Convulsive episodes in patients with group A xeroderma pigmentosum.
OBJECTIVE
To clarify the incidence of convulsive episodes in patients with group A xeroderma pigmentosum (XPA).


METHODS
By investigating the history of convulsive episodes of our 33 XPA patients through either their medical charts or direct interviews with their caretakers.


RESULTS
Five patients had several episodes of afebrile convulsion at ages older than 12. With the exception of one patient who began to show convulsive episodes at 13, no other XPA patients exhibited febrile seizures. As far as our 33 XPA patients were concerned, 15% exhibited epilepsy, and 3% experienced febrile seizures.


CONCLUSIONS
Japanese XPA patients showed a lower incidence of febrile seizures, while exhibiting a higher incidence of epilepsy. It is assumed that the brain of young patients with XPA is difficult to develop convulsions.
Kohyama
J
J
Department of Pediatrics, Tokyo Kita Shakai Hoken Hospital, Tokyo Medical and Dental University, 4-17-56 Akabanedai, Tokyo, Japan. j-kohyama@tokyokita-jadecom.jp
Furushima
W
W
Sugawara
Y
Y
Shimohira
M
M
Hasegawa
T
T
Hayashi
M
M
Moriwaki
S
S
Iwakawa
Y
Y
D000293:Adolescent; D002648:Child; D004268:DNA-Binding Proteins / Q000235:genetics; D004569:Electroencephalography; D004827:Epilepsy / Q000175:diagnosis / Q000453:epidemiology*; D005260:Female; D006801:Humans; D015994:Incidence; D007564:Japan / Q000453:epidemiology; D008297:Male; D012890:Sleep; D014851:Wakefulness; D014983:Xeroderma Pigmentosum / Q000453:epidemiology* / Q000235:genetics; D051760:Xeroderma Pigmentosum Group A Protein
D004268:DNA-Binding Proteins; C497314:XPA protein, human; D051760:Xeroderma Pigmentosum Group A Protein