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与丹迪-沃克综合征相关的神经皮肤黑素沉着症。

Neurocutaneous melanosis associated with Dandy-Walker syndrome.

作者信息

Narayanan H S, Gandhi D H, Girimaji S R

机构信息

Department of Psychiatry, National Institute of Mental Health and Neurosciences, Bangalore, India.

出版信息

Clin Neurol Neurosurg. 1987;89(3):197-200. doi: 10.1016/s0303-8467(87)80055-0.

Abstract

A young boy presented with mental retardation and seizures with extensive hairy naevi. After a fluctuating clinical course over the next 2 years, he worsened rapidly with signs of rising intracranial pressure. A CT scan revealed unsuspected features of Dandy-Walker syndrome. The clinical course is traced till the patient's death, and the implications of the coexistence of these rare conditions are discussed in view of the proposed hypotheses regarding the pathogenesis of Dandy-Walker syndrome.

摘要

一名患有智力迟钝、癫痫并伴有广泛先天性色素痣的小男孩前来就诊。在接下来的两年里,其临床病程波动不定,随后颅内压升高迹象明显,病情迅速恶化。CT扫描显示出意料之外的丹迪-沃克综合征特征。本文追溯了该患者直至死亡的临床病程,并鉴于有关丹迪-沃克综合征发病机制的假设,讨论了这些罕见病症共存的影响。

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