SEIZURE AND EEG PATTERNS IN ANGELMANS SYNDROME

被引:88
作者
VIANI, F
ROMEO, A
VIRI, M
MASTRANGELO, M
LALATTA, F
SELICORNI, A
GOBBI, G
LANZI, G
BETTIO, D
BRISCIOLI, V
DISEGNI, M
PARINI, R
TERZOLI, G
机构
[1] OSPED REGINA ELENA,CYTOGENET LAB,I-20122 MILAN,ITALY
[2] IST CLIN PERFEZIONAMENTO,CYTOGENET LAB,MILAN,ITALY
[3] UNIV MILAN,DEPT PEDIAT,I-20122 MILAN,ITALY
[4] CTR AUXOL ITALIANO PIANCAVALLO,CYTOGENT LAB,MILAN,ITALY
[5] CHILDRENS NEUROPSYCHIAT UNIT,REGGIO EMILIA,ITALY
[6] UNIV PAVIA,CHILDRENS NEUROPSYCHIAT CLIN,I-27100 PAVIA,ITALY
[7] IST CLIN PERFEZIONAMENTO,SERV NEUROPHYSIOL,MILAN,ITALY
[8] OSPED REGINA ELENA,CTR EPILEPSY,CHILDRENS NEUROPSYCHIAT UNIT,I-20122 MILAN,ITALY
关键词
D O I
10.1177/088307389501000609
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
We studied the seizure and polygraphic patterns of 18 patients with Angelman's syndrome. All patients showed movement problems. Eleven patients were also reported to have long-lasting periods of jerky movements. The polygraphic recording showed a myoclonic status epilepticus in nine of them. Seven patients had partial seizures with eye deviation and vomiting, similar to those of childhood occipital epilepsies. These seizures and electroencephalographic patterns suggest that Angelman's syndrome occurs in most of the patients as a nonprogressive, age-dependent myoclonic encephalopathy with a prominent occipital involvement. These findings indicate that, whereas ataxia is a constant symptom in Angelman's syndrome, the occurrence of a transient myoclonic status epilepticus may account for the recurrence of different abnormal movements, namely the jerky ones.
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收藏
页码:467 / 471
页数:5
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