Neurosarcoidosis - a patient series. / Nevrosarkoidose ­ en pasientserie.

BACKGROUND: Neurosarcoidosis is a rare form of sarcoidosis that affects the nervous system. The aim of the study was to survey clinical manifestations, findings from assessments and treatment strategies for patients with neurosarcoidosis. MATERIAL AND METHOD: The study performed a retrospective assessment of 17 patients with definitive, probable and possible neurosarcoidosis diagnosed in the period 2008-2019 at the Department of Neurology, Haukeland University Hospital. RESULTS: The average prevalence of definitive, probable or possible neurosarcoidosis in Norway's Vestland county was 2.7 per 100 000 inhabitants in the period in question. Onset took the form of central nervous affection (8 of 17), hydrocephalus (5 of 17) and cranial neuropathy (5 of 17). Sarcoidosis-like findings were made in 14 of 17 patients by means of contrast-enhanced magnetic resonance tomography (MRT) of the central nervous system, in 7 of 8 patients by positron emission tomography (PET), and in 12 of 16 patients by computed tomography (CT) of the thorax. There were cerebrospinal fluid abnormalities in 15 of 15 patients, with biopsy verification for 13 of 15. The symptoms of 16 of 17 patients improved or stabilised with prednisolone and/or other immunotherapy. INTERPRETATION: Neurosarcoidosis affects both the central and the peripheral nervous system. Cerebrospinal fluid analysis and contrast-enhanced MRT are important means of detecting inflammation. A biopsy is necessary for making a definitive diagnosis, but is not always feasible. PET can be used as a supplement to other examinations to assess various organ manifestations and to pinpoint biopsy sites. Corticosteroid therapy, and in some cases other immunotherapy, elicits a good response.