Salazar A, Mañá J, Albareda J M, Badrinas F, Fernández-Nogués F
Servicio de Medicina Interna, Hospital de Bellvitge-Prínceps d'Espanya, Universidad de Barcelona.
Med Clin (Barc). 1993 Feb 27;100(8):292-5.
The involvement of the nervous system in sarcoidosis occurs with a frequency of approximately 5%. The aim of this study was to review the frequency and characteristics of the neurologic manifestations of the a series of sarcoidosis.
During a period of 18 years (1974-1991) 270 patients were diagnosed with sarcoidosis in the Hospital de Bellvitge-Prínceps d'Espanya. The diagnosis of neurosarcoidosis was performed in those presenting neurologic symptoms no attributable to other causes. The clinical histories were retrospectively reviewed collecting clinical, radiologic, analytic, therapeutic and evolutive data.
Fourteen patients (5.1%) were diagnosed of neurosarcoidosis. Neurologic symptomatology was the first manifestation of the disease in 12 (85%). All showed extra-neurologic manifestations of sarcoidosis with the lung being the most frequently affected organ (78%). The clinical picture consisted of cranial neuropathy in 11 cases of which nine presented peripheral facial paralysis and 4 cases involvement of the central nervous system. In 13 patients the treatment schedule included glucocorticoids. All the patients (100%) with alteration of the central nervous system continued with active disease at 2 years while this only occurred in 33% of the cases with paralysis of the cranial pairs.
The frequency of neurosarcoidosis in the authors' series was similar to that described in the literature and its most frequent clinical form was cranial neuropathy. The treatment of choice was glucocorticoid and the involvement of the cranial pairs presents a better prognosis than that of the central nervous system.
结节病累及神经系统的发生率约为5%。本研究旨在回顾一系列结节病患者神经表现的发生率及特征。
在18年期间(1974 - 1991年),西班牙王子德贝尔维特格医院确诊了270例结节病患者。对那些出现无法归因于其他原因的神经症状的患者进行神经结节病的诊断。回顾性分析临床病史,收集临床、放射学、分析、治疗及病情演变数据。
14例患者(5.1%)被诊断为神经结节病。12例(85%)患者的神经症状是疾病的首发表现。所有患者均有结节病的肺外表现,其中肺部是最常受累的器官(78%)。临床表现为11例颅神经病变,其中9例出现周围性面瘫,4例累及中枢神经系统。13例患者的治疗方案包括糖皮质激素。所有中枢神经系统受累的患者在2年时疾病仍处于活动期,而颅神经麻痹患者中只有33%出现这种情况。
作者系列研究中神经结节病的发生率与文献报道相似,其最常见的临床类型是颅神经病变。首选治疗方法是糖皮质激素,颅神经受累的预后比中枢神经系统受累要好。