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Head growth rate (HGR) in children with neuro-AIDS before and after AZT treatment
35. MATERNALLY T R A N S M I T T E D HIV1 INFECTION: COGNITIVE ASSESSMENT OF SCHOOL AGE CHILDREN M. Tardieu, M.J. Mayaux, N. Seibel, I. Funck-Brentano, E. Straub, J.P. Teglas, and S. Blanche, Paris, France All HIV1 vertically infected children born before 1985 followed in a single center (n = 57) were studied until 1993 and 33 children, having reached the age of 6 years, were tested for school achievements as well as for cognitive abilities, fine motor and language skills, and for 24 of them emotional adaptation. Twenty-two of 33 patients (67%) had normal school results at a mean age of 114 --- 18 months. The mean IQ was 95 --- 11 but 54% of the patients (11 of 24) had pathologic results in visuospatial and time-orientation tests. Twenty-six percent (6 of 23) had abnormal results for vocabulary or phonology. Twenty-nine percent of the children and 42% of the parents had psychoaffective symptoms of intermediate or high intensity. Normal school performances were positively correlated with results at the different cognitive tests and to a lesser extent with the absence of psychoaffective symptoms but was independent of the cause of maternal infection or educational level of the parents. Children with normal test results or normal school achievement had a higher percentage of circulating CD4 lymphocytes (P < .02 at time of tests). Percentages of CD4 lymphocytes obtained earlier during the course of infection were also significantly higher in patients who have normal school achievement (P < .03 at first referral; P = .05 at 52 mo). Children whose HIV1 infection was maternally acquired had better cognitive abilities and school achievements than initially thought and results of percentage of circulating CD4 lymphocytes obtained during the first years of life appeared predictive of future school adaptation or cognitive abilities. This study was supported by Agence Nationale de Recherche sur le SIDA.
36. HEAD GROWTH RATE (HGR) IN CHILDREN WITH NEURO-AIDS BEFORE AND AFTER AZT TREATMENT Liliana A. Czornyj, Julia J. Redondo, Guillermo Roccatagliata, Susana M. Rodriguez, Luis Carniglia, Jos6 L. Torolla, Cecilia Procak-Psaros, and Natalio Fejerman, Buenos Aires, Argentina Ninety-five children with neuro-AIDS were included in a protocol for AZT treatment between 1990 and 1993. Ages ranged from 3 months to 7.5 years. We divided them into 2 groups according to age at onset of treatment and age of confirmed seroconversion: group A: 56 children younger than 18 months (mean age: 10.4 months); and group B: 39 children older than 18 months (mean age: 3.9 years). Among other items, including neurologic state, neuropsychologic development, brain imaging studies, etc., thorough periodic measures of head circumference (HC) were taken in order to evaluate variation in HGR. Decrease in HGR even leading to acquired microcephaly (AM) was found in 55% of patients in group A before AZT treatment and in 20.5% of patients in group B. Following AZT treatment a significant increase in HGR was seen in 100% of the patients in group B and in 75% of those in group A. Thirteen patients of this group reverted HC from the microcephalic range into normal. In
94 PEDIATRICNEUROLOGY Vol. 11 No. 2
3 patients after interrupting the treatment, a rapid fall of HGR was seen. Only children with severe progressive AIDS encephalopathies did not show improvement in HGR after AZT. Decrease in HGR leading to AM is an early finding in children with neuro-AIDS. Present data suggest that AZT treatment may improve HGR in these patients.
37. RE-EVALUATION OF BENIGN (FAMILIAL) INFANTILE CONVULSIONS (FUKUYAMA) AS NEW EPILEPTIC SYNDROME M. Sakauchi, H. Oguni, S. Yanagaki, K. Hayashi, M. Osawa, and Y. Fukuyama, Tokyo, Japan Vigevano et al. [1992] and Lee et al. [1993] advocated benign familial infantile epilepsy as a new epileptic syndrome, characteristically occurring in infancy, which was not included in the 1990 International League Against Epilepsy classification of epilepsies. In Japan, however, a dozen reports on a very similar condition have appeared since Fukuyama first described 53 patients with "recurrent but benign generalized convulsions of unknown etiology in infants" (benign infantile convulsions; BIC) in 1963. This report deals with 108 BIC patients who first visited our hospital during the period between 1967 and 1982, and were followed up for more than 5 years. Entry criteria included the onset of short, generalized (occasionally partial) convulsions before age 2 years, not associated with any trigger, no antecedent history, and no evidence of neurologic abnormality. Mean ages at the first and last seizure were 8 and 13 months, respectively. Generalized tonic-clonic convulsions were most common (94%). The seizure recurrence frequency was 3 or less in more than 90% of patients and only 1 in 32%. Positive family history was found in 40% of patients, including febrile convulsions (23%), epilepsy (8%), BIC (4.6%), and undetermined seizures (4.6%). EEG abnormalities were noted only in 17%, even with multiple followup EEGs.
38. BENIGN INFANTILE FAMILIAL CONVULSIONS: CLINICAL AND GENETIC ASPECTS Federico Vigevano, Lucia Fusco, Matteo Di Capua, Stefano Ricci, Rosella Sebastianelli, Pierpaolo Lucchini, Tiziana Granata, Benedetto Dordi, Antonio Chindemi, Olivier Dulac, Corinne Beck, and Alain Malafosse, Rome, Italy and Montepellier, France Benign infantile familial convulsions (BIFC) are characterized by partial seizures, onset at 4-7 months, absence of etiologic factors, normal psychomotor development, benign course, normal interictal EEGs, family history of seizures with similar age of onset, and course (Vigevano et al.: Eur J Pediatr 1992;151: 608-12). Our multicenter study collected data on 18 families ( 16 Italian, 2 French). Thirty-four first- and second-degree relatives presented with BIFC. Seizures presented as clusters in 17 patients. The most frequently reported ictal symptoms included psychomotor arrest, cyanosis, head-eye deviation, tonic contraction, and bilateral clonic jerks. Interictal EEGs were normal. Ictal EEGs (available in 9 patients) were characterized by fast activity in the occipito-temporal-parietal areas of one hemisphere with subsequent generalization. The follow-up data confirmed
36. HEAD GROWTH RATE (HGR) IN CHILDREN WITH NEURO-AIDS BEFORE AND AFTER AZT TREATMENT Liliana A. Czornyj, Julia J. Redondo, Guillermo Roccatagliata, Susana M. Rodriguez, Luis Carniglia, Jos6 L. Torolla, Cecilia Procak-Psaros, and Natalio Fejerman, Buenos Aires, Argentina Ninety-five children with neuro-AIDS were included in a protocol for AZT treatment between 1990 and 1993. Ages ranged from 3 months to 7.5 years. We divided them into 2 groups according to age at onset of treatment and age of confirmed seroconversion: group A: 56 children younger than 18 months (mean age: 10.4 months); and group B: 39 children older than 18 months (mean age: 3.9 years). Among other items, including neurologic state, neuropsychologic development, brain imaging studies, etc., thorough periodic measures of head circumference (HC) were taken in order to evaluate variation in HGR. Decrease in HGR even leading to acquired microcephaly (AM) was found in 55% of patients in group A before AZT treatment and in 20.5% of patients in group B. Following AZT treatment a significant increase in HGR was seen in 100% of the patients in group B and in 75% of those in group A. Thirteen patients of this group reverted HC from the microcephalic range into normal. In
94 PEDIATRICNEUROLOGY Vol. 11 No. 2
3 patients after interrupting the treatment, a rapid fall of HGR was seen. Only children with severe progressive AIDS encephalopathies did not show improvement in HGR after AZT. Decrease in HGR leading to AM is an early finding in children with neuro-AIDS. Present data suggest that AZT treatment may improve HGR in these patients.
37. RE-EVALUATION OF BENIGN (FAMILIAL) INFANTILE CONVULSIONS (FUKUYAMA) AS NEW EPILEPTIC SYNDROME M. Sakauchi, H. Oguni, S. Yanagaki, K. Hayashi, M. Osawa, and Y. Fukuyama, Tokyo, Japan Vigevano et al. [1992] and Lee et al. [1993] advocated benign familial infantile epilepsy as a new epileptic syndrome, characteristically occurring in infancy, which was not included in the 1990 International League Against Epilepsy classification of epilepsies. In Japan, however, a dozen reports on a very similar condition have appeared since Fukuyama first described 53 patients with "recurrent but benign generalized convulsions of unknown etiology in infants" (benign infantile convulsions; BIC) in 1963. This report deals with 108 BIC patients who first visited our hospital during the period between 1967 and 1982, and were followed up for more than 5 years. Entry criteria included the onset of short, generalized (occasionally partial) convulsions before age 2 years, not associated with any trigger, no antecedent history, and no evidence of neurologic abnormality. Mean ages at the first and last seizure were 8 and 13 months, respectively. Generalized tonic-clonic convulsions were most common (94%). The seizure recurrence frequency was 3 or less in more than 90% of patients and only 1 in 32%. Positive family history was found in 40% of patients, including febrile convulsions (23%), epilepsy (8%), BIC (4.6%), and undetermined seizures (4.6%). EEG abnormalities were noted only in 17%, even with multiple followup EEGs.
38. BENIGN INFANTILE FAMILIAL CONVULSIONS: CLINICAL AND GENETIC ASPECTS Federico Vigevano, Lucia Fusco, Matteo Di Capua, Stefano Ricci, Rosella Sebastianelli, Pierpaolo Lucchini, Tiziana Granata, Benedetto Dordi, Antonio Chindemi, Olivier Dulac, Corinne Beck, and Alain Malafosse, Rome, Italy and Montepellier, France Benign infantile familial convulsions (BIFC) are characterized by partial seizures, onset at 4-7 months, absence of etiologic factors, normal psychomotor development, benign course, normal interictal EEGs, family history of seizures with similar age of onset, and course (Vigevano et al.: Eur J Pediatr 1992;151: 608-12). Our multicenter study collected data on 18 families ( 16 Italian, 2 French). Thirty-four first- and second-degree relatives presented with BIFC. Seizures presented as clusters in 17 patients. The most frequently reported ictal symptoms included psychomotor arrest, cyanosis, head-eye deviation, tonic contraction, and bilateral clonic jerks. Interictal EEGs were normal. Ictal EEGs (available in 9 patients) were characterized by fast activity in the occipito-temporal-parietal areas of one hemisphere with subsequent generalization. The follow-up data confirmed