title: Moyamoya disease presenting as thalamic hemorrhage in a patient with neuromyelitis optica and Sjögren's syndrome.
abstract: A 52-year-old woman was admitted to the hospital with right thalamic hemorrhage. A carotid angiogram revealed occlusion of the terminal portions of the bilateral internal carotid arteries with basal moyamoya vessels, which was diagnosed as moyamoya disease (MMD). At 31 years of age, she was diagnosed with multiple sclerosis because of optic neuritis and myelitis. Paraplegia appeared 14 days after admission. T2-weighted thoracic magnetic resonance imaging revealed a high intensity lesion extending from T4 to T6. Her left upper limb was partially paralytic and her lower limbs exhibited paraplegia and dysesthesia. Anti-aquaporin 4 and anti-Sjögren's syndrome-A and -B antibodies were positive. The pathogenesis of neuromyelitis optica may be associated with such immunologic factors, but there are no reports of simultaneous presentations of neuromyelitis optica and MMD. Autoimmunity may be associated with the etiology of MMD.
authors.affiliation: Department of Neurology at Tottori Prefectural Central Hospital, Tottori, Japan. yasuyasu_005@mail.goo.ne.jp
authors.forename: Yasumasa
authors.initials: Y
authors.lastname: Asai
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Nakayasu
authors.forename: Emi
authors.initials: E
authors.lastname: Fusayasu
authors.forename: Kenji
authors.initials: K
authors.lastname: Nakashima
mesh_terms: D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers / Q000097:blood; D002533:Cerebral Angiography; D002543:Cerebral Hemorrhage / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology / Q000628:therapy; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009072:Moyamoya Disease / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D009460:Neurologic Examination; D009471:Neuromyelitis Optica / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D010264:Paraplegia / Q000209:etiology; D011237:Predictive Value of Tests; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D013786:Thalamic Diseases / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology / Q000628:therapy; D013997:Time Factors; D014057:Tomography, X-Ray Computed
chemical_list: D001323:Autoantibodies; D015415:Biomarkers