care must be taken to ensure a culturally appropri ate evaluation that includes both psy- chotic and affective symptoms. Suicide Risk The lifetime risk of suicide for schizophreni a and schizoaffective disorder is 5%, and the presence of depressive symptoms is correlated with a higher risk for suicide. There is ev- idence that suicide rates are higher in North American populations than in European, Eastern European, South American, and Indian populations of individuals with schizo- phrenia or schizoaffective disorder. Functional Consequences of Schizoaffective Disorder Schizoaffective disorder is associated with social and occupational dysfunction, but dys- function is not a diagnostic criterion (as it is for schizophrenia), an d there is substantial variability between individuals diagno sed with schizoaffective disorder. Differential Diagnosis Other mental disorders and medical conditions. A wide variety of psychiatric and med- ical conditions can manifest with psychotic and mood symptoms that must be considered in the differential diagnosis of schizoaffective disorder. Th ese include psychotic disorder due to another medical condition; delirium; major neurocognitive disorder; substance/ medication-induced psychotic disorder or neurocognitive disorder; bipolar disorders with psychotic features; major depressive diso rder with psychotic features; depressive or bipolar disorders with catatonic features; schi zotypal, schizoid, or paranoid personality disorder; brief psychotic disorder; schizophre niform disorder; schizophrenia; delusional disorder; and other specified and unspecified schizophrenia spectrum and other psychotic disorders. Medical conditions and substance use can present with a combination of psy- chotic and mood symptoms, and thus psychoti c disorder due to another medical condition
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chotic and mood symptoms, and thus psychoti c disorder due to another medical condition needs to be excluded. Distinguishing schizoaffective disorder from schizophrenia and from depressive and bipolar diso rders with psychotic features is often difficult. Criterion C is designed to separate schizoaffective di sorder from schizophrenia, and Criterion B is designed to distinguish schizoaffective diso rder from a depressive or bipolar disorder with psychotic features. More specifically, schizoaffective disorder can be distinguished from a depressive or bipolar disorder with psyc hotic features due to the presence of prom- inent delusions and/or hallucinations for at least 2 weeks in the absence of a major mood episode. In contrast, in depressive or bipo lar disorders with psychotic features, the psy- chotic features primarily occur during the mo od episode(s). Because the relative propor- tion of mood to psychotic symptoms may ch ange over time, the appropriate diagnosis may change from and to schizoaffective disord er (e.g., a diagnosis of schizoaffective dis- order for a severe and prominent major depre ssive episode lasting 3 months during the first 6 months of a persistent psychotic illnes s would be changed to schizophrenia if active psychotic or prominent residual symptoms pe rsist over several years without a recurrence of another mood episode). Psychotic disorder due to another medical condition. Other medical conditions and substance use can manifest with a combinat ion of psychotic and mood symptoms, and thus psychotic disorder due to another medical condition needs to be excluded. Schizophrenia, bipolar, and depressive disorders. Distinguishing schizoaffective dis- order from schizophrenia and from depressiv e and bipolar disorders with psychotic fea- tures is often difficult. Criterion C is design ed to separate schizoaffective disorder from
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tures is often difficult. Criterion C is design ed to separate schizoaffective disorder from schizophrenia, and Criterion B is designed to distinguish schi zoaffective disorder from a
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110 Schizophrenia Spectrum and Other Psychotic Disorders depressive or bipolar disorder with psychoti c features. More specifically, schizoaffective disorder can be distinguished from a depressive or bipolar disorder with psychotic features based on the presence of prom inent delusions and/or hallucinations for at least 2 weeks in the absence of a major mood episode. In cont rast, in depressive or bipolar disorder with psychotic features, the psychoti c features primarily occur duri ng the mood episode(s). Be- cause the relative proportion of mood to ps ychotic symptoms may change over time, the appropriate diagnosis may change from and to schizoaffective disorder. (For example, a diagnosis of schizoaffective disorder for a se vere and prominent major depressive episode lasting 3 months during the first 6 months of a chronic psychotic illness would be changed to schizophrenia if active ps ychotic or prominent residual symptoms persist over several years without a recurrence of another mood episode.) Comorbidity Many individuals diagnosed with schizoaffect ive disorder are also diagnosed with other mental disorders, especially substance use d isorders and anxiety disorders. Similarly, the incidence of medical conditions is increase d above base rate for the general population and leads to decre ased life expectancy. Substance/Medication-Induced Psychotic Disorder Diagnostic Criteria A. Presence of one or both of the following symptoms: 1. Delusions. 2. Hallucinations. B. There is evidence from the history, physical examination, or laboratory findings of both (1) and (2): 1. The symptoms in Criterion A developed during or soon after substance intoxication or withdrawal or after exposure to a medication. 2. The involved substance/medication is capable of producing the symptoms in Crite- rion A.
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rion A. C. The disturbance is not better explained by a psychotic disorder that is not substance/ medication-induced. Such evidence of an independent psychotic disorder could in- clude the following: The symptoms preceded the onset of the substance/medication use; the symptoms persist for a substantial period of time (e.g., about 1 month) after the cessation of acute withdrawal or severe intoxication; or there is other evidence of an indepen- dent non-substance/medication-induced psychotic disorder (e.g., a history of recur- rent non-substance/medication-related episodes). D. The disturbance does not occur exclusively during the course of a delirium. E. The disturbance causes clinically significant distress or impairment in social, occupa- tional, or other important areas of functioning. Note: This diagnosis should be made instead of a diagnosis of substance intoxication or substance withdrawal only when the symptoms in Criterion A predominate in the clinical picture and when they are sufficiently severe to warrant clinical attention.
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Substance/Medication-Induced Psychotic Disorder 111 Coding note: The ICD-9-CM and ICD-10-CM codes for the [specific substance/medica- tion]-induced psychotic disorders are indicated in the table below. Note that the ICD-10- CM code depends on whether or not there is a comorbid substance use disorder present for the same class of substance. If a mild substance use disorder is comorbid with the sub- stance-induced psychotic disorder, the 4th position character is “1,” and the clinician should record “mild [substance] use disorder” before the substance-induced psychotic disorder (e.g., “mild cocaine use diso rder with cocaine-induced ps ychotic disorder”). If a m oderate or severe substance use disorder is comorbid with the substance-induced psychotic disor- der, the 4th position character is “2,” and the clinician should record “moderate [substance] use disorder” or “severe [substance] use disorder,” depending on the severity of the co- morbid substance use disorder. If there is no comorbid substance use disorder (e.g., after a one-time heavy use of the substance), then the 4th position character is “9,” and the cli- nician should record only the substance-induced psychotic disorder. Specify if (see Table 1 in the chapter “Substance-Related and Addictive Disorders” for diag- noses associated with substance class): With onset during intoxication: If the criteria are met for intoxication with the sub- stance and the symptoms develop during intoxication. With onset during withdrawal: If the criteria are met for withdrawal from the sub- stance and the symptoms develop during, or shortly after, withdrawal. Specify current severity:
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stance and the symptoms develop during, or shortly after, withdrawal. Specify current severity: Severity is rated by a quantitative assessment of the primary symptoms of psychosis, including delusions, hallucinations, abnormal psychomotor behavior, and negative symptoms. Each of these symptoms may be rated for its current severity (most severe in the last 7 days) on a 5-point scale ranging from 0 (not present) to 4 (present and severe). (See Clinician-Rated Dimensions of Psychosis Symptom Severity in the chap- ter “Assessment Measures.”) Note: Diagnosis of substance/medication-induced psychotic disorder can be made without using this severity specifier.ICD-10-CM ICD-9-CMWith use disorder, mildWith use disorder, moderate or severeWithout use disorder Alcohol 291.9 F10.159 F10.259 F10.959 Cannabis 292.9 F12.159 F12.259 F12.959 Phencyclidine 292.9 F16.159 F16.259 F16.959 Other hallucinogen 292.9 F16.159 F16.259 F16.959 Inhalant 292.9 F18.159 F18.259 F18.959 Sedative, hypnotic, or anxiolytic292.9 F13.159 F13.259 F13.959 Amphetamine (or other stimulant)292.9 F15.159 F15.259 F15.959 Cocaine 292.9 F14.159 F14.259 F14.959 Other (or unknown) substance 292.9 F19.159 F19.259 F19.959
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112 Schizophrenia Spectrum and Other Psychotic Disorders Recording Procedures ICD-9-CM. The name of the substance/medication -induced psychotic disorder begins with the specific substance (e.g., cocaine, de xamethasone) that is presumed to be causing the delusions or hallucinations. The 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 (e.g., dexamethasone), the code for “other substance” should be used; and in cases in which a substance is judged to be an etiological factor but the specific class of sub- stance is unknown, the category “u nknown substance” should be used. The name of the disorder is followed by the sp ecification of onset (i.e., onset during in- toxication, onset during with drawal). Unlike the recording procedures for ICD-10-CM, which combine the substance-induced disorder and substance use disorder into a single code, for ICD-9-CM a separate diagnostic code is given for the substance use disorder. For example, in the case of delusio ns occurring during intoxication in a man with a severe co- caine use disorder, the diagnosis is 292.9 cocaine-induced psychotic disorder, with onset during intoxication. An additional diagnosis of 304.20 severe cocaine use disorder is also given. When more than one subs tance is judged to play a significant role in the development of psychotic symptoms, each should be listed separately (e.g., 292.9 cannabis-induced psy- chotic disorder with onset during intoxicati on, with severe cannabis use disorder; 292.9 phencyclidine-induced psychotic disorder, wi th onset during intoxication, with mild phencyclidine use disorder).
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phencyclidine use disorder). ICD-10-CM. The name of the substance/medication-induced psychotic disorder begins with the specific substance (e.g., cocaine, de xamethasone) that is presumed to be causing the delusions or hallucinations. The diagnostic code is selected from the table included in the criteria set, which is based on the drug class and presence or absence of a comorbid substance use disorder. For substances that do not fit into an y of the classes (e.g., dexa- methasone), the code for “other substance” with no comorbid substance use should be used; and in cases in which a substance is judged to be an etiological factor but the specific class of substance is unknown, the category “unknown substance” wi th no comorbid sub- stance use should be used. When recording the name of the disorder, th e comorbid substance use disorder (if any) is listed first, followed by the word “with,” followed by the name of the substance-induced psychotic disorder, followed by the specification of onset (i .e., onset during intoxication, onset during withdrawal). For example, in the case of delusions occurring during intoxi- cation in a man with a severe cocaine use d isorder, the diagnosis is F14.259 severe cocaine use disorder with cocaine-induced psychotic disorder, with onset during intoxication. A separate diagnosis of the comorbid severe co caine use disorder is not given. If the sub- stance-induced psychotic disorder occurs without a comorbid substance use disorder (e.g., after a one-time heavy use of the substance), no accompanying substance use disor- der is noted (e.g., F16.959 phencyclidine-induce d psychotic disorder, with onset during in-
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toxication). When more than one substance is judged to play a significant role in the development of psychotic symptoms, each should be listed separately (e.g., F12.259 severe cannabis use disorder with cannabis-induced ps ychotic disorder, with onset during intox- ication; F16.159 mild phencyclidine use diso rder with phencyclidine-induced psychotic disorder, with onset during intoxication). Diagnostic Features The essential features of substance/medicati on-induced psychotic disorder are prominent delusions and/or hallucinations (Criterion A) that are judged to be due to the physiolog-
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Diagnostic Features The essential features of substance/medicati on-induced psychotic disorder are prominent delusions and/or hallucinations (Criterion A) that are judged to be due to the physiolog- ical effects of a substance/medication (i.e., a drug of abuse, a medication, or a toxin expo- sure) (Criterion B). Hallucinations that the individual realizes are substance/medication- induced are not included here and instead w ould be diagnosed as substance intoxication
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