Amyotrophic lateral sclerosis with respiratory onset that exhibited a significant time lag between respiratory arrest and the onset of limb weakness: a case report

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  • Yonezawa Naoki
    Department of Emergency and Critical Care Medicine, Yokohama City Minato Red Cross Hospital
  • Takei Tetsuhiro
    Department of Emergency and Critical Care Medicine, Yokohama City Minato Red Cross Hospital
  • Nakayama Yusuke
    Department of Emergency and Critical Care Medicine, Yokohama City Minato Red Cross Hospital
  • Yamada Hiroyuki
    Department of Emergency and Critical Care Medicine, Yokohama City Minato Red Cross Hospital
  • Toh Masafumi
    Department of Emergency and Critical Care Medicine, Yokohama City Minato Red Cross Hospital
  • Nagata Isao
    Department of Emergency and Critical Care Medicine, Yokohama City Minato Red Cross Hospital

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Other Title
  • 呼吸筋麻痺と四肢麻痺の時期が乖離し診断に苦慮した呼吸筋型筋萎縮性側索硬化症の1例

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Abstract

In amyotrophic lateral sclerosis (ALS) with respiratory onset, no limb weakness at the time of ventilator dependency is rare. A 78-year-old man was transferred to our emergency department with sudden dyspnea and unconsciousness. He was ambulatory and was able to commute the day before. On arrival, his respiration was nearly arrested, with PaCO2 of 138 mmHg. He was intubated and mechanically ventilated. He regained consciousness, along with improvement of hypercapnia. Ultrasonographic study revealed almost complete paralysis of the bilateral diaphragm, and his differential diagnosis included neuromuscular disease. Nevertheless, he remained ambulatory in our ICU after tracheotomy on day 7, while being dependent on a ventilator. The patient insidiously presented limb muscle atrophy and increased deep tendon reflexes. Neurogenic change in needle electromyography led to a final diagnosis of ALS on day 36. While vital capacity remained approximately 600 ml, frequent recurrence of atelectasis due to decreased airway clearance made weaning difficult and resulted in ventilator dependency. The patient remained ambulatory at the time of ICU discharge on day 118. As diagnosis of ALS is often made during an ICU stay, intensivists should include ALS in differential diagnosis of patients with respiratory muscle paralysis, even without evident limb weakness.

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