A prospective study of 32 patients with neurosarcoidosis

Abstract

Background and aim: There are few published prospective studies of neurosarcoidosis. To establish the incidence of neurological involvement and the response to treatment in patients presenting with sarcoidosis.

Methods: From 1991 to 1994, 123 patients were studied prospectively at the Prince Charles Hospital using a purpose designed computerised database (1). Consecutive patients were referred for neurological and psychiatric assessment when clinically indicated. Nerve conduction studies were done to confirm the presence of peripheral neuropathy.

Results: Neurological involvement was identified in 32/123 patients (15 male, 17 female, all white), mean age 48 years, age range 21-80 years. Of the 32 patients, the following frequencies of abnormalities were observed: papilloedema (6%), cranial neuropathy (59%), peripheral neuropathy (47%), mononeuropathy (25%), myopathy (25%), psychiatric disorders (19%), cerebellar ataxia (13%), and hydrocephalus (6%). A neurological improvement was seen in 16/19 (84%) of our patients as a result of therapy, and in 5/13 (38%) who were untreated. Corticosteroid treatment was used in 19/32 patients, with 6/32 requiring pulse intravenous methylprednisolone for initial poor response. The most predictable response incurred in patients with peripheral neuropathy; 12/14 treated patients responding. Only 1/8 patients who remained untreated for this, improved spontaneously.

Conclusion: Neurological involvement was found more commonly than previously reported (26%). Corticosteroid treatment was found to be effective, although the response was often slow. High dose intravenous methylprednisolone was useful in poor responders (2). Peripheral neuropathy responded predictably to treatment. A formal neurological examination is recommended in all patients with sarcoidosis as neurological involvement may be overlooked.