IACAPAP Textbook of Child and Adolescent Mental Health

Chapter

A.9

INTRODUCTION

DSM-5
MAJOR CHANGES FOR CHILD AND ADOLESCENT DISORDERS

Julia D Machado, Arthur Caye, Paul J Frick & Luis A Rohde

Julia D Machado
Arthur CayeADHD Outpatient Program,
Hospital de Clínicas de Porto
Alegre, Federal Universityof Rio Grande do Sul, Porto
Alegre, Brazil.
Conflict of interest: nonereported

Paul J Frick PhD
Department of Psychology,
University of New Orleans, New
Orleans, Louisiana, USA.
Conflict of interest: nonereported

Luis A Rohde MD, PhD
Institute for Developmental
Psychiatry for Children andAdolescents, Brazil & ADHD
Outpatient Program, Hospitalde Clínicas de Porto Alegre,
Federal University of Rio
Grande do Sul, Porto Alegre,
Brazil.
Conflict of interest: in the last
three years, speakers’ bureau
and/or consultancies for EliLilly, Janssen-Cilag, Novartis
and Shire; authorship royalties
from Oxford University
This publication is intended for professionals training or practicing in mental health and not for the general public. The opinions
expressed are those of the authors and do not necessarily represent the views of the Editor or IACAPAP. This publication seeks todescribe the best treatments and practices based on the scientific evidence available at the time of writing as evaluated by the authorsand may change as a result of new research. Readers need to apply this knowledge to patients in accordance with the guidelines andlaws of their country of practice. Some medications may not be available in some countries and readers should consult the specific drug
information since not all dosages and unwanted effects are mentioned. Organizations, publications and websites are cited or linked toillustrate issues or as a source of further information. This does not mean that authors, the Editor or IACAPAP endorse their content or
recommendations, which should be critically assessed by the reader. Websites may also change or cease to exist.
©IACAPAP 2013. This is an open-access publication under the Creative Commons Attribution Non-commercial License. Use,
distribution and reproduction in any medium are allowed without prior permission provided the original work is properly cited andthe use is non-commercial. Send comments about this book or chapter to jmreyATbigpond.net.au
Suggested citation: Machado JD, Caye A, Frick PJ, Rohde LA. DSM-5. Major changes for child and adolescent disorders. In Rey
JM (ed), IACAPAP e-Textbook of Child and Adolescent Mental Health. Geneva: International Association for Child and Adolescent
Psychiatry and Allied Professions 2013.

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IACAPAP Textbook of Child and Adolescent Mental HealthClassification is of undeniable importance in human progress, frommere recognition of patterns for easy memorization to complex
scientific classification systems. In most fields of study, the main usesof classification include clarifying relationships among objects or organisms;
facilitating memorization and recall of data; enabling clear communication;
improving predictive power; and stimulating the search for explanations (Volkmar
et al, 2007).

Press and ArtMed; flights andaccommodation from Novartis
and Janssen-Cilag in 2010 totake part in two child psychiatricmeetings. The ADHD andJuvenile Bipolar Disorder
Outpatient Programs received
unrestricted educationaland research support from
Abbott, Eli-Lilly, Janssen-Cilag,
Novartis, and Shire.

Classification systems in child and adolescent psychiatry serve similar
purposes. However, unlike what is increasingly seen in other areas of medicine,the role of classification in prediction and explanation is still limited. Up tonow, the main function of classification in child and adolescent psychiatry has
been to improve the transmission of information by facilitating communication
among professionals in both clinical and research environments. Other uses ofclassification include information that can be useful for treatment and prognosis(Martin & Wolkmar, 2007).

A HISTORICAL PERSPECTIVE
The Diagnostic and Statistical Manual of Mental Disorders (DSM) is oneof the two leading classification systems. Since its first publication in 1952, theDSM has undergone five major revisions until the current one, DSM-5 (American
Psychiatric Association, 2013). DSM-III (American Psychiatric Association, 1980)
is recognized as revolutionary as it improved—operationalized—diagnostic criteria
and, consequently, the reliability of psychiatric diagnoses. DSM-IV, published 14
years later (American Psychiatric Association, 2000), attempted to both harmonize
the DSM with the International Classification of Diseases (ICD) and provide
greater emphasis on the validity of psychiatric diagnoses by conducting systematic
reviews and field trials. However, experience with its use highlighted significant
limitations in both the harmonization with ICD-10 (First, 2009) and the validityof many of the psychiatric diagnoses (Kapur et al, 2012).
DSM-5 was initially hoped to be a paradigm change in psychiatry bylinking diagnosis to pathophysiology. However, the lack of neurobiologicalmarkers with sufficient diagnostic specificity hampered this expectation (Kendler& First, 2010). This challenge was left for others to pursue, such as in the National
Institute of Mental Health (NIMH) Research Domain Criteria (RDoC) initiative(see http://www.youtube.com/watch?v=i8Dh33cMipY). In this context, the main
modifications introduced by DSM-5 can be summarized as follows:
1) A greater emphasis on a dimensional perspective. There has been
considerable criticism of the categorical approach in previous DSM
editions since there is not enough empirical data supporting thepresent/absent symptom cut-off for many disorders, from attentiondeficit/hyperactivity disorder (ADHD) to personality disorders. This
categorical approach has been strongly criticized in that it classifies as
pathologic some normal patterns of behaviour, lowers the detection of
atypical presentations and comorbidities, supports inflexible and contextblind treatment regimens, and weakens statistical power in research.
Incorporating dimensional measures without making it unfriendly
for clinicians was perhaps the most difficult challenge for the DSM-5

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IACAPAP Textbook of Child and Adolescent Mental Healthworkgroups. Dimensionality was introduced in DSM-5 in various ways.
For example, by incorporating crosscutting symptom assessment (Narrow etal, 2013); i.e., using screening questions for 12 dimensions of behaviour
linked to a second level of questions if any of the answers to the initial
screening questions is positive, resembling the traditional review ofsystems in general medicine. This change addresses part of the problem
by capturing different symptom dimensions and potentially increasing
the detection of atypical presentations and comorbidities. Dimensionality
was also strengthened by incorporating severity scales for all disorders andin re-structuring diagnostic criteria for some conditions such as autistic
spectrum disorder and substance use disorders.
2) The structure was modified to increasingly group disorders on the basesof shared etiological factors (Andrews et al, 2009a). In fact, this was the
only major change made based on neurobiological mechanisms. Thus,
obsessive compulsive disorder became independent from the anxiety
disorders; pathological gambling was incorporated into a ‘substancerelated and addictive disorders’ chapter and a new, broader group of‘trauma and stressor related disorders’ was created (see Table A.9.1).
3) A more developmentally oriented approach was adopted—recognizingthat a large proportion of mental disorders begin in childhood oradolescence (Kim-Cohen et al, 2003). This is of particular interest forchild and adolescent psychiatrists and consistent with WHO’s vision thatmental disorders are largely chronic disorders of youth (Guilbert, 2003). All
disorders in DSM-5 are conceptualized in a lifespan perspective. Thus,
the DSM-IV chapter on ‘disorders usually first diagnosed in infancy,
childhood or adolescence’ is no longer in DSM-5. Developmental
considerations have been emphasized particularly in some diagnoses (e.g.,
ADHD, PTSD); though timid, these considerations are stronger than in
DSM-IV. The new organization of chapters in DSM-5 also reflects anattempt to incorporate a lifespan perspective. Disorders usually diagnosedin childhood, like neurodevelopmental disorders, appear first; they arefollowed by those typically diagnosed in adulthood, such as bipolar
disorder; and disorders mostly diagnosed later in life, like neurocognitive
disorders, are located towards the end. A new chapter dealing with‘medication-induced movement disorders and other adverse effects ofmedication’ has been added also.

David Kupfer, MD
Chair of the DSM-5 Task
Force4) The multi-axial structure of DSM-IV has been replaced by a uniaxial
approach. In DSM-IV, a patient’s clinical diagnosis would be described
into three axes: clinical syndrome (e.g., schizophrenia, major depressive
disorder) in axis I; personality disorders in axis II; and general medical
conditions (e.g., HIV, hypothyroidism) in axis III (the phrase ‘general
medical condition’ is replaced in DSM-5 with ‘another medical condition’).
The other two axes were used to report psychosocial stressors and level offunction. Some problems were identified with this structure. First, it useda psychiatry-specific language that was often not understood by other
health professionals, hindering communication. Second, it implied thata psychiatric diagnosis is different from a medical diagnosis. In DSM-

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IACAPAP Textbook of Child and Adolescent Mental Health5, axes I, II and III have been merged to include both psychiatric andrelevant medical diseases. It is expected this change may improve theinteraction between psychiatry and general medicine (Kupfer et al, 2013).
5) Redefinition of diagnostic criteria for several disorders. The approximately
160 members of the 13 workgroups, 6 study groups, and a contingentof almost 300 consultants worked intensively for 5 years reviewing theaccumulated evidence dealing with the validity of diagnostic criteria for
specific disorders. They also assessed findings from secondary data analyses
provided by researchers in different fields and results on reliability andclinical utility from the field trials (Regier et al, 2013). Based on this work,
modifications to diagnostic criteria were proposed taking into accounta careful balance between pros (e.g., increased validity) and cons (e.g.,
artificial increases in prevalence). Like in its recent predecessors, eachdisorder includes supplementary text with, among others, descriptions ofprevalence, course and culture-related issues.

Table A.9.1

Organization of chapters in DSM-5

Neurodevelopmental disorders
Schizophrenia spectrum and other psychotic disorders
Bipolar and related disorders
Depressive disorders
Anxiety disorders
Obsessive-compulsive disorders
Trauma- and stressor-related disorders
Dissociative disorders
Somatic symptom and related disorders
Feeding and eating disorders
Elimination disorders
Sleep-wake disorders
Sexual dysfunctions
Gender dysphoria
Disruptive, Impulse-control, and conduct disorders
Substance-related and addictive disorders
Neurocognitive disorders
Personality disorders
Paraphilic disorders
Other mental disorders
Medication-induced movement disorders and other adverse effects of medication
Other conditions that may be a focus of clinical attention

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IACAPAP Textbook of Child and Adolescent Mental Health6) New diagnoses. There is a misconception that DSM creates disorders.
The DSM includes a new diagnosis only when careful examination ofclinical and research data show that it warrants being considered aseparate diagnostic entity—although, as expected, not all scientists may
agree about it. In fact, DSM-5 includes fewer diagnoses than previous
editions. Examples of new diagnoses in DSM-5 are hoarding disorderand disruptive mood dysregulation disorder (see below). Some diagnoses
had been included already in DSM-IV in an appendix as warranting
further study. Since then, enough data had accumulated to support theiraddition as a diagnosis in DSM-5. Examples are binge eating disorder andpremenstrual dysphoric disorder. It is important to highlight that DSM5 continues to have a chapter for conditions ‘for further study’ (section3), such as attenuated psychosis syndrome, Internet gaming disorder andnonsuicidal self-injury.
Discussion of modifications for all mental disorders in DSM-5 is beyond
the scope of this chapter. We present below the changes more relevant for childand adolescent mental health professionals and those that generated controversy.

Click on the picture to hear
Dr Thomas Insel, director of
the US National Institute of
Mental Health talking about‘Toward a new understandingof mental illness’ (13:04)

NEURODEVELOPMENTAL DISORDERS
The new group of neurodevelopmental disorders is one of the changes inthe DSM structure supported by pathophysiologic features. These are disorders
characterized by a delay or deviation in brain development influencing phenotypicfeatures (Rutter et al, 2006); for example, ADHD, learning disorders, mental
retardation and autistic spectrum disorders (see Table A.9.2). Neverthelessuncertainty remains on what is the best place for some of these conditions. Whilethe inclusion of ADHD under this cluster is based on recent brain imaging data
Table A.9.2

Neurodevelopmental disorders in DSM-5•

Intellectual Disability•

Communication disorders−

Language disorder−

Speech sound disorder (difficulty with speech sound production that prevents
verbal communication)−

Stuttering (childhood-onset fluency disorder)−

Social (pragmatic) communication disorder•

Autism spectrum disorder•

Attention-deficit/hyperactivity disorder•Specific learning disorder•

Motor disorders−

Developmental coordination disorder−

Stereotypical movement disorder−

Tic disorders

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IACAPAP Textbook of Child and Adolescent Mental Healthshowing a delay in cortical maturation (Shaw et al, 2007), it has been argued
that ADHD would fit as comfortably within the disruptive, impulse-control andconduct disorders cluster because of its genetic overlap and similar symptomatology(Andrews et al, 2009b).

Autism spectrum disorders
Autism spectrum disorders (ASD) is a new category in DSM-5 on theunderstanding that three previously separate disorders listed under the pervasive
developmental disorders (PDD) rubric in DSM-IV are better conceptualized
as different levels of severity of one single condition. Thus, DSM-5 introduced
here the concept of spectrum, reinforcing the relevance of dimensionality. ASD
includes the former autistic disorder, Asperger’s syndrome, and PDD not otherwise
specified (NOS). Rett’s syndrome and childhood disintegrative disorder have been
removed from the section.
There are several reasons behind this change. The validity and reliability ofthe different diagnoses under the PDD rubric has been questioned, in particular
the distinction between Asperger’s syndrome and the so-called high-functioning
autism (Howlin, 2003; Mayes et al, 2001). For example, one study assessing
diagnostic features of DSM-IV PDD showed that the best predictor of which
diagnosis individuals would receive was the clinic they were referred to rather
than the individuals’ clinical features (Lord et al, 2012). The DSM-5 workgroupconcluded that previous distinctions between these conditions were the result of
artificially clustering presentations of different levels of severity of the same disorderthat in fact existed as a continuum. The new criteria are potentially more able todifferentiate ASD from normal development and from other psychiatric disorders.

Rett’s syndrome
Rett’s syndrome has been
removed from DSM-5. Thisis a neurodevelopmenal
disorder that affects
girls almost exclusively.
It is characterized bynormal early growth anddevelopment followed bya slowing of development,
loss of purposeful use of
the hands, distinctive hand
movements, slowed brain
and head growth, problems
with walking, seizures,and intellectual disability.
To read more about thiscondition click on this box.

In addition, diagnostic criteria for ASDs were reduced from three totwo core symptom domains:••

Deficits in social communication and social interaction andRestrictive repetitive behaviour, interests and activities.

The social communication and interaction deficits domain was created bymerging two former DSM-IV domains, ‘social’ and ‘communication’ deficits—
based on the premise that they are manifestations of a single set of symptoms.
In other words, these two domains overlap and resulted in double-counting ofsymptoms (Mandy et al, 2012).
The proposed new criteria generated concern among some researchers, themedical community and relatives of sufferers. First, it was claimed that the existing
diagnostic subtypes were clinically useful even without good inter-rater reliability,
and that their criteria should have been improved rather than abandoned since
the DSM-IV subtypes had existed for a relatively short period of time and future
research could improve their validity and reliability (Ghaziuddin, 2010). Second,the extensive body of research on autism built in the last two decades will have
limited value as the changed diagnostic criteria would fundamentally alter these
diagnoses (Singh et al, 2009). For example, one study assessed sensitivity andspecificity of the, at the time, proposed DSM-5 criteria using DSM-IV as thereference (McPartland et al, 2012). Results showed good specificity (95%), as
expected by the Workgroup, but variable sensitivity for different PDD subtypes:
76% for autistic disorder, 25% for Asperger’s disorder, and 28% for PDD-NOS.

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IACAPAP Textbook of Child and Adolescent Mental Health

Table A.9.3

Main changes in pervasive developmental disorders in DSM-5

ChangeMagnitude

Rationale•

Merging the former three pervasive
developmental disorder diagnoses
into one

Major

One single spectrum better describes symptom
presentation, development and response to treatment.

•

Defining two core domains of the
syndrome instead of threeModerate

Two previous domains (impairment in social interaction
and in communication) were considered overlapping.

•

Removing Rett’s Syndrome

Minor

ASD behaviors are present only for a brief period during
development in Rett’s syndrome patients – they can still
be diagnosed with ASD.

•

Removing childhood disintegrative
disorder

Minor

Childhood disintegrative disorder patients exhibit
particular physical symptoms and experience a distinct
pattern of developmental regression

Sensitivity was lower for individuals with above average IQ. The conclusion wasthat DSM-5 produced stricter, higher-threshold criteria that tend to exclude highfunctioning individuals from the diagnosis, consequently precluding their eligibility
for services—even when they may stand to benefit from treatment. However, it isof note that this higher threshold might help to deal with the explosion of PDD
diagnosis in recent years that might be partially fuelled by criteria with less welldefined boundaries (King & Bearman, 2009). The main changes in the pervasive
developmental disorders are summarized in Table A.9.3.

Learning disorders
Important changes have been made in the core diagnostic features oflearning disorders including:••••Merging the 4 previous DSM-IV subtypes into one category and theconsequent modifications of the diagnostic criteria
Change in the minimum age for diagnosis
Creation of the criterion of persistence and resistance to intervention;
andChange in the definition of low achievement.

The decision to consolidate learning disorders into one single category was
based on the lack of evidence to support the mutual exclusiveness, coverage anddevelopmental sensitivity of the DSM-IV learning disorders subtypes. A reviewof the literature conducted by the workgroup found conflicting evidence. The
final decision was made considering not only the evidence available but also theopinion of experts and of professional and advocacy groups. The conclusion was toamalgamate the subtypes under one single category but to maintain developmental
distinctions as specifiers, capturing and even extending the key features of theprevious subtypes (Tannock, 2013). This involved changing criterion A, which
now requires only one out of six heterogeneous symptoms (inaccurate or slow
reading, difficulty understanding what is read, spelling, writing, number sense
or calculation, mathematical reasoning). Furthermore, the minimum age for

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IACAPAP Textbook of Child and Adolescent Mental Healthdiagnosis that was specific for each one of the DSM-IV subtypes has been widened:
symptoms should begin during the school-age years but may not become fully
manifest until the demands on the affected academic skills exceed the individual’s
limited capabilities.
Criterion A also includes a new—controversial—requirement that symptoms
need to persist despite the provision of interventions that target the difficulties.
This model, response to intervention, is already integrated into the requirements
used by many school systems in the US. It is based on the premise that somechildren diagnosed with learning disorders are actually inadequately instructed,
encourages an emphasis on prevention, and decreases the number of false positives.
However, this has been strongly criticized by some experts: interventions are notstandardized or evidence-based, allowing for misinterpretation of the failure (Waesche et al, 2011). Also, response to the intervention suggests a dichotomous
outcome when in fact it is likely to exist as a continuum (Fletcher & Vaughn,
2009). The few studies available showed poor agreement among different response
to intervention-based definitions of learning disorders (Waesche et al, 2011). This
criterion tends to be ignored for developing countries because interventions are
not widely available. Furthermore, this represents a major rupture in the conceptof nosology—possibly this is the only diagnosis in medicine that requires a negative
response to treatment as a criterion.
The quantification of poor academic achievement has also changed in DSM5. According to DSM-IV academic skills should be substantially below average
considering the individual’s chronological age, measured intelligence, and ageappropriate education. The criteria now define low academic skills as substantiallybelow average according to assessment tests standardized for age, education andculture, and exclude the diagnosis in the presence of intellectual disability. Thus,
DSM-5 excludes the need for a discrepancy between IQ and academic achievement
for learning disorders. There is evidence this approach may lead to lower diagnostic
accuracy, overdiagnosing learning disorders in high IQ children while doing the
opposite in those with low IQ (Francis et al, 2005). A shortcoming of both DSMIV and DSM-5 criteria is their lack of applicability in many countries, especiallyin the underdeveloped world, since they rely on nonexistent standardized tests
adapted for each language and culture.
Table A.9.4

Main changes in criteria for learning disorders

ChangeMagnitude

Rationale•

Merging the four subtypes into one
unique category with specifiers

Moderate

Uncertainty about boundaries between the previously
separated diagnoses.

•

Requiring a lack of response totreatment

Moderate

Learning disorder might actually be the result of
inadequate instruction.

•

Removing the discrepancy between
IQ and academic achievement

Moderate

The IQ-corrected approach had lower diagnostic
accuracy.

•

Requirement that symptoms bepresent for 6 months

DSM-5 A.9

Minor

Exclusion of temporary cases

8

IACAPAP Textbook of Child and Adolescent Mental HealthCriteria for learning disorder did not formally specify duration of symptoms
in DSM-IV. DSM-5 now requires symptoms to be present for at least 6 months.
This is not expected to have much impact (Tannock, 2013). See a summary of themain changes in Table A.9.4.

Attention-deficit/hyperactivity disorder
There have not been important changes to the diagnostic construct—the
18 core symptoms remain the same. Changes include age of onset, symptom
threshold for adults, and the removal of autism spectrum disorder from theexclusion criteria. Less significant changes include modifications to the ADHD
subtypes and inclusion of more developmentally appropriate examples for the 18
core symptoms.
Considerable evidence challenges the validity of the criterion that required
onset of impairment before age 7 in DSM-IV. Several studies have found nodifference in phenotypic presentation, neuropsychological impairment, course,
severity or treatment response between children with onset before and after 7 yearsof age (Kieling et al, 2010). However, ADHD, as a neurodevelopmental disorder,
requires an age of onset limit. Barkley and Brown (2008) and Kessler et al (2006)
provided evidence that an age of onset before 12 would capture around 95% of
the cases in their studies. Although the medical and general community voiced
concerns that such a change may artificially increase the prevalence of ADHD—
though evidence from population studies does not support this claim (Polanczyk
et al, 2010)—DSM-5 accepted this change and requires that symptoms be present
before 12 years of age.
The number of symptoms needed for the diagnosis of ADHD in adults has
been reduced to five in both inattention and hyperactivity/impulsivity dimensions.
Table A.9.5

Summary of main changes in ADHD criteria

ChangeMagnitude

Rationale

Minor

Shared etiology•

Listing ADHD in theneurodevelopmental disorders
chapter•

Changing the age of onset criterion
from 7 to 12 years of age•

Lowering the diagnostic threshold for
adults from 6 to 5 symptoms in bothdimensions•

Changing subtypes to current
presentation

Minor

There is not enough evidence of developmental stability
to support subtyping.

•

Changing examples of diagnostic
symptoms

Minor

New examples seek to better fit the symptoms fordifferent developmental stages.

•

Removing ASD from the exclusion
criteria

Moderate

DSM-5 A.9

Moderate

Increased diagnostic sensitivity: several cases (especiallythose with ADHD predominantly inattentive) were notdetected with the old criterion.

Moderate

Increased diagnostic sensitivity

ADHD is often comorbid with ASD; comorbidity is
associated with higher impairment and comorbid ADHD
can be successfully treated with stimulants.

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The DSM-IV field trials included only samples of children and adolescents.
Therefore, the cut-off of six symptoms in DSM-IV is not necessarily optimal for
adults. Barkley and Brown (2008) have shown that this cut-off represents 2.5 to 3
standard deviations above the mean, capturing only the most extreme adult cases.
Using four out of nine symptoms predicts impairment with the best sensitivity andspecificity and is supported by neurobiological data (Hoogman et al, 2012; Matteet al, 2012). However, to be cautious, DSM-5 only lowered the threshold to fivesymptoms.
There is no evidence supporting the exclusion of a diagnosis of ADHD
when ASD is present. In fact ADHD and autism frequently co-exist (Simonoff
et al, 2008), and the presence of ADHD in patients with ASD is associated withdifferent clinical correlates from those found in pure ASD (Rommelse al, 2011).
Also, stimulants may lessen ADHD symptoms in patients with ASD ( Research
Units on Pediatric Psychopharmacology Autism Network, 2005). Table A.9.5
summarises the main changes to the ADHD criteria in DSM-5 and their rationale.

Intellectual Disability
Changes are summarized in Table A.9.6. Intellectual disability is locatedin the neurodevelopmental disorders chapter. In DSM-5 the term intellectual
disability replaces DSM-IV mental retardation (the forthcoming revision of the
ICD proposes to use the term ‘intellectual developmental disorders’ instead). The
expression ‘mental retardation’ has gradually been abandoned both in clinical andacademic settings and among the lay public because of its pejorative connotations.
Other modifications include changes emphasizing a more comprehensive patient
assessment and evaluation of functioning.
Even though IQ testing is encouraged and remains a cornerstone ofassessment, the new criteria emphasize the importance of functioning. IQ measures
Table A.9.6

Main changes in the diagnosis of intellectual disability

ChangeMagnitude

Rationale
Pejorative connotations of ‘mental retardation’.

•‘Mental retardation’ is replaced by‘intellectual disability’.

Minor

Term widely used already in clinic and academic
settings, among advocacy groups and the lay public.
Consistency with the ICD.
IQ less valid at the lower end of the range.

•

Evaluation emphasizes level offunctioning rather than IQ.

Moderate•

Evaluation of adaptive functioning
in three domains: conceptual, socialand practical.

Moderate

Multilevel evaluation can better determine how well an
individual copes with everyday tasks.

•

Define severity levels based on
adaptive functioning rather than IQ

Moderate

Adaptive functioning determines the level of support
required•

Remove age of onset

DSM-5 A.9

Minor

IQ alone insufficient to assess functioning in real-lifesituations and practical tasks.

Variable duration of developmental period

10IACAPAP Textbook of Child and Adolescent Mental Healthare less valid in the lower end of the IQ range; additionally, co-occurring disorders,
practice effects and the ‘Flynn effect’ (increase in average IQ scores worldwide inrecent years) are examples of factors that may influence the diagnosis of intellectual
disability (Kanaya et al, 2003). This approach reinforces the need for an evaluation
by trained professionals (Harris, 2013).
Criterion A concerns deficits in intellectual functioning and general abilities
measured by IQ tests. Individuals with intellectual disability are expected to have
IQ scores two standard deviations or more below the population mean. Criterion
B refers to deficits in adaptive functioning across three domains (not specified inprevious editions of the manual): conceptual (e.g., academic skills); social (e.g.,language, communication, interpersonal skills); and practical (e.g., living skills,
self-care). This multidimensional approach seeks to ensure that diagnosis is madebased on a broad evaluation of the disorder’s impact on functioning instead ofbased solely on specific complaints and intelligence testing.
DSM-5 keeps the traditional severity levels – mild, moderate, severe andprofound – but replaces IQ scores as the defining criterion by the extent of adaptive
dysfunction in the above three domains. While previous editions established anage of onset cut off for diagnosis of less than 18 years, DSM-5 does not give a
specific age but requires symptoms to emerge during childhood or adolescence.
Table A.9.6 summarises the main changes.

DISRUPTIVE, IMPULSE-CONTROL, AND CONDUCTDISORDERS
Conduct disorderDSM-5 has moved conduct disorder and oppositional defiant disorder(ODD) to a new chapter also containing antisocial personality disorder, pyromania,
and kleptomania. The essential diagnostic features of conduct disorder remainunchanged in DSM-5—a repetitive and persistent pattern of behaviour in which
the basic rights of others or major societal rules are violated, as evidenced by thepresence of at least three out of 15 criteria (almost identical to those in DSM-IV)
in the previous 12 months. There were two subtypes of conduct disorder in DSMIV depending on whether onset had been during childhood or during adolescence.
DSM-5 adds a new specifier—with limited prosocial emotions. This specifier goesbeyond the presence of negative behaviour and would reflect an individual’s typical
pattern of emotional and interpersonal functioning in multiple contexts. People
with ‘conduct disorder with limited prosocial emotions’ would display limited
empathy and little concern for the feelings, wishes, and well-being of others as
evidenced by at least two of the following:••••

DSM-5 Implementationand Support
Click on the image toaccess the American
Psychiatric association(APA) website to support theimplementation of DSM-5.
The site includes information
on implementation issues,
answers to frequently asked
questions, lists DSM-5
corrections, and provides amechanism for submitting
questions and feedback.
Researchers and clinicians
can also give feedback on
the usefulness of the online
assessment measures ofcross-cutting symptoms,
disorder severity, personality,and disability. The site also
provides links to educational
webinars about the DSM5. The site will continue toprovide historical information
about the development
process and overall rationale
for changes from DSM-IV.

Lack of remorse and guilt
Callous lack of empathy
Lack of concern about performance (e.g., not motivated or concerned
about performing at school or work)
Shallow or deficient affect (e.g., feelings or emotions expressed areshallow or insincere).

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The belief that youth with conduct disorder are a very heterogeneousgroup considering severity, life course, and presumed aetiology has led to multiple
proposals for subdividing this diagnosis. In fact, decades ago, DSM-III alreadyincluded two subtypes: socialized/undersocialized, and aggressive/non-aggressive.
There was some evidence suggesting that the undersocialized and aggressive
subtypes represented more severe and difficult to treat groups. However, definitions
were not clear enough and were often misused, leading to the discontinuation ofthis subdivision in later editions. DSM-IV introduced a metric system of severity(i.e., depending on the number of symptoms present), but most clinicians andresearchers ignored it.
The current ‘with limited prosocial emotions’ specifier (similar to the‘callous and unemotional’ construct) is the outcome of several attempts at finding
interpersonal, affective and behavioural features of psychopathy in children andadolescents. Several personality dimensions have been identified in people with
antisocial behaviour but limited prosocial emotions seems the most robust (Fricket al, 2000). Further, these symptoms appear to detect a more severe, aggressive
and stable group, and are an independent risk factor for antisocial outcomes inadulthood (Burke et al, 2007; Frick & Dickens, 2006; Lynam et al, 2007). It
remains to be seen whether this distinction survives the passage of time and thefurnace of clinical practice.

Oppositional defiant disorder
Criteria for ODD have not changed substantially in DSM-5. To highlightthat ODD has both emotional and behavioural symptomatology, criteria
are now grouped into:•

Angry/irritable mood•Argumentative/defiant behaviour, and•Vindictiveness.

Also, the requirement that ODD should not be diagnosed if conductdisorder is present has been removed on the basis that ODD symptoms seem
to predict important outcomes, especially emotional disorders, independently of
conduct disorder (Stringaris & Goodman, 2009).
A third change has been made to specify the frequency needed for abehaviour to be considered symptomatic of ODD—given that many behaviours
associated with ODD occur commonly in normally developing children andadolescents. For children under 5 years of age, the behaviour should occur mostdays for a period of at least six months, with the exception of ‘vindictiveness’. For
individuals 5 years or older, the behaviour should occur at least once per week forat least six months, again with the exception of ‘vindictiveness’.
The final change adds a severity rating to the criteria to reflect researchshowing that, although children who display ODD symptoms in only one setting
are at risk for both current and future impairment, the degree of pervasivenessof symptoms across settings is an important indicator of severity (Frick & Nigg,2012).

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TRAUMA- AND STRESSOR-RELATED DISORDERS‘Trauma- and stressor-related disorders’ is a new diagnostic group in DSM5. It contains disorders included in other chapters in DSM-IV, such as anxiety
disorders and adjustment disorders. This new class includes reactive attachment
disorder, disinhibited social engagement disorder, posttraumatic stress disorder (PTSD),
acute stress disorder and adjustment disorders. All these conditions require exposure
to a traumatic or stressful event but vary in the expression of psychological distressfollowing this exposure. In fact, the rationale for grouping these disorders into asingle class is based on the recognition that their common feature is significant
distress following exposure to a traumatic or stressful event.

Posttraumatic stress disorder
Diagnosis of PTSD has undergone important changes, especially concerningpreschool children. Recent research has focused on the different expression ofmental disorders across the lifespan. PTSD is the first disorder in DSM-5 to havea separate set of diagnostic criteria according to developmental stage.
Preschool children are exposed to potentially traumatic events such as
abuse, witnessing interpersonal violence, motor vehicle accidents, natural disasters,
Table A.9.7. Summary of DSM-5 diagnostic criteria for PTSD for children aged 6 years andyounger
To make a diagnosis of PTSD in children aged 6 years and younger, the four criteria listed below
should be met, symptoms must have been present for more than one month, cause clinicallysignificant distress or impairment and are not the manifestation of a substance or medical condition.
A

Personal exposure to actual or threatened death, serious injury, or sexual violence such as by directly experiencing
the traumatic event; witnessing the event as it occurred to others, especially primary caregivers; or learning thatthe traumatic event occurred to a parent or caregiver.

B

Presence of one or more of the following symptoms associated with the traumatic event:• Recurrent, involuntary and intrusive distressing memories of the event• Distressing dreams related to the event• Dissociative reactions (e.g., flashbacks) in which the child feels or acts as if the event was recurring• Intense or prolonged distress when exposed to internal or external cues that symbolize or resemble an aspectof the event• Marked reaction to reminders of the eventC

One or more of the following symptoms:• Persistent avoidance of activities, places, people or situations that remind the event• Negative cognitions (frequent fear, guilt, shame, decreased participation in activities, withdrawal)

D

•
•
•
•
•

DSM-5 A.9

Alterations in arousal and reactivity associated with the traumatic event as evidenced by two or more of thefollowing: Irritability and angry outbursts with little or no provocation
Hypervigilance
Exaggerated startle response
Concentration problems
Sleep disturbance.

13

IACAPAP Textbook of Child and Adolescent Mental Healthwar, dog bites and invasive medical procedures, to name just a few. Thus, thisyoung population would be expected to be at risk for PTSD. However, according
to DSM-IV criteria the prevalence of PTSD among pre-schoolers is much lower
than in older children and adults (Scheeringa et al, 2011). Lower rates may be
attributable to real differences in prevalence, possibly because of children’s
immature perceptions of events, or to low sensitivity of current criteria to detect
the peculiarities of PTSD’s developmental manifestations.
Aware of the latter possibility, researchers proposed alternatives to the
DSM-IV criteria and conducted a series of studies on young children in orderto evaluate the modified criteria and their effects (Scheeringa et al, 1995; 2003;
2012; Task Force on Research Diagnostic Criteria, 2003). It was concluded thatwhen a developmentally-sensitive set of criteria was used approximately three toeight times more children qualified for PTSD compared to when using DSM-IV(Scheeringa et al, 2011; 2012). As a consequence, the DSM-5 task force decided
not only to change PTSD criteria but also to create a PTSD subtype for preschool
children: PTSD for children 6 years and younger. Diagnostic criteria are summarizedin Table A.9.7.

PTSD is the first disorder in
DSM-5 to have a separate
set of diagnostic criteria
according to developmental
stage.

PTSD criteria for older children, adolescents and adults are almost
unchanged from DSM-IV. The distinction between acute and chronic PTSD has
been eliminated due to the scarce evidence available, insufficient to justify theutility of this distinction. A subtype, —‘with dissociative symptoms’—has been
added. Individuals who qualify for this subtype meet the criteria for PTSD andexperience recurrent symptoms of depersonalisation or derealisation.

Reactive attachment disorder and disinhibited social
engagement disorder
The DSM-IV diagnosis of ‘reactive attachment disorder’ is a clinical entitywith low prevalence and, until recently, poorly studied. Research in the last decade
highlights deficits in the diagnostic criteria and substantial changes were suggested(Zeanah & Gleason, 2010). While reactive attachment disorder in DSM-IV was
divided into inhibited and disinhibited subtypes, DSM-5 defines these subtypes as
separate disorders: reactive attachment disorder and disinhibited social engagement
disorder, respectively. The two disorders share the requirement for neglect—definedas the absence of adequate caregiving during childhood—which is considered thecausal factor, resulting in the limitation of a young child’s opportunity to form
adequate attachments. However, the two disorders also differ in important ways,
from clinical manifestations to life course, response to intervention and clinicalcorrelates, which support their being considered separate conditions.

DEPRESSIVE DISORDERS
Disruptive mood dysregulation disorder‘Disruptive mood dysregulation disorder’ is a new diagnosis in DSM-5.
Before describing its core features, it is important to clarify the rationale behind
this controversial decision. In recent years, the field of child and adolescent mental
health has experienced growing debate regarding diagnoses of bipolar disorder.
Rates of bipolar disorder in children and adolescents have increased much faster

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Table A.9.8

Diagnostic criteria for disruptive mood dysregulation disorder•

The four criteria listed below should be met:
− Severe, recurrent, disproportionate temper outbursts− On average, three or more times per week.
− Temper outbursts are inconsistent with developmental level.
− Between outbursts, mood is persistently irritable or angry, most of the day and nearly every day.

•Onset of symptoms must be before age 10•

Symptoms must have been present for 12 or more months•

Symptoms must not be absent for three or more consecutive months•

Children must be between 6 and 18 years of age•

Symptoms should be present in at least two of three settings (home, school, social situations) and are severe in atleast one setting•

Symptoms are not better explained by another mental disorder•

Symptoms are not the manifestation of a substance or medical condition•

Full symptom criteria for manic/hypomanic episode have not been met for more than one day•

Behaviours do not occur solely during an episode of major depressive disorderthan in adults (Blader & Carlson, 2007; Moreno et al, 2007), accompanied by
growing prescription of antipsychotic drugs to this population (Olfson et al, 2006).
Possible explanations include a real increase in prevalence and improvement in caseidentification; however, some studies have attributed such high rates to changes
in diagnostic practices, with children who lack traditional manic symptoms being
diagnosed as bipolar on the basis of alternative symptoms – mainly, irritability(Leibenluft, 2011).
The diagnosis of ‘severe mood dysregulation’ emerged a decade ago(Leibenluft et al, 2003), to describe children and adolescences who present withhyper-arousal along with severe, chronic, non-episodic irritability and frequent
temper outbursts (Zepf & Holtman, 2012). This new diagnosis stimulated
research aimed at both elucidating the key features of severe mood dysregulationand comparing severe mood dysregulation to bipolar disorder.
Evidence from longitudinal studies in community samples suggests thatbipolar disorder and severe mood dysregulation have a different course. While
youths with bipolar disorder show a high correlation with lifetime bipolar disorder,
studies did not find an association between severe mood dysregulation and thelater development of bipolar disorder (Brotman et al, 2006; Stringaris et al, 2009);
instead, children with severe mood dysregulation tend to develop other psychiatricdisorders later on, especially major depressive disorder, generalized anxiety disorderand dysthymia (Brotman et al, 2006; Stringaris et al, 2009). Studies of referredsamples have shown similar results, with children with bipolar disorder being at
much higher risk for experiencing manic episodes over a median period of 29
months than those with severe mood dysregulation (62% and 1%, respectively)(Stringaris et al, 2010).

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IACAPAP Textbook of Child and Adolescent Mental HealthFamily history data are scarce but corroborate the findings of longitudinalstudies. A small study compared the prevalence of parental bipolar disorder inchildren diagnosed with severe mood dysregulation or bipolar disorder and foundsignificant differences: parental bipolar disorder was present in 33% in the bipolar
disorder group but in only 3% in the severe mood dysregulation group (Brotman
et al, 2007).
Thus, the assumption that severe and chronic irritability represents analternative phenotype or developmental presentation of bipolar disorder isunfounded. There is no scientific support for the diagnosis of mania in youths
who present fundamentally with symptoms of non-episodic irritability.
The evidence also underlines the high burden of severe mood dysregulation.
The disorder has a lifetime prevalence of about 3% in community samples, while
the prevalence of bipolar disorder is no more than 0.1% (Brotman et al, 2006).
Additionally, impairment, as measured by Children’s Global Assessment Scale(CGAS) scores, is equivalent in youths diagnosed with bipolar disorder or severemood dysregulation (Leibenluft, 2011).
In DSM-IV the symptom ‘irritability’ was included in the diagnostic criteria
for at least six conditions in children: manic episode, oppositional defiant disorder,
generalized anxiety disorder, dysthymic disorder, posttraumatic stress disorder, andmajor depressive episode. However, none of the above diagnoses applies to children
showing chronic and severe irritability. Therefore, children who meet criteria forsevere mood dysregulation constitute a considerably large population of severely
impaired individuals who do not fit well into any DSM-IV category. The DSM5 task force decided in favour of the validity of severe mood dysregulation by its
inclusion in the classification. A different term—disruptive mood dysregulation
disorder—is used to replace ‘severe mood dysregulation’, which did not seem toreflect fully the complexity of the clinical picture. To highlight the association
between chronic irritability and mood outcomes, disruptive mood dysregulation
disorder has been placed in the mood disorders chapter. Criteria for diagnosis of
disruptive mood dysregulation disorder are summarized in Table A.9.8. For a moredetailed description of this condition see also Chapter E.3 of the Textbook.

EMERGING MEASURES AND MODELS
Cross-cutting symptom measures
Some concepts presented in the new Section III have special interest for childand adolescent mental health. As mentioned above, the introduction of the level1 cross-cutting symptom measures (dimensional) is relevant. The parent/guardianrated version was created for assessment of children and adolescents (6-17 yearsof age); a self-rated version is also available for adolescents. The rationale behind
its development is to provide an instrument for clinicians to perform a kind ofsystems review, as done in general medicine. For this reason, the idea is to use
the measure at the beginning of the clinical assessment, which may help to direct
clinicians to domains where specific diagnosis should be assessed. The instrument

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Table A.9.9 Cross-cutting and symptom measures
Click on the table to find the DSM-5 assessment measures. These measures can be reproduced
without permission by researchers and by clinicians for use with their patients.
•

Level 1 Cross-Cutting Symptom Measures−−•

Level 2 Cross-Cutting Symptom Measures for Parents of Children Ages 6–17
−
−
−
−
−
−
−
−
−

•

−
−
−
−
−
−
−
−

Somatic Symptom—Child Age 11–17 (Patient Health Questionnaire 15 Somatic Symptom Severity Scale[PHQ-15])
Sleep Disturbance—Child Age 11–17 (PROMIS—Sleep Disturbance—Short Form)
Depression—Child Age 11–17 (PROMIS Emotional Distress—Depression—Pediatric Item Bank)
Anger—Child Age 11–17 (PROMIS Emotional Distress—Calibrated Anger Measure—Pediatric)
Irritability—Child Age 11–17 (Affective Reactivity Index [ARI])
Mania—Child Age 11–17 (Altman Self-Rating Mania Scale [ASRM])
Anxiety—Child Age 11–17 (PROMIS Emotional Distress—Anxiety—Pediatric Item Bank)
Repetitive Thoughts and Behaviors—Child Age 11–17 (Adapted from the Children’s Florida Obsessive
Compulsive Inventory [C-FOCI] Severity Scale)
Substance Use—Child Age 11–17 (Adapted from the NIDA-Modified ASSIST)

Disorder-Specific Severity Measures for Children Ages 11–17
−
−
−
−
−
−
−
−
−
−

•

Somatic Symptom—Parent/Guardian of Child Age 6–17 (Patient Health Questionnaire 15 Somatic
Symptom Severity Scale [PHQ-15])
Sleep Disturbance—Parent/Guardian of Child Age 6–17 (PROMIS—Sleep Disturbance—Short Form)
Inattention—Parent/Guardian of Child Age 6–17 (Swanson, Nolan, and Pelham, version IV [SNAP-IV])
Depression—Parent/Guardian of Child Age 6–17 (PROMIS Emotional Distress—Depression—Parent ItemBank)
Anger—Parent/Guardian of Child Age 6–17 (PROMIS Emotional Distress—Calibrated Anger Measure—
Parent)Irritability—Parent/Guardian of Child Age 6–17 (Affective Reactivity Index [ARI])
Mania—Parent/Guardian of Child Age 6–17 (Adapted from the Altman Self-Rating Mania Scale [ASRM])
Anxiety—Parent/Guardian of Child Age 6–17 (Adapted from PROMIS Emotional Distress—Anxiety—
Parent Item Bank)
Substance Use—Parent/Guardian of Child Age 6–17 (Adapted from the NIDA-Modified ASSIST)

Level 2 Cross-Cutting Symptom Measures for Children Ages 11–17−•

DSM-5 Parent/Guardian-Rated Level 1 Cross-Cutting Symptom Measure—Child Age 6–17
DSM-5 Self-Rated Level 1 Cross-Cutting Symptom Measure—Child Age 11–17

Severity Measure for Depression—Child Age 11–17 (PHQ-9 modified for Adolescents [PHQ-A]—Adapted)
Severity Measure for Separation Anxiety Disorder—Child Age 11–17
Severity Measure for Specific Phobia—Child Age 11–17
Severity Measure for Social Anxiety Disorder (Social Phobia)—Child Age 11–17
Severity Measure for Panic Disorder—Child Age 11–17
Severity Measure for Agoraphobia—Child Age 11–17
Severity Measure for Generalized Anxiety Disorder—Child Age 11–17
Severity of Posttraumatic Stress Symptoms—Child Age 11–17 (National Stressful Events Survey PTSD
Short Scale [NSESS])
Severity of Acute Stress Symptoms—Child Age 11–17 (National Stressful Events Survey Acute Stress
Disorder Short Scale [NSESS])
Severity of Dissociative Symptoms—Child Age 11–17 (Brief Dissociative Experiences Scale [DES-B])

Disorder-Specific Severity Measures Clinician-Rated
−
−
−
−
−
−

DSM-5 A.9

Severity of Autism Spectrum and Social Communication Disorders
Dimensions of Psychosis Symptom Severity
Severity of Somatic Symptom Disorder
Severity of Oppositional Defiant Disorder
Severity of Conduct Disorder
Severity of Nonsuicidal Self-Injury

17IACAPAP Textbook of Child and Adolescent Mental Healthincludes 19 questions scored on a 5-point Likert scale (0 to 4) and six questions
allowing ‘yes’, ‘no’ or ‘don’t know’ answers for substance use and suicidal ideation/
attempt in 12 domains. These domains are: somatic symptoms, sleep problems,
inattention, depression, anger, irritability, mania, anxiety, psychosis, repetitive
thoughts and behaviours, substance use and suicidal ideation/suicide attempts.
It is important to note that a positive score in any question allowing
a dimensional answer (score equal or higher than 2–mild, except for questions
related to inattention or psychosis–score equal or higher than 1: slight), or a‘yes’ or ‘don’t know’ answer for questions on substance use and suicidal ideation/
attempt indicates the need for a more comprehensive assessment. This assessment
might include the use of the new DSM-5 level 2 cross-cutting symptom measures,
based on well validated instruments used in child mental health (e.g., SNAP-IV
for inattention and hyperactivity; NIDA modified ASSIST for substance use).
These measures are listed in Table A.9.9, are available in the website and you may
reproduce them for clinical and research use without permission.
Section III also deals with cultural assessment providing an updated versionof the DSM-IV outline for cultural formulation and introducing the cultural
formulation interview. Although this instrument was tested only in adults, childmental health professionals might benefit from including a cultural formulationin the assessment of their patients since the impact of cultural context in thedevelopment or expression of mental health symptoms during childhood andadolescence is tremendous (Canino & Alegria, 2008).

Attenuated psychosis syndrome
Section III is also the home for provisional diagnoses needing more research(‘conditions for further study’). For child and adolescent psychiatry, a very relevant
diagnosis in this section is attenuated psychosis syndrome. The DSM-5 (American
Psychiatric Association, 2013) proposes the following diagnostic criteria for thissyndrome:•

Attenuated symptoms of delusions, hallucinations, or disorganizedspeech with relatively intact reality testing•

Frequency (at least once a week in the last month) or severity causes
distress or disability that requires clinical attention•

Symptoms must have begun or worsened in the last year and are notbetter explained by any major mental disorder or effect of a medical
condition or substance use•

Has never met criteria for psychotic disorder.

Previous research suggests that a significant proportion of individuals withthese phenotypic characteristics progress to full psychotic disorders (Ruhrmann etal, 2010) and intervention might modify this progression (e.g., Amminger et al,2010). However, the risk of extending the frontiers of psychiatric diagnoses too
far, since the majority of these cases do not seem to ever progress to a full blown
psychotic disorder, and the inherent risks of antipsychotic treatment decreased theinitial enthusiasm to include this diagnosis in the main part of the manual.

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MODIFICATIONS BEYOND THE SCOPE OF THISCHAPTER
Several diagnoses pertinent to child and adolescent psychiatry not discussed
here were reallocated due the elimination of the DSM-IV chapter on diagnoses firstevidenced in childhood and adolescence. As mentioned above, this gives a higher
developmental slant to the classification, acknowledging that a large proportion of
the mental disorders have their roots in childhood and adolescence. Motor disorders(e.g., developmental coordination disorder) and stereotyped movement disorders(e.g., tic disorders) have been moved to the chapter on neurodevelopmental
disorders; separation anxiety disorder to the chapter on anxiety disorders; pica
and rumination to the chapter on feeding and eating disorders; and enuresis andencopresis form a new chapter on elimination disorders.
In addition, minor adjustments have been made in several diagnoses
to incorporate a developmental perspective. For example, the criteria for social
anxiety disorder emphasizes that the anxiety in children must occur also wheninteracting with peers and not only in interactions with adults, and that fear andanxiety provoked by social situations in children might be expressed by behaviouralsymptoms like crying and tantrums. In obsessive-compulsive disorder, the criteria
specify that children might have compulsions without recognizing a clear aimof reducing or preventing anxiety, distress or the occurrence of a dreaded event.
Because of the dearth of research on the diagnosis of intermittent explosive disorderin young children and the potential difficulty in distinguishing the outbursts
associated with this disorder from normal temper tantrums, a minimum age of 6
years (or equivalent developmental level) is now required to make this diagnosis.

CONCLUSION
In our view, DSM-5 represents the best evidence-based classification ofmental disorders currently available. Although the initial, optimistic goal was toproduce a document that would represent a paradigm shift in psychiatry, this has
fallen short due to the lack of evidence in many areas. The real process that has
occurred is one of iteration—each DSM version is an attempt to get closer tothe latent construct of mental disorders (Kendler & First, 2010)—and the formal
acknowledgement that psychiatric classification will need to continue evolving andupdating.
For child and adolescent psychiatry, there has been some progress in thesense that a developmental perspective begins to emerge, criteria for relevant
and prevalent disorders have improved in light of new evidence, and some newdisorders are recognized. Yet, much work remains to be done before an aetiologyand pathophysiology-based classification system is built.

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