Diagnosis and management of neurological sarcoidosis

Neurological involvement is a significant cause of morbidity and mortality in patients with sarcoidosis. Detection and management of neurosarcoidosis remains problematic. Our interest in immunosuppressive agents for chronic sarcoidosis has given us experience with various agents for the treatment of sarcoidosis, including cyclophosphamide and methotrexate.

We analyzed all patients with sarcoidosis seen in our clinic during a 10-year period. Evaluation for neurological disease included routine physical examination. Magnetic resonance imaging, cerebral spinal fluid analysis, and neural tissue biopsy were performed where clinically indicated. Patients were treated with corticosteroids, methotrexate, or cyclophosphamide.

Neurological disease was identified in 71 of 554 patients with sarcoidosis. Seventh (facial) cranial nerve paralysis was the most common manifestation identified in 39 patients. This included 24 patients with facial nerve palsy as the only manifestation of neurological sarcoidosis in whom complete recovery was seen in all but 1 patient. Forty-eight patients with disease other than facial nerve palsy received corticosteroids or other therapies. Corticosteroids benefited only 14 patients (29%). Methotrexate successfully treated 17 (61%) of 28 patients and cyclophosphamide controlled disease in 9 (90%) of 10 assessable patients. Methotrexate and cyclophosphamide were each associated with a higher response rate than corticosteroids alone (chi 2, 14.6; P < .001).

Neurological symptoms can be significant manifestations of sarcoidosis. Facial nerve paralysis is a common, but usually self-limited form of disease. Other manifestations are usually chronic and agents other than corticosteroids appear to have increased efficacy with lower morbidity.