Late-Onset Pyridoxine-Dependent Seizures: Report of Two Cases

晚發性維乙六素依賴型痙孿：二病例報告

周明亮(Ming-Liang Chou) ； 王煇雄(Huei-Shyong Wang) ； 洪伯誠(Po-Cheng Hung) ； 孫佩傑(Pei-Chieh Sun) ； 黃嵩雀(Song-Chei Huang)

《Acta Paediatrica Sinica》 36卷6期 (1995/12) Pp. 434-437

https://doi.org/10.7097/APS.199512.0434

摘要

我們報告兩例晚發性維乙六素依賴型痙孿，一例在14個月大發生，另一例在9個月大發生。他們都以頑固性痙孿來表現。對於一般的抗癲癇藥物如DIAZEPAM，PHENOBARBITAL，PHENYITOIN，CARBAMAZEPINE，VALPROIC ACID及ACTH沒有反應，痙孿在維乙六素靜脈給予之後，就馬上停止下來，不幸二位病人在心智運動上均有發育遲緩/維乙六素依賴型痙攣是少數在嬰兒期發生且能治癒的疾病。因此對於每一位頑固性痙攣的形態，痙攣的形式，對傳統的抗癲癇藥物是否有反應，都要心存有維乙六素依賴型痙攣的可能性。

關鍵字

維乙六素依賴型痙孿 ； 頑固性痙孿

並列摘要

We report two cases of late-onset pyridoxine-dependent seizures with age onset at 14 and 9 months, respectively. Their intractable seizures were refractory to diazepam, phenobarbital, phenytoin, carbamazepine, valproic acid, and adrenocorticotropic hormone, but stopped soon after intravenous administration of pyridoxine. The psychomotor development was retarded in both cases. Pyridoxine dependency is one of the few treatable intractable seizures in infancy. It should always be kept in mind, even in patients with intractable seizures beyond the neonatal period, regardless of previous patterns of electrencephalography and seizure types and responsiveness to the conventional anticonvulsants.

並列關鍵字

pyridoxine-dependent seizures ； intractable seizures