Journal
EPILEPSIA
Volume 44, Issue 5, Pages 701-707Publisher
BLACKWELL PUBLISHING INC
DOI: 10.1046/j.1528-1157.2003.05302.x
Keywords
Glut-1 deficiency syndrome; epilepsy; hypoglycorrhachia; electroencephalogram; infant
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Funding
- NCRR NIH HHS [RR 00645] Funding Source: Medline
- NINDS NIH HHS [NS 37949] Funding Source: Medline
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Purpose: To characterize seizure types and electroencephalographic features of glucose transporter type I deficiency syndrome (Glut-1 DS). Methods: Twenty children with clinical and laboratory features of Glut-1 DS were evaluated. Age at seizure diagnosis, seizure classification, and response to treatment were determined by chart review. Thirty-two continuous 24-h EEG monitoring sessions and reports of 42 routine EEG studies were assessed. Results: Age at seizure diagnosis was between 4 weeks and 18 months (mean, 5 months). Seizure types were generalized tonic or clonic (14), absence (10), partial (nine), myoclonic (six), or astatic (four). During 24-h EEGs, background activity showed generalized 2.5- to 4-Hz spike-wave discharges (41%), generalized slowing or attenuation (34%), no abnormalities (34%), focal epileptiform discharges (13%), or focal slowing or attenuation (9%). No seizures were captured during 69% of the studies; the remainder had absence (19%), myoclonic (9%), or partial seizures (3%). On evaluation of routine and 24-h EEG studies, focal epileptiform discharges (24%) and slowing (11%) were more frequent in ages 0-24 months. In older children (2-8 years), generalized epileptiform discharges (37.5%) and slowing (21 %) were more common. Conclusions: In all ages, a normal interictal EEG was the most common EEG finding. When abnormalities occurred, focal slowing or epileptiform discharges were more prevalent in the infant. In older children (2 years or older), a generalized 2.5- to 4-Hz spike-wave pattern emerged. Seizure types observed included, absence, myoclonic, partial, and astatic.
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