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[结节病的神经系统受累。格勒诺布尔系统性疾病联合会]

[Neurologic involvement in sarcoidosis. Federation of Systemic Diseases of Grenoble].

作者信息

Pavese P, Brion J P, Chabre O, Fauconnier J, Pasquier B

机构信息

CHU de Grenoble.

出版信息

Presse Med. 1999 Jan 30;28(4):168-72.

Abstract

OBJECTIVE

Study the incidence, clinical features, prognosis and diagnostic and therapeutic strategies in neurological lesions of sarcoidosis.

PATIENTS AND METHODS

The 207 cases of sarcoidosis followed at the Grenoble University Hospital between 1992 and 1998 were identified. After collecting data, the cases with neurological signs related to sarcoidosis were selected.

RESULTS

Sixteen patients (7.7%) had neurosarcoidosis. Ten had central nervous system involvement, with 5 reaching the hypothalamus and pituitary glands, 2 cases of meningoencephalitis, 2 pseudotumoral lesions, and 1 bitemporal lesion. Six had peripheral nervous system involvement, 3 had facial palsies and 3 had neuropathies. Laboratory tests were not contributive to diagnosis. Ten magnetic resonance imaging series were pathological out of 11 performed. Three central nervous system biopsies were obtained. Corticosteroid therapy was the most frequent treatment. For hypothalamic and pituitary dysfunction, the only treatment was substitutive hormone therapy. The course was favorable in 11 cases, stable in 4 cases. Symptoms worsened despite treatment in only 1 case.

CONCLUSION

The incidence of neurosarcoidosis may be much higher than is generally realized. The association of suggestive MRI signs, and clinical and laboratory findings evoke the diagnosis. Brain biopsy remains necessary for the pseudotumoral forms and the primitive neurological forms.

摘要

目的

研究结节病神经病变的发病率、临床特征、预后以及诊断和治疗策略。

患者与方法

确定了1992年至1998年间在格勒诺布尔大学医院随访的207例结节病患者。收集数据后,选择出有与结节病相关神经体征的病例。

结果

16例患者(7.7%)患有神经结节病。10例累及中枢神经系统,其中5例累及下丘脑和垂体,2例为脑膜脑炎,2例为假瘤性病变,1例为双侧颞叶病变。6例累及周围神经系统,3例为面神经麻痹,3例为神经病变。实验室检查对诊断无帮助。11次磁共振成像检查中有10次结果呈病理性。进行了3次中枢神经系统活检。皮质类固醇治疗是最常用的治疗方法。对于下丘脑和垂体功能障碍,唯一的治疗方法是替代激素治疗。11例病情好转,4例病情稳定。仅1例患者尽管接受了治疗但症状仍恶化。

结论

神经结节病的发病率可能比普遍认识的要高得多。具有提示性的磁共振成像征象、临床和实验室检查结果相结合可做出诊断。对于假瘤性和原发性神经病变形式,脑活检仍然是必要的。

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