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Short-term seizure prognosis in West syndrome
% improvement = 100 -
time to X initial seizure × 100 time to X final seizure We present various examples of how this fbrmula maintains applicability in different epileptic settings. We believe that this is an objective measure of outcome expressed in % improvement. A series of patients was followed with this method (also presented to this meeting); some results are presented to illustrate the method. Reference: [1] Shofer J, Temkin N. Comparison of alternative outcome measures for antiepileptic drug trials. Arch Neurol 1986;43:877-81. 197. T R E A T M E N T S T R A T E G I E S SYNDROMES Jorge Eslava-Cobos, Bogotfi, Colombia
FOR
EPILEPTIC
Two hundred and eighteen patients in a pediatric outpatient epilepsy clinic were enrolled over 18 months and followed for 1-2V2 years. In accordance with present tendencies, epilepsy was confronted from a syndromatic not a symptomatic perspecfive. Emphasis was placed on monotherapy with valproate for the treatment of generalized syndromes, carbamazepine or phenytoin for partial syndromes, and benzodiazepines as add-on drugs. Rationale for that general strategy is discussed. Results are presented for each epileptic syndrome in terms of anticonvulsant(s) used, dose usually effective, feasibility of monotherapy, expectancy of seizure control and aggressiveness in management. Overall, results were excellent in 73.4%, good in 8.3%, fair in 6.9%, and poor in l 1.4%.
tween the PCB and control groups. In SSEPs, ~lo significanl differences were found in wave latencies and amplitudes b e tween both groups. In P300, the mean latencics ¢~1 P30(1 were significantly longer in PCB children than in controls at Cz and Pz, P = .005 and P = .027, respectively. The amplitude ,,ff P300 was lower in the PCB group than in the ~ontrol group (P = .022). In conclusion, this study indicates that PCB is less involved in the sensory pathways of CNS in children, hut PCB affected the cognitive function of brain more,
200. I M M U N O C Y T O C H E M I C A L ABNORMALITIES IN A M O U S E M O D E L O F SPINAL M U S C U L A R ATROPHY Ratil N. Mandler, MD and Pam C. Allgood, Albuquerque, New Mexico
We present 25 patients with West syndrome, treated with ACTH and/or prednisone plus usual anticonvulsants. Short-term seizure control is presented. ACTH was better than prednisone and prednisone better than usual anticonvulsants. We discuss the short-term evolution of West syndrome into other syndromes, in particular partial symptomatic epilepsy, and implications for treatment. In terms of seizure control, we believe a less than the usual nihilistic attitude is in order, when aggressive treatment is employed.
The mechanisms that lead to motoneuron dysfunction in spinal muscular atrophies (SMA) are not completely known. Because of the paucity of patients affected with SMA and the impossibility of examining the spinal cord pathology at various disease stages, we studied cell phenotypes in spinal cords of the wobbler mouse, an autosomal-recessive congenic model for SMA. This study attempted to characterize abnormal cell subpopulations in cervical spinal cord areas that were affected the most. Wobbler mice and normal littermates were studied at 3-6 weeks of age. After euthanasia, spinal cords were removed and dissected into cervical, thoracic and lumbar areas, according to predetermined landmarks. Spinal cords were then processed: (1) for tissue immunocytochemistry by plastic resin embedding and semithin sectioning followed by staining using immunogold techniques; (2) for flow cytometric quantitative immunocytochemistry by the following established procedures [Mandler RN, et al. J Neurosci Meth 1988;22:203-13; Mandler RN, et al. Brain Res 1990;522:46-54]. In symptomatic animals, inarked increases in glial acidic fibrillary protein (GFAP) cells were found in the cervical spinal cord. Computerized video-enhancing and flow cytometric GFAP cell quantitation revealed a 1520-fold augmentation in the wobbler compared to the normal twin. On the contrary, strychnine-sensitive glycine receptor cells were diminished in the wobbler. This study demonstrates not only neuronal but also glial phenotypic abnormalities in mice with SMA. These results suggest that in SMA CNS cells other than motoneurons could also contribute to the pathogenesis.
199. EFFECTS OF PRENATAL EXPOSURE TO POLYCHLORIDE BIPHENYLS ON NEUROLOGIC FUNCTION IN CHILDREN Yung-Jung Chen and Chen-Chin Hsu, Tainan, Taiwan
201. E F F E C T O F A M E N T A L L Y R E T A ~ E D CHILD ON FAMILY FUNCTIONING IN A THIRD WORLD COMMUNITY Peter M. Leary and Fiona Verth, Cape Town, South Africa
One hundred and sixty-three children were examined with general and soft neurologic examination. They were divided into two groups, PCB group (n = 79) and control group (n = 84). Thirty-one of 79 in PCB group and 27 of 84 in control group had been examined with evoked potentials recording including P-VEPs, SSEPs and P300. On neurologic examination, 37 of 74 children in the PCB group and 43 of 84 controls do not have soft neurologic signs. Nineteen of 79 had difficulty in performing finger-thumb opposition, 17 had mild and 2 had moderate degrees of abnormality. In P-VEPs, there was a significant reduction in the amplitude of P2 in PCB group (P = .02), while there was no apparent difference in tatencies of N1 and P2 be-
This study was undertaken to examine the functioning of 22 families which, under conditions of extreme poverty, had to raise a child with severe mental handicap. The children were all younger than 18 years with an IQ or DQ tess than 50. The family income was less than US $170.00 per month. Information was gathered by a questionnaire devised to give information about the family and to explore parental attitudes and perceptions regarding the child. It was found that, despite a very restricted cash income, family units endured. Seventy-nine percent of marriages in the study were stable and a majority of siblings did not show disturbed behavior. Ninety-one percent of the mothers confessed to on-going grief and distress but only
198. SHORT-TERM SEIZURE PROGNOSIS IN WEST SYNDROME Jorge Eslava-Cobos, Leonardo Palacios, Alberto Velez, and Carlos Mora, Bogota, Colombia
394 PEDIATRIC NEUROLOGY Vol. 8 No. 5
time to X initial seizure × 100 time to X final seizure We present various examples of how this fbrmula maintains applicability in different epileptic settings. We believe that this is an objective measure of outcome expressed in % improvement. A series of patients was followed with this method (also presented to this meeting); some results are presented to illustrate the method. Reference: [1] Shofer J, Temkin N. Comparison of alternative outcome measures for antiepileptic drug trials. Arch Neurol 1986;43:877-81. 197. T R E A T M E N T S T R A T E G I E S SYNDROMES Jorge Eslava-Cobos, Bogotfi, Colombia
FOR
EPILEPTIC
Two hundred and eighteen patients in a pediatric outpatient epilepsy clinic were enrolled over 18 months and followed for 1-2V2 years. In accordance with present tendencies, epilepsy was confronted from a syndromatic not a symptomatic perspecfive. Emphasis was placed on monotherapy with valproate for the treatment of generalized syndromes, carbamazepine or phenytoin for partial syndromes, and benzodiazepines as add-on drugs. Rationale for that general strategy is discussed. Results are presented for each epileptic syndrome in terms of anticonvulsant(s) used, dose usually effective, feasibility of monotherapy, expectancy of seizure control and aggressiveness in management. Overall, results were excellent in 73.4%, good in 8.3%, fair in 6.9%, and poor in l 1.4%.
tween the PCB and control groups. In SSEPs, ~lo significanl differences were found in wave latencies and amplitudes b e tween both groups. In P300, the mean latencics ¢~1 P30(1 were significantly longer in PCB children than in controls at Cz and Pz, P = .005 and P = .027, respectively. The amplitude ,,ff P300 was lower in the PCB group than in the ~ontrol group (P = .022). In conclusion, this study indicates that PCB is less involved in the sensory pathways of CNS in children, hut PCB affected the cognitive function of brain more,
200. I M M U N O C Y T O C H E M I C A L ABNORMALITIES IN A M O U S E M O D E L O F SPINAL M U S C U L A R ATROPHY Ratil N. Mandler, MD and Pam C. Allgood, Albuquerque, New Mexico
We present 25 patients with West syndrome, treated with ACTH and/or prednisone plus usual anticonvulsants. Short-term seizure control is presented. ACTH was better than prednisone and prednisone better than usual anticonvulsants. We discuss the short-term evolution of West syndrome into other syndromes, in particular partial symptomatic epilepsy, and implications for treatment. In terms of seizure control, we believe a less than the usual nihilistic attitude is in order, when aggressive treatment is employed.
The mechanisms that lead to motoneuron dysfunction in spinal muscular atrophies (SMA) are not completely known. Because of the paucity of patients affected with SMA and the impossibility of examining the spinal cord pathology at various disease stages, we studied cell phenotypes in spinal cords of the wobbler mouse, an autosomal-recessive congenic model for SMA. This study attempted to characterize abnormal cell subpopulations in cervical spinal cord areas that were affected the most. Wobbler mice and normal littermates were studied at 3-6 weeks of age. After euthanasia, spinal cords were removed and dissected into cervical, thoracic and lumbar areas, according to predetermined landmarks. Spinal cords were then processed: (1) for tissue immunocytochemistry by plastic resin embedding and semithin sectioning followed by staining using immunogold techniques; (2) for flow cytometric quantitative immunocytochemistry by the following established procedures [Mandler RN, et al. J Neurosci Meth 1988;22:203-13; Mandler RN, et al. Brain Res 1990;522:46-54]. In symptomatic animals, inarked increases in glial acidic fibrillary protein (GFAP) cells were found in the cervical spinal cord. Computerized video-enhancing and flow cytometric GFAP cell quantitation revealed a 1520-fold augmentation in the wobbler compared to the normal twin. On the contrary, strychnine-sensitive glycine receptor cells were diminished in the wobbler. This study demonstrates not only neuronal but also glial phenotypic abnormalities in mice with SMA. These results suggest that in SMA CNS cells other than motoneurons could also contribute to the pathogenesis.
199. EFFECTS OF PRENATAL EXPOSURE TO POLYCHLORIDE BIPHENYLS ON NEUROLOGIC FUNCTION IN CHILDREN Yung-Jung Chen and Chen-Chin Hsu, Tainan, Taiwan
201. E F F E C T O F A M E N T A L L Y R E T A ~ E D CHILD ON FAMILY FUNCTIONING IN A THIRD WORLD COMMUNITY Peter M. Leary and Fiona Verth, Cape Town, South Africa
One hundred and sixty-three children were examined with general and soft neurologic examination. They were divided into two groups, PCB group (n = 79) and control group (n = 84). Thirty-one of 79 in PCB group and 27 of 84 in control group had been examined with evoked potentials recording including P-VEPs, SSEPs and P300. On neurologic examination, 37 of 74 children in the PCB group and 43 of 84 controls do not have soft neurologic signs. Nineteen of 79 had difficulty in performing finger-thumb opposition, 17 had mild and 2 had moderate degrees of abnormality. In P-VEPs, there was a significant reduction in the amplitude of P2 in PCB group (P = .02), while there was no apparent difference in tatencies of N1 and P2 be-
This study was undertaken to examine the functioning of 22 families which, under conditions of extreme poverty, had to raise a child with severe mental handicap. The children were all younger than 18 years with an IQ or DQ tess than 50. The family income was less than US $170.00 per month. Information was gathered by a questionnaire devised to give information about the family and to explore parental attitudes and perceptions regarding the child. It was found that, despite a very restricted cash income, family units endured. Seventy-nine percent of marriages in the study were stable and a majority of siblings did not show disturbed behavior. Ninety-one percent of the mothers confessed to on-going grief and distress but only
198. SHORT-TERM SEIZURE PROGNOSIS IN WEST SYNDROME Jorge Eslava-Cobos, Leonardo Palacios, Alberto Velez, and Carlos Mora, Bogota, Colombia
394 PEDIATRIC NEUROLOGY Vol. 8 No. 5