Xeroderma pigmentosum with versive seizures.
A boy with group A xeroderma pigmentosum and a series of progressive neurologic complications developed versive seizures at 8 years of age. Electroencephalography at 6 years of age revealed no epileptic changes or focal abnormalities. He was seizure-free until versive seizures developed; electroencephalography revealed frequent spike discharges in the right central and temporal regions. Neurologic complications and electroencephalographic abnormalities of 34 patients with group A xeroderma pigmentosum also were assessed. Only 3 patients with xeroderma pigmentosum have been reported to have a seizure disorder. This patient is the fourth reported with group A xeroderma pigmentosum associated with a convulsive disorder. Although neurologic manifestations in group A xeroderma pigmentosum are progressive and severe, it is unknown why so few of these patients develop seizure disorders.
Mimaki
T
T
Department of Pediatrics, Osaka University Medical School, Japan.
Ito
N
N
Tagawa
T
T
Yabuuchi
H
H
D001921:Brain / Q000503:physiopathology; D002648:Child; D004569:Electroencephalography; D006801:Humans; D008297:Male; D012640:Seizures / Q000209:etiology* / Q000503:physiopathology; D014983:Xeroderma Pigmentosum / Q000150:complications* / Q000503:physiopathology