인공 호흡기 이탈 실패를 통해 발견한 근위축성 측삭경화증

Abstract

근위축성 측삭경화증은 그 빈도가 매우 드물지만, 호흡부전을 주 증상으로 나타낼 수 있다. 고령의 환자에서 잘설명되지 않는 호흡 부전이나, 인공 호흡기 이탈 실패 및모호한 근력 저하를 보인다면 근위축성 측삭경화증을 의심해보아야 한다. 이를 위해서는 면밀한 병력 청취 및 신경학적신체 검진이 필수적이며, 더 나아가 정확한 감별 진단을 위해서 신경과적 질환 및 검사에 대한 폭넓은 이해가 필요하다. 이러한 일련의 과정들이 환자의 진단을 돕고, 예후 및 삶의질을 향상시킬 수 있다. In most cases of amyotrophic lateral sclerosis (ALS), Respiratory muscle involvement is a late complication. Only 2.7% of ALS patients presenting with their first clinical symptoms in the extremities also experience respiratory symptoms. We report a case of an 81-year-old man with ALS, diagnosed by an unexplained failure to wean from mechanical ventilation. The patient presented with acute respiratory failure, tachypnea and respiratory acidosis. CT of the chest showed large amounts of sputum in the trachea. An endotracheal tube was inserted, and the patient was placed on volume-controlled ventilation. However, in the course of recovery, he could not be weaned from mechanical ventilation, despite the absence of cardiopulmonary impairment. Having considered other causes of respiratory failure, we diagnosed ALS after a physical and neurologic examination and electrodiagnostic testing.