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Titolo:
SCHIZENCEPHALY AND UPPER-LIMB MALFORMATIO N
Autore:
HUMBERTCLAUDE V; PEDESPAN JM; AZAIS M; FONTAN D; ECHENNE B;
Indirizzi:
CLIN ST ELOI,SERV NEUROL PEDIAT F-34295 MONTPELLIER FRANCE HOP PELLEGRIN,SERV PEDIAT B F-33076 BORDEAUX FRANCE
Titolo Testata:
Archives de pediatrie
fascicolo: 4, volume: 3, anno: 1996,
pagine: 357 - 359
SICI:
0929-693X(1996)3:4<357:SAUMN>2.0.ZU;2-T
Fonte:
ISI
Lingua:
FRE
Keywords:
ABNORMALITIES; BRAIN; EXTREMITIES; ECTROMELIA;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
4
Recensione:
Indirizzi per estratti:
Citazione:
V. Humbertclaude et al., "SCHIZENCEPHALY AND UPPER-LIMB MALFORMATIO N", Archives de pediatrie, 3(4), 1996, pp. 357-359

Abstract

Background.- Schizencephaly, a failure of the cerebral mantle to formis usually unilateral and not associated with anomalies of the controlateral limbs. We present two cases associated with such anomalies. Case reports Case no 1.- A boy born with a lobster claw deformity in theright arm. His IQ was 60 and he developed generalized seizures at theage of 4 years with spastic diplegia. MRI examination showed unilateral left schizencephaly also lined with pachygyric cortex. Case no 2.- A girl was born with a right ectromelic-hand and developed left spastic hemiparesy. Her le was normal. She had a generalized seizure at the age of 11 years. MRI showed unilateral right schizencephaly also linedwith pachygyric cortex and heterotopic periventricular gray matter. Discussion.- Schizencephaly seems to be due to localized ischemia in the periventricular germinal matrix during the 7th week of gestation. The limb deformity could be explained by the same vascular mechanism in the first case but not in the second one since it was homolateral To brain damage. Conclusion.- Description of further cases is necessary tounderstand this association. We suggest MRI examination of brain in patients with limb deformity associated with neurological damage.

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Documento generato il 30/11/20 alle ore 10:02:41