Radziwill A, Weder B
Klinik für Neurologie, Kantonsspital St. Gallen.
Nervenarzt. 1995 Dec;66(12):915-22.
We present retrospective and follow-up data of eight patients with sarcoidosis involving the central nervous system (CNS). The diagnosis was established definitely by histological evidence in seven cases. Biopsies were taken directly from the CNS or the covering meninges in two of them. Additionally, we included a further case with the characteristic triad of arthralgia, erythema nodosum and pulmonary hilar adenopathy on X-ray, representing Löfgren's syndrome. Systemic manifestation of sarcoidosis was hardly recognized in some cases and sometimes became evident only in the long term after recurring manifestations. The symptomatology in our cases can be summarized as follows: Mostly, meningopolyneuritic and meningoencephalopathic syndromes occurred, with, in addition, one case of chronic meningitis and two cases of spinal involvement. In two cases, CNS symptoms represented the initial manifestation of the disease. Furthermore, the stringent interdependency of non-neurologic and neurologic episodes of the disease was not recognized easily. Under these circumstances, the histological confirmation was often difficult. Thus, the latency between the first neurological signs of the disease and the diagnosis of neurosarcoidosis may be long, as in our cases (mean 27 months). The course of the disease was followed for a mean 8.1 (range 1-15) years. Seven patients showed at least partial recovery from progressive disease after corticosteroid therapy. One patient developed, subacutely, a fatal pontine-medullary syndrome. The potentially poor prognosis emphasizes the importance of diagnostic efforts.
我们展示了8例累及中枢神经系统(CNS)的结节病患者的回顾性和随访数据。7例通过组织学证据明确诊断。其中2例直接从CNS或覆盖的脑膜进行活检。此外,我们纳入了另一例具有关节痛、结节性红斑和X线显示肺门淋巴结病这一特征三联征的病例,代表 Löfgren 综合征。结节病的全身表现在某些病例中很难识别,有时仅在反复出现症状后的长期才变得明显。我们病例中的症状学可总结如下:主要出现脑膜多神经炎和脑膜脑病变综合征,此外,有1例慢性脑膜炎和2例脊髓受累。2例中,CNS症状是疾病的初始表现。此外,该疾病的非神经和神经发作之间的严格相互依存关系不容易识别。在这种情况下,组织学确诊往往困难。因此,如我们的病例所示(平均27个月),疾病的首个神经症状与神经结节病诊断之间的间隔可能很长。对疾病进程进行了平均8.1年(范围1 - 15年)的随访。7例患者在接受皮质类固醇治疗后至少部分从进行性疾病中恢复。1例患者亚急性发生致命的脑桥 - 延髓综合征。潜在的不良预后强调了诊断工作的重要性。