Diagnosis and Management of Neurological Sarcoidosis

doi:10.1001/archinte.1997.00440370104011

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Vol. 157 No. 16, 8 SEPTEMBER 1997

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Diagnosis and Management of Neurological Sarcoidosis

Elyse E. Lower, MD; Joseph P. Broderick, MD; Thomas G. Brott, MD; Robert P. Baughman, MD

Arch Intern Med. 1997;157(16):1864-1868.

Abstract

Background
Neurological involvement is a significant cause of morbidityand mortality in patients with sarcoidosis. Detection and managementof neurosarcoidosis remains problematic. Our interest in immunosuppressiveagents for chronic sarcoidosis has given us experience withvarious agents for the treatment of sarcoidosis, including cyclophosphamideand methotrexate.

Methods
We analyzed all patients with sarcoidosis seen in our clinicduring a 10-year period. Evaluation for neurological diseaseincluded routine physical examination. Magnetic resonance imaging,cerebral spinal fluid analysis, and neural tissue biopsy wereperformed where clinically indicated. Patients were treatedwith corticosteroids, methotrexate, or cyclophosphamide.

Results
Neurological disease was identified in 71 of 554 patients withsarcoidosis. Seventh (facial) cranial nerve paralysis was themost common manifestation identified in 39 patients. This included24 patients with facial nerve palsy as the only manifestationof neurological sarcoidosis in whom complete recovery was seenin all but 1 patient. Forty-eight patients with disease otherthan facial nerve palsy received corticosteroids or other therapies.Corticosteroids benefited only 14 patients (29%). Methotrexatesuccessfully treated 17 (61%) of 28 patients and cyclophosphamidecontrolled disease in 9 (90%) of 10 assessable patients. Methotrexateand cyclophosphamide were each associated with a higher responserate than corticosteroids alone (X², 14.6; P<.001).

Conclusions
Neurological symptoms can be significant manifestations of sarcoidosis.Facial nerve paralysis is a common, but usually self-limitedform of disease. Other manifestations are usually chronic andagents other than corticosteroids appear to have increased efficacywith lower morbidity.

Arch Intern Med. 1997;157:1864-1868

Author Affiliations

From the Departments of Internal Medicine (Drs Lower and Baughman) and Neurology (Drs Broderick and Brott), University of Cincinnati Medical Center, Cincinnati, Ohio.

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