72 COF NIOklsifiksi RM
72 COF NIOklsifiksi RM
72 COF NIOklsifiksi RM
OF MENTAL RETARDATION
CONTENTS
Publication of this supplement was supported by contract NIH72-C-563 from the National Institute of Child Health and
Human Development, National Institutes of Health.
SUPPLEMENT
The changing attitudes of psychiatrists, pediatricians, and obstetricians toward the mentally retarded stem in part from this new conceptualization of the problem and the growing conviction that even where
"cures" are not possible, informed treatment of the individual and his
family can significantly aid life adjustment. To capitalize more fully on
this burgeoning interest, these disciplines need more precise information on hazards to fetal development, symptomatology and treatment
potentials for specific diagnostic conditions, and the values and limitations of psychological test measurements. Furthermore, to keep abreast
of new discoveries and program developments, these disciplines must
share a terminology and language that permit communication. Our
failure in this latter area has seriously handicapped efforts of professionals from different countries to learn from one another.
The World Health Organization, mindful of these deficiencies and of
our increasing fund of knowledge, has embarked upon a series of
seminars to develop an international scheme for the diagnosis, classification, and reporting of statistics in psychiatric disorders, including
mental retardation. This effort comes at a most opportune time. Comparative data among countries on the incidence and prevalence of
mental retardation and the factors with which specific conditions are
associated are not highly reliable. Although there are significant variations in prenatal care, population homogeneity, disease control, degree
of environmental deprivation, and other factors causative or contributory to mental retardation, reported statistical differences may be more
artifactual than real. Differences in the definition and conceptualization
of mental retardation, inadequacies and variations in classification
schemes used, confusion of terminology, and cultural variability in
demands and expectations for human performance are only a few of the
artifacts that preclude valid comparisons. Within and among countries,
meaningful planning for the retarded cannot be accomplished until
these issues are resolved.
The 1969 seminar, cosponsored by the World Health Organization
and the National Institute of Child Health and Human Development,
was a milestone in the realization of these goals. It is clear that the
complex issues confronted will require continuing attention, but meaningful dialogue has begun and a sounder base for assessing the extent
and diversity of this problem is being established. Community planners
and professional practitioners should profit from this activity, but the
ultimate beneficiaries and the raison d'etre of the seminar will be the
mentally retarded and their families.
MICHAEL J. BEGAB, P H . D .
GERALD D. LAVECK, M.D.
cent of the participants giving the same coding for the level of retardation. The only
patient over whom there was appreciable disagreement was a six-week-old boy with
chromosomal abnormalities. It was agreed
that it was not possible to make a meaningful
assessment of intelligence in infancy and that
judgments on level of retardation in very
young children could only be approximate.
Participants were unanimous in agreeing that
the greatest difficulties in assessing retardation existed for individuals from minority
groups, from cultures other than those in
which the participants were living, and from
socially deprived communities. Whereas
intelligence tests are of great value in assessing level of retardation, they should never be
used in isolation from clinical considerations
of social-adaptive functioning. When
individuals come from cultures other than
those used for the standardization of intelligence quotient (IQ) tests, the tests are of
limited value.
ly that the categories of "mild," "moderate," ry of "associated and etiologic factors" use,
"severe," and "profound" be retained. How- where appropriate, ICD codings from other
ever, in view of the advice that IQ scores sections. The organization of this method of
should never be the sole measure of degree of coding needs further consideration and it may
retardation, it was recommended that IQ be necessary to provide special codings for
levels that define the categories be omitted definite neurological disorders that do not
from the category headings. Instead, the constitute a clearly defined disease of a recterms should be carefully defined in the ognized type. A working party needs to be
Manual on Psychiatric Disorders and Classi- set up to determine how this should best be
fications. Where appropriate, the IQ ranges done. Alternatives to be considered by the
proposed by the Expert Committee should be working party are that the fourth digits
used as a guide instead of the current limits should merely indicate the presence of an asICD-8 suggests.3 However, the IQ limits sociated physical condition that would then
should constitute just one aspect of the defini- be coded under its ICD number, or that the
tion of categories that should include a care- provision for coding such disorders under the
ful description of the degree of handicap in fourth digits be revised to provide a more
social and adaptive terms.
satisfactory system. The list of available
The category "unspecified mental retarda- terms in ICD-8 should be reviewed to ensure
tion" (315 in ICD-8) should be retained but that all diagnoses required for the satisfactoinstructions to coders should indicate very ry classification of mental retardation were
clearly that it be used as sparingly as pos- available and that added provision be made
sible; it is intended solely for patients whose where necessary. The Seminar recommended
current level of intellectual functioning can- that the terms describing conditions comnot be assessed either by standardized tests monly found and reported in the classificaor by clinical judgments (e.g., a newborn).
tion of mental retardation be brought toAssociated or etiological organic factors. gether in a glossary accompanying the
As the case history exercise showed, the classification.
fourth-digit coding for mental retardation
Occasionally more than one associated
proved to be quite unreliable. This is partly organic condition may be present. For exbecause it demands a knowledge of the etiol- ample, one of the patients in the exercise had
ogy of the retardation, which is often lacking diabetes, as well as epilepsy, but only a small
due to the pathogenesis of mental retardation number of the participants coded diabetes. It
being only imperfectly understood. Further- was recommended that, as a rule, the diagmore, nine of the fourth digits combine into nosis of the condition most closely associated
groups a larger number of conditions repre- with the pathogenesis of mental retardation
sentative of many areas of ICD-8, so that as a be recorded. Where feasible, and where the
statement of etiology, they are inadequate. patient's condition demands this for purposes
Moreover, clinicians or coders who work of medical care, more than one diagnosis
mainly with Section V of ICD-8 do not al- should be entered on the second axis. (The
ways have the complete manual available to problems of dealing with data involving
them; hence they may be unable to code cor- multiple coding present no difficulties in
rectly conditions other than those specifically modern computer technology, but coding
mentioned as inclusions of the fourth digits as more than one diagnosis may present problisted, and in some cases, they may fail to re- lems to the personnel involved in maintaining
cord relevant information on diagnosis.
records systems.) Where there are no organic
The Seminar recommended that these dif- features associated with the patient's reficulties be eliminated by making the catego- tardation, this fact should be recorded on the
second axis.
Associated or etiological psychosocial factors. Problems of intellectual retardation
arise not infrequently in relation to psychosocial factors, and the Seminar considered
it desirable that there be provisions for the
coding of such factors. The provision and
definition of categories of psychosocial
Seminar on Psychiatric Diagnosis, Classification, and Statistics, that dealing with child
psychiatry (4). This would require that for
each patient, the following four types of
information would be recorded: 1) degree of
mental handicap, 2) etiological or associated
biological or organic factors, 3) associated
psychiatric disorder, and 4) psychosocial factors. For each patient, all four types of
information would be routinely reported,
instead of only the degree of mental handicap.
Degree of mental retardation. In the assessment of the degree of mental retardation,
relevant information about the sociocultural
background of a patient and his social and
adaptive functioning must be taken into account. The grade of mental retardation recommended by the Expert Committee on
Mental Health (8) should be used in the ninth
revision of ICD. These comprise ICD categories 311-314"mild," "moderate," "severe," and "profound" mental retardation,
together with category 315"unspecified
mental retardation." Category 310, "borderline mental retardation," which includes
backwardness, borderline intelligence, deficientia intelligentiae, borderline mental
deficiency, or subnormality, and an IQ range
of 68 to 85 should be replaced by a category
of normal variations in intelligence in ICD-9.
The Seminar departed from the recommendations of the WHO Expert Committee
on Mental Retardation in recommending
that IQ ranges should not be included in the
ICD manual, but rather, should be specified
in an accompanying glossary that would draw
attention to the limitations, as well as to the
usefulness, of IQ data for the assessment of
intellectual handicaps. The glossary should
also stress that in evaluating the grade of
intellectual retardation, social and cultural
background be taken into account.
Organic aspects. The second type of
information to be recorded for each patient
should, at a minimum, consider the principal
organic feature, if any, associated with the
retardation. If no such features are reported,
Summary and Recommendations
this should be recorded. Users should employ
multiple coding on this axis, etiological and
The Ninth Revision of ICD
The Seminar considered alternative ap- other diagnoses being included where approproaches to the problems of classification in priate.
Psychiatric and behavioral aspects. The
mental retardation. It decided in favor of a
scheme compatible with, and derived from, third class of information should include
the proposals recommended by the Third psychiatric symptoms or syndromes catego-
instructions to accompany ICD-9. It welcomed both WHO initiative and the efforts of
national and of professional organizations in
taking steps to provide an acceptable glossary. The group recommended that the glossary should bring together terms commonly
applicable to the mentally retarded and found
in all sections of the ICD, and that the accompanying manual of instructions should
give guidance to users concerning where particular diagnoses should or should not be
placed.
Promoting the Effective Use of the ICD
The Seminar recommended that WHO
should consult with government agencies and
international and professional organizations
about steps that might be taken to promote
the effective use of the ICD in member
countries. These steps include: further diagnostic exercises, national and regional seminars organized on the lines successfully pioneered in the series of WHO seminars on
classification, and short training courses for
medical students and for persons particularly
responsible for coding and classification.
The Need for Field Trials
The Seminar recommended that WHO
should consider as soon as it can the possibility of sponsoring field trials in different
countries in which the proposed classification
would be tried in practice. The results of any
field studies should be reported back to
WHO. It was also recommended that a future meeting be convened to discuss the results of the field trials and also the integration
of the various recommendations of the seminars on different mental disorders.
The Seminar endorsed the recommendations of the Paris Seminar with regard to the
need for an adequate provision of categories
regarding child psychiatric disorders in ICD9.
The 1969 recommendation of the Educational, Scientific, Cultural, and Health
Commission of OAU (7) was noted with approval. The recommendation proposes that
appropriate intelligence tests be developed
for different cultures and that tests developed
for one culture should not be applied without
modification in very different cultures. The
Seminar considered that the same issue might
apply to different cultures within one
country.
REFERENCES
1. World Health Organization: Report of the Sixth
Seminar on Standardization of Psychiatric Diagnosis, Classification, and Statistics. Geneva, WHO,
1971
2. Shepherd M, Brooke EM, Cooper JE, et al: An
experimental approach to psychiatric diagnosis. Acta Psychiat Scand 44 (suppl 201), 1968
3. Astrup C, Odegard O: Continued experiments in
psychiatric diagnosis. Acta Psychiat Scand 46:180209, 1970
4. Rutter M, Lebovici S, Eisenberg L, et al: A tri-axial
classification of mental disorders in childhood. An
international study. J Child Psychol Psychiat
10:41-61, 1969
5. Averbuch ES, Melnik EM, Serebrjakova ZN, et al:
Diagnosis and Classification of Psychiatric Disease
in Old Age. Leningrad, Institute of Psychiatry, 1968
6. World Health Organization: International Classification of Diseases, 8th revision. Geneva, WHO, 1968
7. Educational, Scientific, Cultural, and Health
Commission, Organization of African Unity: Resolution on psycho-technical tests. Passed at the first
ordinary session, Addis Ababa, Ethiopia, June
30-July4, 1969
8. Organization of services for the mentally retarded.
WHO Techn Rep Ser 392, 1968
9. Heber R: A manual on terminology and classification in mental retardation, 2nd ed. Amer J Ment
Defic 65 (monograph suppl), April 1961
APPENDIX 1
List of P a r t i c i p a n t s
M e m b e r s of Nuclear G r o u p
Dr. Jack R. Ewalt, Bullard Professor of Psychiatry, Harvard Medical School, and Superintendent, Massachusetts Mental Health Center,
74 Fenwood Rd., Boston, Mass. 02115, U.S.A.
Dr. Masaaki Kato, Division of Adult Mental
Health, National Institute of Mental Health,
Konodai, Ichikawa City, Chiba-Ken, Japan
Dr. Morton Kramer, Chief, Biometry Branch,
National Institute of Mental Health, 5454 Wisconsin Ave., Chevy Chase, Md. 20014, U.S.A.
Dr. Ornuly O. Odegard, Medical Superintendent, Overlege Ved Gausted Sykebus, Vinderen,
Oslo, Norway
Dr. H. Rotondo, Professor of Psychiatry, San
Fernando Medical School, San Marcos University, Lima, Peru6
Dr. Michael L. Rutter, Institute of Psychiatry,
De Crespigny Park, Denmark Hill, London
S.E. 5, England
Dr. Raymond Sadoun, Directeur de Recherche,
Institut National de la Sante et de la Recherche
Medicale, 3 rue Leon-Bonnat, 75 Paris 16e,
France
Dr. Z.N. Serebrjakova, Chief Specialist in Psy-
toms, motor dysfunctions, cultural conformity, and reading and arithmetic skills. The
utilization of all dimensions in the AAMD
classification system provides, in addition
to basic typology, a reasonably adequate
profile of the patient.
Controversial Issues of Diagnosis
Sociocultural Retardation
The first of the two special issues we wish
to discuss pertains to sociocultural retardation, a category frequently used in the United
States but not in some other countries. In the
AAMD classification two diagnostic categories are applicable to this group within the
major class of "mental retardation due to
uncertain (or presumed psychologic) cause
with the functional reaction alone manifest."
They are: "cultural-familial mental retardation" (code no. 81) and "psychogenic mental
retardation associated with environmental
deprivation" (code no. 82). In DSM-II the
appropriate category is mental retardation
"with psycho-social (environmental) deprivation" (subdivision .8), which includes two
subclasses: "cultural-familial mental retardation" and mental retardation "associated with environmental deprivation." Two
decades ago the diagnoses usually assigned to
the same group of patients were "familial"
and, less frequently, "undifferentiated" mental deficiency (3).
A general description of the patients who
qualify for these diagnoses follows. In most
instances retardation is of a mild degree, with
IQs in the range of 50 to 70. The condition is
usually not diagnosed prior to the individual's
entrance into school, and the overt diagnosis
generally disappears when he reaches
adulthood. Thus most patients are of school
age, i.e., six to 18 years old. An important
cause of the age specificity of the diagnosis
results from the basic clinical definition of
mental retardation in the United States,
which requires that subaverage general intellectual functioning and impairment in adaptive behavior be present concurrently. The
correlation between these two impairments is
highest during school years, when academic
demands make evident deficits that may not
be apparent when the practical skills of the
patient function adequately in the job market.
These generally mildly retarded patients
behavioral scientists, and the syndrome acquired its labels: sociocultural deprivation,
psychosocial deprivation, cultural-familial
retardation, etc. (6).
Two current etiological questions that have
a bearing on diagnostic classification need
discussion. The first involves the role of organic factors in the causation of sociocultural
retardation. The AAMD manual describes
mental retardation due to "uncertain (or
presumed psychologic) cause with the functional reaction alone manifest" as suited only
for those instances of mental retardation that
occur "in absence of any clinical or historical
indication of organic disease or pathology
which could reasonably account for the retarded intellectual functioning" (1, p. 39).
"Cultural-familial mental retardation" and
mental retardation "associated with environmental deprivation," into which groups
patients with sociocultural retardation are
placed, are two subclasses of this major
category. If clinicians were to adhere literally
to the category description, they might not
diagnose anyone as socioculturally retarded
because, in most instances, the history of poor
medical care and the multiple exposures to
somatic noxae in themselves would contradict the requirement of absence of historical
indications of organic disease.
Observations on the longitudinal development of impoverished children and on the
effects of major changes in the mode of rearing favor the psychosocial etiological model.
On the other hand, the uncritical acceptance
of pure functional causation does not take
into account the probable effects of the biomedical traumata. As a consequence, the erroneous conclusion might be drawn that
somatic noxae do not play a role in the
causation of sociocultural retardation.
The second etiologic question involves the
specific roles of the various elements of deprivation. Global conclusions have been
drawn concerning the total effect of deprivation without specific information on the components of deprivation in regard to quality,
quantity, specificity, or timing.
Transcultural studies, in addition to solving diagnostic and classification problems,
have much to offer toward a better understanding of the causation of sociocultural retardation (7). More specifically, much is to be
gained from such studies about the roles of
genetic, somatic, and experiential forces as
this semantic chaos. It states: "'Mental retardation is placed first to emphasize that it is
to be diagnosed whenever present, even if due
to some other disorder" (2, p. 1). The manual's encouragement of multiple psychiatric
diagnoses assures against loss of information.
The decision of the manual might seem arbitrary, but it offers the most for comparability
of biostatistical information from diversified
geographic settings.
4.
5.
6.
7.
REFERENCES
1. Heber R: A manual on terminology and classification in mental retardation, 2nd ed. Amer J Ment
Defic 65 (monograph suppl), April 1961
2. American Psychiatric Association: Diagnostic and
Statistical Manual of Mental Disorders, 2nd ed.
Washington, DC, APA, 1968
3. American Association on Mental Deficiency: Sta-
8.
9.
tistical Manual for the Use of Institutions for Mental Defectives, 3rd ed. New York, National Committee for Mental Hygiene, 1946
Tarjan G: Some thoughts on socio-cultural retardation, in Social-Cultural Aspects of Mental Retardation. Edited by Haywood CH. New York, AppletonCentury-Crofts, 1970, pp 745-758
Tarjan G: The next decade: expectations from the
biological sciences. JAMA 191:226-229, 1965
Eisenberg L: Social class and individual development, in Crosscurrents in Psychiatry and Psychoanalysis. Edited by Gibson RW. Philadelphia, JB
Lippincott Co, 1967, pp 65-88
Cravioto J, Delicarde ER, Birch HG: Nutrition,
growth, and neurointegrative development. Pediatrics 38:319-372, 1966
Eisenberg L: Emotional determinants of mental
deficiency. Arch Neurol Psychiat 80:114-121, 1958
Rutter M: The influence of organic and emotional
factors on the origins, nature and outcome of childhood psychosis. Develop Med Child Neurol 7:518528. 1965
on an international classification of
diseases may be attributed to: 1) differences
in the perception of symptoms, and 2) differences in the inference and interpretation of
the meaning of a symptom in diagnostic
terms. These problems have been amply il-
lustrated by international diagnostic exercises (1) and by a joint exercise of the United
States and the United Kingdom (2).
International uniformity in classification
may be easier to achieve for the disorders
known as mental retardation or mental subnormality than for the psychoses, neuroses,
and character disorders. However, differences
in the concepts and interpretations of disordered behavior between countries must be
c o n s i d e r e d in our a t t e m p t s to r e a c h
agreement on a scheme of classification for
mental retardation. In the British Glossary of
Mental Disorders (3), mental retardation is
divided by degree of severity into six categories; these categories appear to be the same as
those in the eighth revision of the International Classification of Diseases (ICD-8) (4)
and as those in the second edition of the Diagnostic and Statistical Manual of Mental
Disorders (DSM-II) (5). All of these systems
define their subdivisions by estimates of the
levels of retardation expressed in terms of
numerical IQs, as well as in descriptive
phrases ranging from " b o r d e r l i n e " to
"severe." This apparent agreement between
British and U.S. classifications is not firm,
however, since it seems that the manner in
which the level of retardation is assessed and
As a general rule, the children classed as retarded are the victims of disease, constitutional debility
or malnutrition. We find included in our lists some
whether of diseases or
of any other phenomena, will be used
only if it is found useful. To be really useful, a
classification of mental retardation must
serve a variety of purposes: medical (clinical,
genetic, and epidemiological) as well as social (giving guidelines for education and
training and for the planning of services).
A useful classification of mental retardation must accommodate children and adults.
It cannot be too elaborate or no one will use
it; yet it must also be both comprehensive and
compatible with other sections of the eighth
revision of the International Classification of
Diseases (ICD-8) (1).
CLASSIFICATION,
In practice the categories will tend to overlap, but the IQ has some value within the
range of mental retardation both as a diagnostic and a prognostic guide.
Although some of the terms used in both
the ICD-8 (1) and the WHO report (2) to describe the gravity of intellectual handicaps
are perhaps somewhat sanguine, the actual
division by grade makes good clinical sense.
Unfortunately, however, the intention that all
mentally retarded persons should be categorized first by the severity of their intellectual
handicap may be nullified if ICD-8 category
315, "unspecified mental retardation," allows
clinicians to avoid making judgments about
the grade of defect. It would be preferable to
require that particulars of grade of defect be
recorded for all patients classified as mentally retarded, a clinical estimate being given
if no psychometric data were available.
It is therefore recommended that in future
revisions of ICD-8, category 315 should be
omitted. Every person classified as mentally
retarded should be assigned to one or other of
the grades finally agreed upon. (In the case of
infants the grading must be provisional, but
this could be specified in the instructions).
The second axis in ICD-8 (the fourth digit)
is broadly medical, and disorders are divided
into ten categories. Most of these are
reasonably satisfactory, although they are too
broad. (Category .2, for example, lumps together "disorders of metabolism, growth, or
nutrition," and category .5 is for those "with
chromosomal abnormalities.") It is recommended that the second axis be expanded to
two digits to allow for finer differentiation
within each category.
While several categories in the present
fourth-digit subdivision would probably
benefit from the results of discussion among
pediatricians and psychiatrists (e.g., category
.6, mental retardation "associated with prematurity"), there are two categories in particular that are important for behavioral
scientists. These are category .7 ("following
major psychiatric disorder") and category .8
("with psycho-social [environmental] deprivation").
Tarjan and Eisenberg discuss these problems in relation to the classification of mental
retardation in the United States of America
(3). They point out:
It is not uncommon to find children in the
United States who sequentially, and in any combination, acquire a series of diagnoses that include
early infantile autism, mental retardation, childhood schizophrenia, brain damage, early childhood autism, and minimal brain dysfunction. At
times the clinical pictures in these patients are
further complicated by a variety of organic or
functional sensory impairments. . . .
At the present time no firm scientific conclusions can be drawn on the basis of etiologic research [as to which is primary]. The argument, although often heated, therefore remains a philosophic and semantic one (3, p. 17).
Nonetheless, they feel that it is desirable
that children who have both severe emotional
disorders and symptoms of mental retardation be classified in a consistent fashion,
independent of the idiosyncrasies of clinicians. They themselves favor the solution recently advocated by the American Psychiatric Association (APA): "Mental retardation is placed first to emphasize that is to be
diagnosed whenever present, even if due to
some other disorder" (4, p. 1). This decision,
they say, might seem arbitrary, but it offers
the most for comparability of biostatistical
information from diversified geographic settings.
ICD-8 uses one of the fourth digits for this
type of patient (category .7, "following major
psychiatric disorder"). The word "following," however, prejudges the issue; the
term "with major psychiatric disorder"
would be better.
The other type of mental retardation that
Tarjan and Eisenberg discuss (3) is that
usually referred to as sociocultural retardation or, as in ICD-8 category .8, retardation
"with psycho-social (environmental) deprivation." Both the APA and the American
Association on Mental Deficiency (AAMD)
classifications (4, 5) divide sociocultural retardation into two categories: "cultural-familial mental retardation" and either mental
retardation "associated with environmental
deprivation" (APA) or "psychogenic mental retardation" (AAMD).
As Tarjan and Eisenberg point out, both
categories discount the possible effects of
biomedical traumata in bringing about functional retardation. "As a consequence, the
erroneous conclusion might be drawn that
somatic noxae do not play a role in the causation of sociocultural retardation. . . .
[Moreover] global conclusions have been
drawn concerning the total effect of deprivation without specific information on the
components of deprivation in regard to
quality, quantity, specificity, or timing" (3,
p. 16).
ICD-8's categorization of mental retardation "with psycho-social (environmental)
deprivation" is both clumsy and inaccurate.
"Psycho-social (environmental)," like "sociocultural" and "cultural-familial," tells us
no more than the older terms "familial,"
"undifferentiated," "residual," "aclinical,"
"subcultural," or "primary." The term "deprivation" is also a misnomer because it almost inherently contains the value judgment
that anyone who has not had a middle-class
upbringing is somehow deprived.
At the present time there is no way of
disentangling the effects of genetic, biological, and social factors in the causation of sociocultural retardation, and it would seem
more honest to acknowledge that fact
particularly since doing so is likely to lead to
a more reliable, and hence more useful, system of classification. Moreover, the degree of
environmental deprivation is usually judged
by the social circumstances of the family. A
more useful indicator of sociocultural retardation can probably be obtained from twoway tables that record the social class of the
parents and the clinical condition of the child.
Is "psycho-social (environmental) deprivation" worth recording as a cause of mental
defect at the present time?
Compatibility of the Various Sections
of ICD-8
The working group in child psychiatry of
the Third WHO Seminar on Standardization
of Psychiatric Diagnosis held in Paris in 1967
(6) opted for a triaxial system in which the
first axis described the "clinical psychiatric
syndrome," the second the "intellectual level," and the third the "associated or etiological factors." The group made little reference
to the classification of mental retardation in
ICD-8 and assigned mental retardation only
a single number9.0on the clinical axis. It
would be extremely unsatisfactory if diagnosis in child psychiatry were to remain in this
form, for the classification of a patient would
become a function of the place of referral or
the allegiance of a clinician.
This is, of course, the case today. ICD-8's
instructions say: "For primary mortality
REFERENCES
1. World Health Organization: International Classification of Diseases, 8th revision. Geneva, WHO, 1968
2. Organization of services for the mentally retarded.
WHO Techn Rep Ser 392, 1968
3. Tarjan G, Eisenberg L: Some thoughts on the classification of mental retardation in the United States
of America. Amer J Psychiat 128 (May suppl): 1418, 1972
4. American Psychiatric Association: Diagnostic and
Statistical Manual of Mental Disorders, 2nd ed.
Washington, DC, APA, 1968
5. Heber R: A manual on terminology and classification in mental retardation, 2nd ed. Amer J Ment
Defic 65 (monograph suppl), April 1961
6. Rutter M, Lebovici S, Eisenberg L, et al: A triaxial classsification of mental disorders in childhood. An international study. J Child Psychol Psychiat 10:41-61, 1969
7. Wing L: Observations on the psychiatric section of
the International Classification of Diseases and the
British Glossary of Mental Disorders. Psycho Med
1:79-85, 1970
Addendum
The first draft of this paper was sent for comment to a number of colleagues ' in Britain,
Europe, and the United States; many of their suggestions have been incorporated into the text.
Several colleagues felt that two aspects of the
classification system required more discussion.
Dr. Michael Begab, of the National Institute of
Child Health and Human Development, explained
this point of view as follows:
The terms "sociocultural," "culturalfamilial," and "psychosocial deprivation" are
indeed vague categories because of the uncertain
etiological factors underlying. Biological and
genetic contributors may well be involved but
their significance to intellect and function (when
too minimal to measure with current techniques) is yet to be established. In the absence of
more definitive substitutes, I doubt whether we
can do away with the only classification denoting the role of social-environmental circumstances as a cause of retardation. I am concerned this would lead to a conceptualization of
retardation as one deriving from biological determinants alone, thus drawing attention away
from the major dimensions of the problem. Although admittedly imprecise, the emphasis on
index of current performance or future adaptation and we continue to struggle with more
refined measures of behavior. I doubt whether
the clinical classification proposed by the
Working Group on Child Psychiatry would
adequately cover this dimension. While I agree
that compatibility with other sections of the
ICD is important, the sacrifice to mental retardation is, in my view, too great. If we delete
this element because of limitations in
measurement, we may discourage research on
adaptive behavior scales.
The author proposes a new system of classifying the various forms of mental retardation. Using the time of exposure to a pathogenic agent and its etiology as a basis, she
classifies the forms of mental retardation into
three groups: 1) those caused by a pathological condition of the reproductive cells of the
parents; 2) those caused by harmful factors
that act during the intrauterine period; and 3)
those caused by damage to the central nervous system in the perinatal period or in the
first three years of life.
4. Mental retardation with gross underdevelopment of the prefrontal areas of the brain
and that is characterized by specific changes
in personality and motor activity. This form
is of an exogenous etiology.
5. Mental retardation combined with
damage to the subcortical structures. This
form includes underdevelopment of the cognitive faculty and psychopathic behavior.
Need for a New Classification System
However, a different classification scheme
is required for the purposes of clinical practice and for scientific research; preferably,
this would be a classification system based on
the criterion of etiology and pathogenesis. A
criterion of this type for classifying mental
retardation has been used by many eminent
scientists who have studied the condition. Although these classifications reflect very well
the multiplicity of forms of mental retardation encountered in clinical practice, no
generally accepted classification exists. The
lack of unanimity on this question is not difficult to explain if it is remembered that the
very concept of mental retardation is interpreted differently by different workers.
For this reason I thought it necessary, before describing my classification scheme, to
insert a short introduction describing my
point of departure in defining the concept of
mental retardation and in delimiting it from
other clinical manifestations of intellectual
defect. In studying the clinical features of
mental retardation in children and in adolescents, I thought it essential to make a strict
distinction between the following two concepts: 1) an intellectual defect that is the
manifestation of the anomalous development
of the brain, and 2) intellectual disturbances
caused by damage to brain structures that
have already been formed. This distinction
fully accords with the ideas prevalent in teratology; the investigator sets himself the task
of distinguishing a developmental defect from
a disablement caused by damage to an organ
that has already been formed.
The clear-cut differentiation between these
two concepts makes it possible to distinguish
two forms of deficiency that differ in structure. The first, oligophrenic dementia, is a
nonprogressive, pathological condition that
constitutes a form of mental underdevelopment. The second is dementia in the sense of a
6. Laurence-Moon-Biedl syndrome
7. Mental retardation combined with the disturbance of endochondral ossification, with congenital epiphyseal dysplasia
8. Mental retardation combined with ichthyosis
(Rud's syndrome)
9. Some of the nevoid defects with a nonprogressive course
10. Mental retardation caused by damage to the
reproductive cells of the parents through
exposure to exogenous factors, e.g., ionizing
radiation
11. Other genetic forms
Enzymopathic F o r m s of Mental R e t a r d a t i o n
Disturbances of Protein Metabolism
1. Phenylketonuria (blockage of phenylalaninehydroxylase)
2. Maple syrup urine disease (disorders in the
metabolism of valine, isoleucine, and leucine)
3. Hyperlysinemia (disturbed metabolism of lysine)
4. Hypervalinemia (disturbed metabolism of
valine)
5. Histidinemia (disturbed metabolism of histidine)
6. Citrullinuria (disturbed metabolism of citrulline)
7. Homocystinuria (disturbed metabolism of
methionine)
8. Arginosuccinicaciduria (disturbed metabolism
of arginine)
Disturbances of Carbohydrate Metabolism
9. Galactosemia (a disturbance in the action of
the enzyme galactose-L-phosphate-uridyltransferase)
10. Fructosuria (hyperaminoaciduria)
11. Sucrosuria (intolerance of saccharose)
Disturbances in Pigment Metabolism
12. Methemoglobinemia (blockage of the enzyme
needed to convert methemoglobin into hemoglobin)
13. Deficiency of glucuronyl transferase and incapability of converting indirectly acting
bilirubin into the directly acting form (CriglerNajjar syndrome)
Clinical Forms of Mental R e t a r d a t i o n
Caused by C h r o m o s o m a l Aberrations
1. Mental retardation caused by a chromosomal
aberration in Group A chromosomes (ring
chromosomes)
2. Mental retardation caused by an aberration in
Group B that is connected with the deletion of
the short arm of the fourth pair of chromosomes (Wolfs syndrome)
3. Mental retardation connected with deletion of
the short arm of the fifth pair of chromosomes
("Cri du chat" syndrome)
etiological biological factors, there are problems in deciding how to deal with some conditions. Time and testing will show whether
this proves to be the best system.
Application of the Multiaxial
Classification to Adult Patients
Little time was spent at the seminar in discussing classification of mentally retarded
adults. In the same way that the classification
of child psychiatric disorders must be compatible with the scheme used to classify adult
psychiatric disorders, so must the classification of mental retardation be developed in
such a way as to apply to all age groups.
In general, it was agreed that the multiaxial scheme devised for children should be
equally suitable for adult patients. It is just as
necessary in adults as in children to classify
the degree of intellectual impairment, associated biological condition, associated psychosocial factors, and accompanying mental
disorder or clinical psychiatric syndrome.
However, there is a less close relationship
between intellectual level and school attainment. This means that there will be a
more tenuous relationship between IQ and
social handicap in adults and, furthermore,
that many retarded individuals who were
handicapped in childhood will not be retarded
as adults (30). These are matters of detail,
however, and the principles of classification
are the same at all age levels. Whether in fact
the scheme proposed works as well for adults
as for children is an empirical question that
needs to be answered by field trials.
Field Trials
In the past the production and revision of
schemes of classification have all too often
been an armchair exercise, with changes
made largely for diplomatic rather than
scientific reasons. The current series of WHO
seminars is an exciting new endeavor in
which, for the first time, there is a systematic
attempt to assess the strengths and weaknesses of the existing ICD classification by
means of carefully planned case history and
videotape diagnostic studies. These have been
invaluable in highlighting where and why
there were difficulties in classification, and
they have clearly shown which parts of the
classification need revision or deletion be-
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