Unique electroencephalographic change of acute encephalopathy in glutaric aciduria type 1

Citation
S. Fujimoto et al., Unique electroencephalographic change of acute encephalopathy in glutaric aciduria type 1, TOH J EX ME, 191(1), 2000, pp. 31-38
Citations number
14
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Medical Research General Topics
Journal title
TOHOKU JOURNAL OF EXPERIMENTAL MEDICINE
ISSN journal
0040-8727 → ACNP
Volume
191
Issue
1
Year of publication
2000
Pages
31 - 38
Database
ISI
SICI code
0040-8727(200005)191:1<31:UECOAE>2.0.ZU;2-J
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 ence phalopathy. The patient developed Reye-like syndrome triggered by celluliti s. Cranial magnetic resonance imaging demonstrated diffuse softening of cer ebral hemisphere. The EEC on the day following onset of acute encephalopath y showed suppression burst pattern including continuous 14-15 Hz rhythmic w aves at first. Then, periodic synchronous discharge appeared and lasted for about 40 minutes. Periodic synchronous discharge finally disappeared and n early total electrocerebral silence continued. There have been no reports i ndicating such a change of EEG in a short period. The serial EEG: changes p robably reflect the process of electrical death of neurons in cerebral hemi spheres.