NEUROSARCOIDOSIS

Neurologic involvement is found in 5% of patients with sarcoidosis and is often the presenting sign in these patients. All parts of the nervous system may be involved. Recovery of cranial nerve function is common, whereas cerebral and peripheral nerve lesions often heal incompletely or deteriorate. Mortality in neurosarcoidosis is 10%. The diagnosis of neurosarcoidosis demands histologic evidence of sarcoidosis in a patient with compatible multisystem disease and signs of neurologic involvement. Exclusion of other etiologic factors is essential. Biopsy of nerve tissue is seldom required. When available, contrast-enhanced MRI is the best method in visualizing neurosarcoidosis. Brain CT can be normal in one third of the patients. VEP, BAEP, and SEP may be suitable for early detection of neurologic involvement. Routine CSF findings are nonspecific and can be normal. CSF lysozyme, ACE, and β2-microglobulin are helpful in diagnosis and follow-up. Corticosteroid treatment is indicated in neurosarcoidosis. If response is unsatisfactory, increase in the dosage or addition of another immunosuppressive agent should be considered. Surgery is limited to selected cases.