title: Neurosarcoidosis in a Tertiary Referral Center: A Cross-Sectional Cohort Study.
abstract: The aim of this study was to evaluate clinical characteristics, diagnostic strategy, and treatment in patients with neurosarcoidosis in a tertiary referral centre.In a cross-sectional study, we included all patients with neurosarcoidosis treated at our tertiary referral center between September 2014 and April 2015.We identified 52 patients, among them 1 patient was categorized as having definite neurosarcoidosis, 37 probable neurosarcoidosis, and 14 possible neurosarcoidosis. Neurologic symptoms were the first manifestation of sarcoidosis in 37 patients (71%). Chronic aseptic meningitis was the most common presentation (19/52 patients [37%]), followed by cranial neuropathy (16/52 patients [31%]). Serum angiotensin-converting enzyme and lysozyme levels were elevated in 18 of 41 (44%) and 12 of 26 cases (46%). Pulmonary or lymph node sarcoidosis was identified by chest X-ray in 21 of 39 cases (54%) and by computed tomography of the chest in 25 of 31 cases (81%); Fluorodeoxyglucose-Positron emission tomography showed signs of sarcoidosis in 15 of 19 cases (79%). Thirty-one of the 46 cases receiving treatment (67%) improved, 13 cases (28%) stabilized, and 2 cases (4%) deteriorated. First-line treatment with corticosteroids resulted in satisfactory reduction of symptoms in 21 of 43 patients (49%). Seventeen patients (33%) needed second-line cytostatic treatment, and 10 patients (19%) were treated with tumor necrosis factor-α inhibitors.The majority of patients with neurosarcoidosis present with chronic meningitis without a history of systemic sarcoidosis. The diagnosis can be difficult to make because of the poor sensitivity of most diagnostic tests. Half of patients had a satisfactory reduction of symptoms on first-line therapy.
authors.affiliation: From the Academic Medical Center, Center of Infection and Immunity Amsterdam (CINIMA), Department of Neurology (SEL, DF, FE, AJV, DV, MCB), Amsterdam, the Netherlands.
authors.forename: Sonja E
authors.initials: SE
authors.lastname: Leonhard
authors.forename: Daan
authors.initials: D
authors.lastname: Fritz
authors.forename: Filip
authors.initials: F
authors.lastname: Eftimov
authors.forename: Anneke J
authors.initials: AJ
authors.lastname: van der Kooi
authors.forename: Diederik
authors.initials: D
authors.lastname: van de Beek
authors.forename: Matthijs C
authors.initials: MC
authors.lastname: Brouwer
mesh_terms: D000328:Adult; D002493:Central Nervous System Diseases / Q000175:diagnosis / Q000628:therapy; D015331:Cohort Studies; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012507:Sarcoidosis / Q000175:diagnosis / Q000628:therapy; D062606:Tertiary Care Centers