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robehavioral and psychologic methods have not disclosed the basic fault in nervous functioning Is there one domain of faulty psychologic function such as failure of learning, inattentiveness, or faulty perception that underlies all forms of mental retardation Or are there several domains, differing from one case to another or one disease to another Only by the most innovative and sophisticated neuropsychologic studies will answers to such questions be obtained The EEG, in addition to exposing asymptomatic seizure activity, shows a high incidence of other abnormalities in the mentally retarded Presumably this is due to a greater degree of immaturity of the cerebrum at any given age However, a normal EEG is not infrequent and of relatively little help Moreover, CT scanning and MRI have been singularly unhelpful in revealing abnormalities in this group of children In the diagnosis of milder grades of retardation, always to be considered are the possible effects of severe malnutrition, neglect and deprivation, chronic systemic disease, iodine de ciency, impaired hearing and vision, and possibly childhood psychosis Of particular importance is the differentiation of a group of patients who are normal for a variable period after birth and then manifest a progressive decline from disease of the nervous system This type of disorder is representative of the group of hereditary metabolic and degenerative diseases discussed in Chap 37 Seizure disorders (and anticonvulsant medications) can impair cerebral function, and several special childhood seizure disorders are associated with a progressive decline in mental function in this group of patients (Chap 16) Management Since there is little or no possibility of treating the condition(s) underlying mental retardation and there is no way of
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restoring function to a nervous system that is developmentally subnormal, the medical objective is to assist in planning for the patient s training, education, and social and occupational adjustments As Voltaire remarked long ago, guidance is needed more than education The parents must be guided in forming realistic attitudes and expectations Psychiatric and social counseling may help the family to maintain gentle but rm support of the patient so that he can acquire, to the fullest extent possible, self-help skills, self-control, good work habits, and a congenial personality Most individuals with an IQ above 60 and no other handicaps can be trained to live an independent life Special schooling may enable such patients to realize their full potential Social factors that contribute to underachievement must be sought out and eliminated if possible Later, there is need for advice about possible occupational attainments Great care must be exercised in deciding about institutionalization Whereas severe degrees of retardation are all too apparent by the rst or second year, less severe degrees are dif cult to recognize early As stated above, psychologic tests alone are not trustworthy The method of assessment suggested many years ago by Fernald still has a ring of soundness It includes (1) physical examination, (2) family background, (3) developmental history, (4) school progress (grade achieved), (5) performance in schoolwork (tests of reading, arithmetic, etc), (6) practical knowledge, (7) social behavior, (8) industrial ef ciency, (9) moral reactions, and (10) intelligence as measured by psychologic tests All these data except (5) and (10) can be obtained by a skillful physician during the initial medical and neurologic examination and are used to guide the family in its dif cult decisions
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ALVAREZ-BUYLLA A, GARCIA-VERDUGO JM: Neurogenesis in adult subventricular zone J Neurosci 22:629, 2002 ANDERSON LD: The predictive value of infancy tests in relation to intelligence at ve years Child Dev 10:203, 1939 ANDRE-THOMAS, CHESNI Y, DARGASSIES SAINT-ANNES S: The Neurological Examination of the Infant London, Medical Advisory Committee, National Spastics Society, 1960 ANDREWS G, HARRIS M: Clinics in Developmental Medicine: No 17 The Syndrome of Stuttering London, Heinemann, 1964 ASPERGER H: Die autistischen Psychopathie in Kindesalter Arch Psychiatr Nervenkr 117:76, 1944 AYOTTE J, PERETZ I, HYDE K: Congenital amusia A group of adults af icted with a music-speci c disorder Brain 1125:238, 2002 BAKER L, CANTWELL DP, MATTISON RE: Behavior problems in children with pure speech disorders and in children with combined speech and language disorders J Abnorm Child Psychol 8:245, 1980 BARLOW C: Mental Retardation and Related Disorders Philadelphia, Davis, 1977 BAYLEY H: Comparisons of mental and motor test scores for age 1 15 months by sex, birth order, race, geographic location and education of parents Child Dev 36:379, 1965 BENDER L: A Visual-Motor Gestalt Test and Its Use New York, American Orthopsychiatric Association, 1938 BENTON AL: Right-left discrimination Pediatr Clin North Am 15:747, 1968 BENTON AL: Revised Visual Retention Test New York, Psychological Corporation, 1974 BIEDERMAN J, MUNIR K, KNEE D, et al: A family study of patients with attention de cit disorder and normal controls J Psychiatr Res 20:263, 1986 BIRCH HG, BELMONT L: Auditory-visual integration in normal and retarded readers Am J Orthopsychiatry 34:852, 1964 BYNE W: The biological evidence challenged, in The Scienti c American Book of the Brain, New York, Lyons Press, 1999, pp 181 194 CANEVINI MP, CHIFARI R, PIAZZINI A: Improvement of a patient with stuttering on levetiracetam Neurology 59:1288, 2002 CAPUTE AJ, ACCARDO PJ: Developmental Disabilities in Infancy and Childhood Baltimore, Brookes, 1991 CASANOVA MF, BUXHOEVEDEN DP, COHEN M, et al: Minicolumnar pathology in dyslexia Ann Neurol 52:108, 2002 CHESS S: Diagnosis and treatment of the hyperactive child NY State J Med 60:2379, 1960 CHESS S, HASSIBI M: Behavioral deviations in mentally retarded children J Am Acad Child Psychiatry 9:282, 1970 CHUGANI HT: Functional brain imaging in pediatrics Pediatr Clin North Am 39:777, 1992 CONEL J: The Postnatal Development of the Human Cerebral Cortex Vols 1 8 Cambridge, MA, Harvard University Press, 1939 1967 COWAN WM: The development of the brain Sci Am 241:112, 1979 CRITCHLEY M, CRITCHLEY EA: Dyslexia De ned Spring eld, IL, Charles C Thomas, 1978 DAMON W: The moral development of children Sci Am, August 1999, pp 72 79 DARGASSIES SAINT-ANNE S: Neurological Development in the Full-Term and Premature Neonate New York, Excerpta Medica, 1977
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