Background: Neurosarcoidosis is an uncommon but severe, sometimes life thre
atening, manifestation of sarcoidosis. Signs of neurological involvement us
ually are seen in patients known to have active disease. Strictly neurologi
cal forms are seen in less than 10% of cases.
Clinic: Neuropsychic manifestations are the most common clinical signs, ind
ependent of corticosteroid therapy or neuroendocrine involvement, epileptic
seizures, and signs related to hypocephalia.
Diagnosis: Diagnosis of systemic sarcoidosis is confirmed on the basis of c
linical arguments and laboratory findings favoring neurosarcoidosis. Key in
vestigations include angiotensin converting enzyme assay in cerebrospinal f
luid, 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 s
atisfactory effect in most cases. Immunosuppressors may be added in case of
failure.