Care of a Child with Respiratory Failure due to Type 1 Glutaric Aciduria Treating by Botulin

戊二酸血症第一型是出生時戊二基輔酶A去氫酶基因(Glutaryl-CoA Dehydrogenase)代謝異常，一種罕見且重要的疾病，最重要的神經症狀包括點、局部或全身的運動障礙與肌肉低張力，治療這些錐體外路症候群，結果通常無法令人滿意，個案係罹患第一型戊二酸血症，以A型肉毒桿菌素治療錐體外路症候群，卻引發呼吸衰竭的合併症。此四歲兒童於每隔六個月注射A型肉毒桿菌素，四次療程後產生呼吸衰竭，主要因呼吸肌肉無力與反覆性吸入性肺炎所引發呼吸衰竭，終至必須長期依賴呼吸器，此類個案以A型肉毒桿菌素治療錐體外路症候群，必須小心注意毒素蓄積的問題。
BACKGROUND: An outline of this rare but important disease, Glutaric aciduria type I is an inborn error of metabolism due to the deficiency of glutaryl-CoA dehydrogenase. The most important neurological symptoms include dyskinesia and dystonia, which can be focal, segmental or generalized. Treatment of the extrapyramidal syndrome is often unsatisfactory. OBJECTIVE: The objective was to describe a case of an Glutaric aciduria type I in the treatment of generalized and focal dystonia with botulinum toxin type A, but induced complication of respiratory failure. METHODS AND MATERIALS: We present a 4-year-old boy who developed respiratory failure after the fourth injection series by botulinum toxin type A. Sixty units of toxin was injected at each series and the intertreatment interval was six months. RESULTS: This ventilatory dependent case was ventilatory muscle weakness and frequency aspiration pneumonia after injection of botulinum toxin type A. CONCLUSION: Our case alerts with the expanding use of botulinum toxin, much concern about the botulinum toxin is arising.