Журнал клинических случаев

A Case of Spinal and Bulbar Muscular Atrophy Requiring Amputation and Airway Support

Abstract

Haruka Makinae, Taichiro Takamatsu, Akimasa Suzuki and Hidenobu Kamohara

Spinal and bulbar muscular atrophy (SBMA) is a rare neuromuscular disorder. Here, we present the case of a 53-year-old male diagnosed with SBMA, who underwent left leg amputation for a compound fracture with gas gangrene. The patient was hospitalized with gas gangrene and sepsis, and required non-invasive positive pressure ventilation, inotropes, and continuous hemodiafiltration. The patient gave consent for mechanical ventilation and underwent an amputation. Before and after the amputation, the patient had recurrent severe atelectasis of the left lower lobe, and large emboli composed of coagulated blood cells and sputum were extracted. SBMA itself is not curable, although the morbidity and complications related to SBMA can be managed using essential procedures.