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无系统性结节病的神经结节病

Neurosarcoidosis without systemic sarcoidosis.

作者信息

Sommer N, Weller M, Petersen D, Wiethölter H, Dichgans J

机构信息

Department of Neurology, University of Tübingen, Federal Republic of Germany.

出版信息

Eur Arch Psychiatry Clin Neurosci. 1991;240(6):334-8. doi: 10.1007/BF02279763.

Abstract

Neurosarcoidosis is a well-recognised complication of systemic sarcoidosis but diagnosis may be difficult if there is no clear evidence of an extracerebral manifestation of the disease. We present the case of a 42-year-old woman with clinical features characteristic of cerebral sarcoidosis including tetraparesis, diabetes insipidus, diencephalic hyperphagia, personality changes, and memory loss. Diagnosis was supported by cerebrospinal fluid (CSF) findings and magnetic resonance imaging (MRI): CSF showed mild lymphocytic pleocytosis, intrathecal production of IgG without oligoclonal bands, and a raised level of lysozyme. MRI revealed multiple contrast-enhanced granulomas at the base of the brain with partial involvement of diencephalic and mesencephalic structures and parts of the spinal cord. There was no evidence of systemic manifestation of sarcoidosis. Administration of corticosteroids led to improvement of the symptoms.

摘要

神经结节病是系统性结节病一种公认的并发症,但如果没有明确的脑外疾病表现证据,诊断可能会很困难。我们报告一例42岁女性病例,其具有脑结节病的临床特征,包括四肢轻瘫、尿崩症、间脑性贪食、人格改变和记忆力减退。脑脊液(CSF)检查结果和磁共振成像(MRI)支持诊断:脑脊液显示轻度淋巴细胞增多、鞘内产生IgG但无寡克隆带,以及溶菌酶水平升高。MRI显示脑底部有多个强化肉芽肿,累及间脑和中脑结构以及部分脊髓。没有结节病全身表现的证据。给予皮质类固醇使症状得到改善。

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