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孤立性神经结节病——一个诊断难题:病例报告与讨论

Isolated neurosarcoidosis--a diagnostic enigma: case report and discussion.

作者信息

Randeva H S, Davison R, Chamoun V, Bouloux P M G

机构信息

Centre for Neuroendocrinology, Royal Free Hospital Medical School, Hampstead, London, United Kingdom.

出版信息

Endocrine. 2002 Apr;17(3):241-7. doi: 10.1385/ENDO:17:3:241.

Abstract

Neurosarcoidosis is a rare, but well-recognized cause of hypopituitarism with a predilection for the hypothalamus. We describe a case of panhypopituitarism in a 57-yr-old Asian lady, associated with an infiltrating hypothalamo-hypophyseal lesion, and other intracranial deposits, initially diagnosed as cerebral tuberculomata. Despite antituberculous therapy, the intracranial lesions progressed with significant clinical deterioration. Repeated lumbar puncture, magnetic resonance imaging scans, liver biopsy and Gallium scan were noncontributory, and the diagnosis of isolated neurosarcoidosis was established only following biopsy of an intracranial lesion. The lesion regressed on steroid and azathioprine therapy. Isolated neurosarcoidosis poses a considerable management problem. We review recent advances in the investigation, diagnosis, and treatment of this condition.

摘要

神经结节病是一种罕见但已被充分认识的导致垂体功能减退的病因,且易累及下丘脑。我们描述了一例57岁亚洲女性全垂体功能减退的病例,该患者伴有浸润性下丘脑 - 垂体病变及其他颅内病灶,最初被诊断为脑结核瘤。尽管进行了抗结核治疗,但颅内病变仍进展,临床症状显著恶化。多次腰椎穿刺、磁共振成像扫描、肝脏活检及镓扫描均无诊断价值,仅在对颅内病变进行活检后才确诊为孤立性神经结节病。该病变在使用类固醇和硫唑嘌呤治疗后消退。孤立性神经结节病带来了相当大的治疗难题。我们综述了该疾病在检查、诊断和治疗方面的最新进展。

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