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大脑半球萎缩:两例临床病理报告及对发病机制和鉴别诊断的探讨

Cerebral hemiatrophy: a clinicopathological report of two cases with a contribution to pathogenesis and differential diagnosis.

作者信息

Vosskämper M, Schachenmayr W

机构信息

Institute of Neuropathology, University of Giessen, FRG.

出版信息

Clin Neuropathol. 1990 Sep-Oct;9(5):244-50.

PMID:2125535
Abstract

"Cerebral hemiatrophy" describes a condition of different etiologies that is characterized by a marked asymmetry of cerebral hemispheres. Case reports of two different forms of cerebral hemiatrophy are presented. In the first case perinatal asphyxia led to severe white matter lesions with predominance on the left side and marked asymmetry of the pyramidal tracts. Symptoms were present immediately after birth ("primary cerebral hemiatrophy"). The second case displayed postictal cerebral hemiatrophy with a widespread loss of cortical neurons of the entire left hemisphere. The disease process started at the age of two years after a widely normal early development ("secondary cerebral hemiatrophy"). A modified classification of cerebral hemiatrophy is presented, and concepts of pathogenesis and differential diagnosis are discussed.

摘要

“大脑半球萎缩”描述了一种病因各异的病症,其特征为大脑半球明显不对称。本文呈现了两种不同形式大脑半球萎缩的病例报告。在第一个病例中,围产期窒息导致严重的白质病变,主要发生在左侧,锥体束明显不对称。出生后立即出现症状(“原发性大脑半球萎缩”)。第二个病例表现为发作后大脑半球萎缩,整个左半球皮质神经元广泛缺失。疾病过程始于两岁,此前早期发育大致正常(“继发性大脑半球萎缩”)。本文提出了大脑半球萎缩的改良分类,并讨论了发病机制和鉴别诊断的概念。

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