NEUROSARCOIDOSIS OF THE CENTRAL-NERVOUS-S YSTEM - ANALYSIS OF THE LONG-TERM COURSE IN 8 CASES

We present retrospective and follow-up data of eight patients with sar coidosis involving the central nervous system (CNS). The diagnosis was established definitely by histological evidence in seven cases. Biopo sies were taken directly from the CNS or the covering meninges in two of them. Additionally, we included a further case with the characteris tic traid of arthralgia, erythema nodosum and pulmonary hilar adenopat hy on X-rat, representing Lofgren's syndrome. Systemic manifestation o f sarcoidosis was hardly recognized in some cases and sometimes became evident only in the long term after recurring manifestations. The sym ptomatology in our cases can be summarized as follows: Mostly, meningo polyneuritic and meningoencephalopathic syndromes occurred, with, in a ddition, one case of chronic meningitis and two cases of spinal involv ement. In two cases, CNS symptoms represented the initial manifestatio n of the disease. Furthermore, the stringent interdependency of non-ne urologic and neurologic episodes of the disease was not recognized eas ily. Under these circumstances, the histological confirmation was ofte n 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 p atient developed, subacutely, a fatal pontine-medullary syndrome. The potentially poor prognosis emphasizes the importance of diagnostic eff orts.