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[神经结节病的临床表现及治疗方法]

[Clinical manifestations and therapeutic approach in neurosarcoidosis].

作者信息

Ferriby D, de Seze J, Stojkovic T, Hachulla E, Wallaert B, Blond S, Destée A, Hatron P Y, Decoulx M, Vermersch P

机构信息

Clinique neurologique, Hôpital Roger Salengro, CHRU Lille.

出版信息

Rev Neurol (Paris). 2000 Nov;156(11):965-75.

Abstract

Neurological impairment is a frequent cause of morbidity and mortality in patients with sarcoidosis. The aim of this study was to evaluate the clinical manifestations of the disease, the response to corticosteroids and alternative treatments. During a 5 year period, diagnosis of neurosarcoidosis was performed in 40 patients. We retrospectively analyzed clinical, laboratory data and response to treatments. Mean age was 41.3 years (range 17-72). Mean time of follow-up was 46 months. Neurologic signs were the first symptom in 50 p. 100 of cases and an isolated manifestation in 12.5 p. 100. Central nervous system impairment was seen in 60.7 p. 100, meningitis in 27 p. 100. Other clinical manifestations were cranial nerve palsies (27 p. 100), peripheral neuropathy (33 p. 100), myopathy (16 p. 100). Eighty percent of the patients were treated by corticosteroids. Because of a lack of efficacy 40 p. 100 of patient required alternative treatment (including methotrexate, cyclophosphamide, azathioprin, cyclosporin). Complete recovery was observed in only 27.5 p. 100 of cases confirming the severity of neurosarcoidosis. Forty percent of patients were clinically stable and 10 p. 100 worsened. No patient died. This study confirms that intensive initial treatment is often necessary to prevent irreversible lesions. Alternative treatment should be rapidly initiated in resistant forms.

摘要

神经功能障碍是结节病患者发病和死亡的常见原因。本研究的目的是评估该疾病的临床表现、对皮质类固醇及其他治疗方法的反应。在5年期间,40例患者被诊断为神经结节病。我们回顾性分析了临床、实验室数据及治疗反应。平均年龄为41.3岁(范围17 - 72岁)。平均随访时间为46个月。神经体征是50%病例的首发症状,12.5%为孤立表现。60.7%可见中枢神经系统损害,27%有脑膜炎。其他临床表现包括颅神经麻痹(27%)、周围神经病变(33%)、肌病(16%)。80%的患者接受了皮质类固醇治疗。由于疗效不佳,40%的患者需要其他治疗(包括甲氨蝶呤、环磷酰胺、硫唑嘌呤、环孢素)。仅27.5%的病例观察到完全康复,这证实了神经结节病的严重性。40%的患者临床稳定,10%病情恶化。无患者死亡。本研究证实,通常需要强化初始治疗以预防不可逆病变。对于耐药形式应迅速启动其他治疗。

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