title: Isolated Central Nervous System Vasculitis as a Manifestation of Neurosarcoidosis.
abstract: A 62-year-old male presented to our clinic with recurrent fever, skin lesions (petechiae), scleral wounds, and hilar adenomegaly. A diagnosis of sarcoidosis was established, which resolved with corticosteroid treatment. After a few months, the patient developed confusion and behavioral changes, with few objective neurological deficits. Brain magnetic resonance imaging showed slight focal meningeal enhancement (prepontine region). The level of angiotensin-converting enzymes was normal in the serum and increased in the cerebrospinal fluid. The patient was diagnosed with neurosarcoidosis, and corticosteroid treatment was prescribed, yielding good clinical response. Nine months later, the patient presented with multiple ischemic strokes, and arteriography demonstrated multiple distal irregularities in all arterial territories, suggesting cerebral vasculitis. Even with corticosteroids, cyclophosphamide, and intravenous immunoglobulin, the patient died. Vasculitis is rarely seen in association with sarcoidosis, and in this case, no systemic manifestation was observed at the time that the patient developed vasculitis.
authors.affiliation: Institute of Neurology Deolindo Couto, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil. Electronic address: macedophilippe@outlook.com.
authors.forename: Philippe Joaquim Oliveira Menezes
authors.initials: PJ
authors.lastname: Macêdo
authors.affiliation: Institute of Neurology Deolindo Couto, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.
authors.forename: Victor Calil
authors.initials: VC
authors.lastname: da Silveira
authors.affiliation: Institute of Neurology Deolindo Couto, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.
authors.forename: Luíza Tavares
authors.initials: LT
authors.lastname: Ramos
authors.affiliation: Institute of Neurology Deolindo Couto, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.
authors.forename: Felipe Resende
authors.initials: FR
authors.lastname: Nóbrega
authors.affiliation: Institute of Neurology Deolindo Couto, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.
authors.forename: Luiz Felipe Rocha
authors.initials: LF
authors.lastname: Vasconcellos
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D002545:Brain Ischemia / Q000209:etiology; D002493:Central Nervous System Diseases / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D002533:Cerebral Angiography; D003520:Cyclophosphamide / Q000627:therapeutic use; D018450:Disease Progression; D017809:Fatal Outcome; D006801:Humans; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012507:Sarcoidosis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D020521:Stroke / Q000209:etiology; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D020293:Vasculitis, Central Nervous System / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*
chemical_list: D000305:Adrenal Cortex Hormones; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide
keywords: Sarcoidosis; angiitis; neurosarcoidosis; vasculitis