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Arquivos de Neuro-Psiquiatria 1996-Dec

[Familial cavernous angioma. Report in 3 generations].

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J L Fobe
J B de Lima
M L de Buone
J Correa Neto

Ključne riječi

Sažetak

Cavernous angioma is a vascular malformation that affect 0.5 to 0.7% of the population making up 8 to 15% of cerebrovascular malformations. It is the second vascular malformation in frequency of the central nervous system, supplanted only by classic arteriovenous malformation. It may occur in two forms: a sporadic form characterized by isolated lesions: and a familial form characterized by multiple lesions with an autosomal dominant mode of inheritance with high penetrance and varied expressivity in the proportion M1:F1. Symptoms related to cavernous angioma are seizures, headache or progressive neurologic deficit. The authors present a Chinese family with familial cavernous angioma. Manifestations of the disease occurred in three generations affecting only females. Clinical, neuroimage, pathological, natural course and genetical aspects of the disease are discussed.

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