Cangemi C F, Miller R J
Department of Anesthesiology, Ohio State University, Columbus 43210, USA.
Anesth Prog. 1998 Fall;45(4):150-3.
Huntington's disease is a dominantly inherited progressive autosomal disease that affects the basal ganglia. Symptoms appear later in life and manifest as progressive mental deterioration and involuntary choreiform movements. Patients with Huntington's disease develop a progressive but variable dementia. Dysphagia, the most significant related motor symptom, hinders nutrition intake and places the patient at risk for aspiration. The combination of involuntary choreoathetoid movements, depression, and apathy leads to cachexia. Factors of considerable concern to the anesthesiologist who treats patients with Huntington's disease may include how to treat frail elderly people incapable of cooperation, how to treat patients suffering from malnourishment, and how to treat patients with an increased risk for aspiration or exaggerated responses to sodium thiopental and succinylcholine. The successful anesthetic management of a 65-yr-old woman with Huntington's disease who presented for full-mouth extractions is described.
亨廷顿舞蹈症是一种常染色体显性遗传性进行性疾病，会影响基底神经节。症状在生命后期出现，表现为进行性精神衰退和不自主的舞蹈样动作。亨廷顿舞蹈症患者会出现进行性但程度不一的痴呆。吞咽困难是最主要的相关运动症状，会妨碍营养摄入，并使患者有吸入风险。不自主的舞蹈徐动症、抑郁和冷漠共同导致恶病质。治疗亨廷顿舞蹈症患者的麻醉医生相当关注的因素可能包括如何治疗无法配合的体弱老年人、如何治疗营养不良的患者，以及如何治疗有吸入风险增加或对硫喷妥钠和琥珀酰胆碱反应过度的患者。本文描述了一名65岁患亨廷顿舞蹈症的女性患者接受全口拔牙时麻醉管理成功的案例。
