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[儿童神经结节病的假瘤形式。诊断困难与治疗管理]

[Pseudotumor forms of neurosarcoidosis in children. Diagnostic difficulties and therapeutic management].

作者信息

Chaix Y, Grouteau E, Sevely A, Boetto S, Delisle M B, Carrière J P

机构信息

Service de Médecine Infantile A, Hôpital de Purpan, Toulouse.

出版信息

Rev Neurol (Paris). 1997 Dec;153(12):771-4.

PMID:9686267
Abstract

In systemic sarcoidosis, neurologic manifestations are possible and occurred in 5 p. 100 of cases. The diagnosis is easier when sarcoiddosis is known, but in half case neurologic symptoms are inaugural. In these cases, involvement of other sites (liver, lung, eyes or skin) by the disease is needed to confirm sarcoidosis. Main manifestations in neurosarcoidosis are cranial nerve palsy, diabetes insipidus or chronic aseptic meningitis, but sometimes sarcoidosis can present as an intracranial mass. The diagnosis of neurosarcoidosis may be difficult and could be confused with infection (such tuberculosis or mycosis), with inflammatory diseases (such multiple sclerosis) or with neoplasm, particularly in case of oedema. Brain biopsy may be necessary in isolated neurosarcoidosis if no peripheral histology evidence of noncaseating granulomas has been obtained. Treatment with steroids alone is the best choice for initial therapy but must be prolonged. It is difficult to predict accurately the response to steroids and relapse may be possible after a long time. Other immunosuppressive therapeutics or radiotherapy have been used in patient with refractory neurosarcoidosis. Sequential magnetic resonance imaging is the useful mean for the follow-up of neurosarcoidosis.

摘要

在系统性结节病中,可能出现神经表现,发生率为100例中有5例。当已知患有结节病时诊断较容易,但半数情况下神经症状为首发症状。在这些病例中,需要疾病累及其他部位(肝脏、肺、眼睛或皮肤)以确诊结节病。神经结节病的主要表现为脑神经麻痹、尿崩症或慢性无菌性脑膜炎,但有时结节病可表现为颅内肿块。神经结节病的诊断可能困难,可能与感染(如结核病或真菌病)、炎症性疾病(如多发性硬化症)或肿瘤混淆,尤其是在有水肿的情况下。如果未获得非干酪样肉芽肿的外周组织学证据,对于孤立性神经结节病可能需要进行脑活检。初始治疗单独使用类固醇是最佳选择,但必须延长疗程。准确预测对类固醇的反应很困难,且长时间后可能复发。其他免疫抑制疗法或放疗已用于难治性神经结节病患者。序列磁共振成像对神经结节病的随访是有用的手段。

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