What is neurosarcoidosis?

Updated: Nov 13, 2018
  • Author: Gabriel Bucurescu, MD, MS; Chief Editor: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS  more...
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Sarcoidosis is a multisystem disease process of unknown etiology whose pathogenesis involves formation of an inflammatory lesion known as a granuloma. [1] Histologically, noncaseating granulomas are prominent in biopsies from lymph nodes or affected organs. The lungs are affected most frequently, but the eyes, nervous system, heart, kidneys, bones, and joints also may be affected.

Most patients with sarcoidosis do not have any symptoms; the disease often is detected on routine chest radiograph. Symptoms, if present, include cough, shortness of breath, and arthritis.

Involvement of the central nervous system, or neurosarcoidosis, occurs in 5-15% of cases of sarcoidosis. Neurosarcoidosis is a severe and sometimes life-threatening disorder.

Neurosarcoidosis generally occurs only in cases of sarcoidosis with substantial systemic involvement, and signs of neurologic involvement usually are seen in patients known to have active disease. Strictly neurologic forms are seen in fewer than 10% of patients; a subset has predominantly neuromuscular involvement.

Definitive diagnosis of neurosarcoidosis requires the exclusion of other causes of neuropathy and the identification of noncaseating sarcoid granulomas by histologic analysis of nerve and muscle biopsy specimens (see Workup).

Neurosarcoidosis has no known cure. Spontaneous remission ha s been observed, but long-term therapy often is required. Immunosuppression is the principal method of controlling the disease, and corticosteroids are the cornerstone of therapy (See Treatment).

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