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Neuromuscular sarcoidosis is recognized with increasing frequency. Although reported to occur in only 5% of patients with sarcoidosis, major centers are reporting up to 20% neuromuscular involvement. While the majority of patients with neurologic involvement present with 7th nerve palsy, the reported frequency of sarcoidosis of the brain, meninges, spinal cord, and muscles is rising.The diagnosis of neuromuscular sarcoidosis is usually accepted when a patient with previous biopsy-proven sarcoidosis presents with a compatible neurologic syndrome. In addition to cranial nerve abnormalities, these syndromes include leptomeningitis, seizures, mass lesions of brain and spinal cord, pituitary dysfunction, and neuropathies. Occasionally, the typical intrathoracic, ocular, cutaneous and lymph node presentations of sarcoidosis are not present or are unrecognized and the diagnosis is established by biopsy of neuromuscular tissue. Biopsy exhibiting granulomas is not specific and requires absence of evidence of known granulomagenic agents plus presence of multiorgan disease. An isolated granuloma in neural tissue without other organ involvement is not diagnostic of sarcoidosis.Treatment of neuromuscular sarcoidosis is usually prolonged. Facial palsy often improves spontaneously. Prednisone is still the drug of choice. Most authorities agree that 30 to 40 mg qd is sufficient with concomitant azathioprine or cyclophosphamide.