3, 10, and 16 years, with early-onset dystonia predominantly distal in the upper limbs with minimal involvement of the legs and moderate to severe mental deficiency. The pattern of in- heritance is strongly suggestive of being X-linked recessive. The mother and one sister are healthy and two maternal uncles who died between 30 and 40 years of age were also mentally retarded and had dystonic features. The dystonic movements became fixed dystonic postures of the hands in the eldest who also has autistic behavior. Cranial MRI, brainstem auditory evoked re- sponses, and metabolic studies (i.e., amino acids, organic acids, oligosaccharides, and lysosomal enzymes) were normal. A mod- erate improvement was noted after treatment with trihexypheni- dyl was started.

126. HEMIMEGALENCEPHALY: THERAPEUTIC PERSPECTIVES Zen6n M. Sfaello, Alicia J. Mauro, Rafil C6rdoba, Andrey Kesman, and Julio C. Sufirez, C6rdoba, Argentina

A patient, age 5 months, born with macrocephaly, developed seizures at 3 months of age. He showed absence of visual fixa- tion or following and marked head lag with mild distal hyper- tonicity without asymmetry. Hemimegalencephaly by CT and MRI was diagnosed. At present, there is partial control of at- tacks with ACTH and valproic acid administration. Last reports suggest that very early hemispherectomy may be the treatment of choice for this condition. It attempts to preserve function of the "normal" hemisphere. A recent communication suggests that there is a growing disturbance factor that controls cell prolifera- tion in the abnormal hemisphere and that also may imply the benefit of early surgical excision. We discuss therapeutic pos- sibilities in our patient.

127. COGNITIVE SENSORIMOTOR AND NEURO- MOTOR DEVELOPMENT IN HEARING-IMPAIRED CHILDREN

Vanda M.G. Gonqalves and Ramiro R.G. Carazas, S~o Paulo, Brazil

We evaluated 2 congenitally and bilaterally deaf children from the Ambulatory of Hearing Loss Neurodiagnose. Chronologic age was 26 and 29 months. Clinical data, physical and neuro- logic examinations [Lef'evre, 1971], developmental diagnosis [Gesell and Amatruda, 1990], and cognitive development [Piaget, 1975] were done. Neurologic examination showed mildly hypotonic arm muscles and profound hearing loss. Ac- quisition of motor, adaptive, and personal-social milestones, were adequate for the chronologic age, except for language ex- pression (QD: 25 and 65%) and comprehension (QD: 55 and 30%). Despite their linguistic deficit, they performed at the cog- nitive level expected on the basis of their chronologic ages. Tertiary circular reactions and conceptual functions were respec- tively observed.

128. NARCOLEPSY IN ADOLESCENCE: STABILITY OF POLYSOMNOGRAPHY AND MULTIPLE SLEEP LATENCY TEST IN 4-YEAR FOLLOW-UP Rubens Reimao, Sao Panlo, Brazil

Polysomnography (PSG) and the multiple sleep latency test (MSLT) are routinely used for the diagnosis of narcolepsy, mainly in adults. However, scarce data are available concerning the long-term variability of these monitorings in adolescent nar- coleptics. Case 1, a 13-year-old male, with intense daytime sleepiness that started a year before, accompanied by cataplcxy, hypnagogic hallucination, and sleep paralysis, has been fol- lowed-up in our Sleep Disorders Center for 4 years. PSG and MSLT were performed (after drug washout) every 6-8, months since then. PSG findings are strikingly constant with sudden onset of REM periods at the beginning of the night. MSLT data are also stable with mean sleep latencies of 0.8- t.7 rain. Stage REM has always been present in 3-5 of the 5 MSLT subtests. This long-term follow-up suggests the stability of PSG and MSLT in narcoleptic adolescents.

129. SLEEP EVALUATION OF SOUTH AMERICAN CROTALUS SNAKE BITES: POLYSOMNOGRAPHIC AND MULTIPLE SLEEP LATENCY TEST

Rubens Reimao and Ros~ilia P. Leite, S~o Paulo, Brazil

South American Crotalus rattle snake venom has complex ac- tions on the CNS. Sleep impairment is seldom suspected on clinical grounds but quantitative studies are still lacking. We evaluated 3 patients: Case 1, 8-year-old male; Case 2, 15-year- old female; and Case 3, 24-year-old male. They were admitted to the hospital 2-5 hours after the Crotalus bite. Neurologic signs were bilateral ptosis, changes in pupilar diameter, external ophthalmoparesis, blurred vision, ataxia, and generalized loss of deep tendon reflexes. Cases 2 and 3 reported sleepiness in the first 3 days but not Case 1. Standard-specific serum was employed but not sedatives. They were submitted to albnight polysomnography (PSG). Cases 2 and 3 had an increase in REM sleep and REM density and mild REM latency reduction. Case 1 did not have any sleep abnormality in the first PSG and mild increase in REM sleep in the second. In Cases 2 and 3, multiple sleep latency test (MSLT) revealed moderate sleep latency re- duction, without REM periods. In Case 1 MSLT data were with- in normal limits, without REM periods. In the 8-year-old child, PSG and MSLT evidenced less impairment as compared to the adolescent and adult.

130. NEUROPSYCHOLOGIC ASSESSMENT OF FRA- GILE X SYNDROME Marilisa M. Guerreiro, Sylvia M. Ciasca, M. Valeriana, and L.M. Ribeiro, S~o Paulo, Brazil

The fragile X syndrome is the most common inherited cause of mental retardation with a overall incidence approaching i per 1,000. Most affected individuals are males. Typical clinical findings are macroorchidism, long and narrow face, and large or prominent ears. Common behavior features include poor eye contact, hand flapping, hand biting, and perseverative speech. Epilepsy can occur in approximately 25% of patients. We eval- uated 8 fragile X syndrome patients (6 males, 2 females) with moderate to profound mental retardation (MR): 1 female with moderate MR; 1 female and 2 males with severe MR, and 4 males with profound MR. The mean age was 14.6 years (range: 9-20 years). We performed the WISC, Bender, and an adapta- tion of the mental state assessment (MSA) by Weintraub and

376 PEDIATRIC NEUROLOGY Vol. 8 No. 5