title: Unique electroencephalographic change of acute encephalopathy in glutaric aciduria type 1.
abstract: We report the peculiar serial electroencephalographic (EEG) findings in a 7-year-old boy with glutaric aciduria type 1 during an episode of acute encephalopathy. The patient developed Reye-like syndrome triggered by cellulitis. Cranial magnetic resonance imaging demonstrated diffuse softening of cerebral hemisphere. The EEG on the day following onset of acute encephalopathy showed suppression burst pattern including continuous 14-15 Hz rhythmic waves at first. Then, periodic synchronous discharge appeared and lasted for about 40 minutes. Periodic synchronous discharge finally disappeared and nearly total electrocerebral silence continued. There have been no reports indicating such a change of EEG in a short period. The serial EEG changes probably reflect the process of electrical death of neurons in cerebral hemispheres.
authors.affiliation: Department of Laboratory Medicine, Nagoya City University Medical School, Japan. sfujimot@med.nagoya-cu.ac.jp
authors.forename: S
authors.initials: S
authors.lastname: Fujimoto
authors.forename: I
authors.initials: I
authors.lastname: Shibata
authors.forename: N
authors.initials: N
authors.lastname: Sugiyama
authors.forename: S
authors.initials: S
authors.lastname: Ohba
authors.forename: H
authors.initials: H
authors.lastname: Togari
authors.forename: Y
authors.initials: Y
authors.lastname: Wada
mesh_terms: D000208:Acute Disease; D001921:Brain / Q000473:pathology / Q000503:physiopathology*; D001930:Brain Injuries / Q000150:complications* / Q000503:physiopathology; D002648:Child; D004569:Electroencephalography; D005977:Glutarates / Q000652:urine*; D006801:Humans; D008052:Lipid Metabolism, Inborn Errors / Q000150:complications* / Q000503:physiopathology / Q000652:urine; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male
chemical_list: D005977:Glutarates; C035736:glutaric acid