개방성 비음을 주소로 내원한 초기 루게릭병 환자 1예

Abstract

Lou Gehrig’s disease, also called amyotrophic lateral sclerosis, is a chronic progressive motor neuron disease of an unknown etiology, characterized by upper and lower motor neuron degeneration with a resultant progressive weakness of bulbar muscles or the extremities. The early symptoms may include twitching, cramping, muscle weakness of distal extremities and slurred nasal speech. Rhinolalia aperta is abnormal speech attributable to inadequate velopharyngeal closure. Hypernasality is thequality of voice in which the emission of air through the nose is excessive due to velopharyngeal insufficiency so that the voice is highpitched and speech intelligibility deteriorates. We experienced a rare case of Lou Gehrig’s disease presenting with rhinolia aperta in its early stage. We report the case with a literature review. (Korean J Otorhinolaryngol-Head Neck Surg 2008;51:1151-4)