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unusually tall males with severe acne vulgaris and aggressive sociopathic behavior may have a karyotype of XYY chromosomes is an example of a possible genetic relationship The patient with Turner syndrome in whom competent social adaptation is linked closely to an X chromosome of paternal origin is another example Further, there is no critical evidence to show that deliberate alteration of the familial and social environment or the mental hygienic measures now so popular have ever prevented a neurosis, psychosis, or sociopathy It is during the period of late childhood and adolescence, when the personality is least stable, that transient symptoms, many resembling the psychopathologic states of adult life, are most frequent and dif cult to interpret Some of these disorders represent the early signs of autism, schizophrenia, or manic-depressive disease Others are forerunners of sociopathy But many of the borderline personality traits have a way of disappearing as adult years are reached, so that one can only surmise that they represented either a maturational delay in the attainment of mature social behavior or were expressions of adolescent turmoil, or what has been called adolescent adjustment reaction Many issues that have been touched upon in the preceding discussion are considered more fully in the section on psychiatric disorders (Chaps 56 to 58)

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The symptom complex of incomplete or insuf cient development of mental capacities and associated behavioral abnormalities (variously referred to as mental retardation, mental subnormality, or formerly, oligophrenia) combines many of the developmental abnormalities already discussed Mental retardation stands as the single largest neuropsychiatric disorder in every civilized society Its overall frequency cannot be stated precisely Rough estimates are that in a group of children between 9 and 14 years of age, about 2 percent or slightly more will be unable to pro t from public education or to adapt socially and, when fully grown up, to live independently Using any one of a number of indices of social and psychologic failure, two somewhat overlapping groups are recognized: (1) the mildly impaired (IQ 45 to 70), and (2) the severely impaired, corresponding to an IQ below 45 The second group, also called the pathologic mentally retarded, makes up approximately 10 percent of the subnormal population The more mildly affected rst group, which was formerly referred to as the subcultural, physiologic, or familial mentally retarded, is a much larger group Because of the objectionable implications of the previously used terms idiot, imbecile, and moron, the American Association on Mental De ciency proposed that the mentally retarded be grouped instead into four categories: (1) those with profound de ciency, incapable of self-care (IQ below 25); (2) those with severe de ciency, incapable of living an independent existence and essentially untrainable (IQ 25 to 39); (3) those with moderate de ciency, trainable to some extent (IQ 40 to 54); and (4) those with mild de ciency, who are impaired but trainable and to some extent educable The above terms, while in common use, satisfy neither neurologists nor psychologists because of their generality, embracing as they do any lifelong global de cit in mental capacities The terms convey no information of the particular type(s) of intellectual impairment, their causes and mechanisms, or their anatomic and pathologic bases Moreover, they express only one aspect of impaired mental function the cognitive and ignore the inadequate development of personality, social adaptation, and behavior Spe-.

Extremes of egocentricity lack of understanding of the feelings, needs, and actions of other members of one s social group and an inability to judge one s own strengths and weaknesses stand as the central issues in the development of certain types of neurosis and of borderline personality disorder Such dif culties usually become manifest by adolescence The complete detachment of the child with psychosis, the amorality of the constitutional sociopath, the major disturbances in thinking of the schizophrenic, and the mood swings of the manic-depressive also express themselves in many if not most instances by adolescence and sometimes by late childhood Here one confronts a key problem in psychiatry the extent to which neurosis, sociopathy, and psychosis have their roots in genetically determined personality traits or in derangements in the affective and social life of the individual, consequent to a harmful environment In other words, in what measure are the neuroses and other psychiatric disorders determined by early life experiences and to what extent are they genetically determined The answers to these questions cannot be given with nality Experienced modern clinicians tend to believe that genetic factors are more important than environmental ones The discovery that

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Subcultural (familial) mental retardation (sociocultural and polygenic etiology)

Pathologic mental retardation (severe structural defects)

Intelligence quotient Figure 28-4 Gaussian or bell-shaped curve of intelligence and its skewing by the group of mentally retardated individuals with diseases of the brain The shaded areas indicate the two groups of mentally retarded The hump representing the pathologically retarded is purely diagrammatic, illustrating its overlap with the subculturally retarded, discussed in the text and in Chap 38 When the population plotted is limited to mental defectives, a truly bimodal distribution is seen, segregating the two groups of retarded

types of hereditary mental retardation, but they have not been fully differentiated clinically or pathologically There being no visible neuropathology, the cases fall into the category of mental retardation without morphologic changes, discussed below As an aid to the neurologist, pediatrician, and child neurologist who must assume responsibility for the diagnosis and management of these backward children, the following descriptions may be of value The more severe forms of mental retardation and the developmental abnormalities and diseases that cause them are discussed in Chap 38, Developmental Diseases of the Nervous System Here only the milder subcultural forms ie, mental retardation without morphologic change are considered