DIAGNOSIS AND MANAGEMENT OF NEUROLOGICAL SARCOIDOSIS

Background: Neurological involvement is a significant cause of morbidi ty and mortality in patients with sarcoidosis. Detection and managemen t of neurosarcoidosis remains problematic. Our interest in immunosuppr essive agents for chronic sarcoidosis has given us experience with var ious agents for the treatment of sarcoidosis, including cyclophosphami de and methotrexate. Methods: We analyzed all patients with sarcoidosi s seen in our clinic during a 10-year period. Evaluation for neurologi cal 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 cort icosteroids, methotrexate, or cyclophosphamide. Results: Neurological disease was identified in 71 of 554 patients with sarcoidosis. Seventh (facial) cranial nerve paralysis was the most common manifestation id entified in 39 patients. This included 24 patients with facial nerve p alsy as the only manifestation of neurological sarcoidosis in whom com plete recovery was seen in all but 1 patient. Forty-eight patients wit h disease other than facial nerve palsy received corticosteroids or ot her therapies. Corticosteroids benefited only 14 patients (29%). Metho trexate successfully treated 17 (61%) of 28 patients and cyclophospham ide controlled disease in 9 (90%) of 10 assessable patients. Methotrex ate and cyclophosphamide were each associated with a higher response r ate than corticosteroids alone (chi(2), 14.6; P<.001). Conclusions: Ne urological symptoms can be significant manifestations of sarcoidosis. Facial nerve paralysis is a common, but usually self-limited form of d isease. Other manifestations are usually chronic and agents other than corticosteroids appear to have increased efficacy with lower morbidit y.