Severe myoclonic epilepsy in infancy - A clinical and electroencephalographic study.
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Detailed clinical and electroencephalographic long-term follow-up studies were made on 14 cases (3 boys and 11 girls) of severe myoclonic epilepsy in infancy (SME).<BR>1) Familial predisposition to convulsions was observed in as many as 9 cases (64.3%).<BR>2) Onset of convulsive seizures or characteristic partial seizures ranged from two to seven months of age, and additionally atypical absences and myoclonic seizures appeared at ages between seven months to 4 years 5 months<BR>3) Alternating hemiconvulsions and/or generalized convulsions were observed in 14 cases (100.0%), characteristic partial seizures with ocular deviation and cyanosis in 11 cases (78.6%), atypical absences in 13 cases (92.2%) and myoclonic seizures in 10 cases (71.4%). Convulsive seizures were characterized by being easily precipitated by fever and hot baths, and often occuring in a cluster or status epilepticus.<BR>These seizures were very intractable; at the time of follow-up convulsive seizures were not suppressed in any case and atypical absences and myoclonic seizures were suppressed at between 4 years to 17 years in only 5 cases.<BR>4) Despite almost normal development prior to the onset of seizures, all the cases showed overt slowing in mental development after one year of age.<BR>5) EEG back ground activity deteriorated with age, although mild in infancy.<BR>6) Epileptic discharges were hardly detectable in infancy, while after age one all the cases showed epiletipc discharges characterized by the combination of diffuse(100.0%) and focal (92.9%) epileptic discharges.<BR>7) Photosensitivity and pattern-sensitivity were observed in as many as 7 cases (50.0%) and 5 cases(37.7%), respectively, and clinical seizures were activated by light or geometric patterns in 5 cases (71.4%) of those with photosensitivity and all 5 cases with pattern-sensitivity.<BR>From these findings, SME was suggested to be a specific type of secondarygeneralized epilepsy with characteristic clinical course and remarkably poor prognosis.
- 一般社団法人 日本てんかん学会の論文
一般社団法人 日本てんかん学会 | 論文
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