PubMed1995
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.