title: MELAS and reversible vasoconstriction of the major cerebral arteries.
abstract: A 26-year-old woman was admitted due to an altered mental status and generalized tonic-clonic seizures. She had experienced chronic migraine-like headaches, progressive bilateral hearing loss, a short stature and nephrotic syndrome. Laboratory data showed elevated lactate and pyruvate levels. Brain MRI using diffusion-weighted imaging revealed a hyperintense lesion in the left temporal lobe. MR angiography revealed segmental stenosis at the C1 and M1-2 junction. A genetic study revealed a mitochondrial DNA A3243G point mutation. The patient's clinical symptoms and MRI/MR angiography (MRA) findings improved within four weeks. We herein discuss the possible pathophysiology involving both stroke-like episodes and reversible vasoconstriction.
authors.affiliation: Department of Neurology, Okinawa Chubu Hospital, Japan. yoshida_takeshi@hosp.pref.okinawa.jp
authors.forename: Takeshi
authors.initials: T
authors.lastname: Yoshida
authors.forename: Atsushi
authors.initials: A
authors.lastname: Ouchi
authors.forename: Daisuke
authors.initials: D
authors.lastname: Miura
authors.forename: Kunihiro
authors.initials: K
authors.lastname: Shimoji
authors.forename: Kazushi
authors.initials: K
authors.lastname: Kinjo
authors.forename: Takeshi
authors.initials: T
authors.lastname: Sueyoshi
authors.forename: Manabu
authors.initials: M
authors.lastname: Jonosono
authors.forename: Vijay
authors.initials: V
authors.lastname: Rajput
mesh_terms: D000328:Adult; D004272:DNA, Mitochondrial / Q000235:genetics; D038524:Diffusion Magnetic Resonance Imaging; D005260:Female; D006801:Humans; D017241:MELAS Syndrome / Q000235:genetics / Q000503:physiopathology*; D018810:Magnetic Resonance Angiography; D017354:Point Mutation; D012074:Remission Induction; D012640:Seizures / Q000235:genetics / Q000503:physiopathology; D020301:Vasospasm, Intracranial / Q000235:genetics / Q000503:physiopathology*
chemical_list: D004272:DNA, Mitochondrial