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[Neurologic forms of sarcoidosis]
1Hôpital du Perpétuel Secours, Levallois-Perret.
Background:
Neurosarcoidosis is an uncommon but severe, sometimes life threatening, manifestation of sarcoidosis. Signs of neurological involvement usually are seen in patients known to have active disease. Strictly neurological forms are seen in less than 10% of cases.
Clinic:
Neuropsychic manifestations are the most common clinical signs, independent of corticosteroid therapy or neuroendocrine involvement, epileptic seizures, and signs related to hypocephalia.
Diagnosis:
Diagnosis of systemic sarcoidosis is confirmed on the basis of clinical arguments and laboratory findings favoring neurosarcoidosis. Key investigations include angiotensin converting enzyme assay in cerebrospinal fluid, and brain stem magnetic resonance imaging with gadolinium injection. Nerve biopsies may be needed in certain cases.
Treatment:
Corticosteroid therapy is given as first line treatment with a satisfactory effect in most cases. Immunosuppressors may be added in case of failure.
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