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Hallervorden-Spatz 병 환자의 전신마취 하 치과치료 -증례보고-
Dental Treatment of a Patient with Hallervorden-Spatz Disease under General Anesthesia -A Case Report-

대한치과마취과학회지 2007년 7권 2호 p.135 ~ 138

이용기, 서광석, 김현정, 염광원, 안병덕,

소속 상세정보

이용기 ( Lee Yong-Ki ) - 서울대학교병원 마취통증의학과
서광석 ( Seo Kwang-Suk ) - 서울대학교 치과병원 치과마취과
김현정 ( Kim Hyun-Jeong ) - 서울대학교 치과병원 치과마취과
염광원 ( Yum Kwang-Won ) - 서울대학교 치과병원 치과마취과
안병덕 ( Ahn Byung-Deok ) - 서울대학교 치과병원 소아치과

KMID : 0980220070070020135

Abstract

Hallervorden-Spatz disease (HSD) is a rare autosomal recessive disorder associated with excessive iron deposition in the basal ganglia. In general, HSD is characterized by onset in first two decade of life and by the presence of extra-pyramidal dysfunction including dystonia, rigidity, choreoathetosis. Other associated features include gait and posture disturbance, intellectual decline, seizure, tremor, dysarthria. These signs and symptoms are progressive. MRI is often demonstrated hypodensity in the basal ganglia which is probably suggestive of accumulation of iron. There is no specific treatment for HSD and 45% of patients die before reaching the age of 20 years. The managements directed at specific symptoms are often helpful. Especially, some surgical procedures like pallidotomy and gastrostomy are performed under general anesthesia. There is special need for careful management because of numerous anesthetic challenges like difficulty in cooperation, life-threatening airway obstruction and possibility of aspiration. We report a successful anesthetic management in a patient with HSD for dental procedures.