Abstract

A 61-year-old man with amyotrophic lateral sclerosis (ALS) on tracheostomy positive pressure ventilation was brought to the emergency room due to decreased consciousness. He was diagnosed with hyperosmolar hyperglycemic nonketotic coma (HHNC) with severe systemic hypotension. He exhibited an adequate response to treatment of continuous intravenous insulin infusion and fluid replacement. On the third day, he developed acute limb ischemia due to extensive occlusion from the left common iliac artery to the femoral artery.

Highlights

  • Amyotrophic lateral sclerosis (ALS) is a progressive disease that affects the motor neurons and results in the development of non-neuronal comorbidities in addition to neuromuscular symptoms [1,2]

  • Glucose intolerance and dyslipidemia are recognized as possible complications in amyotrophic lateral sclerosis (ALS), hyperosmolar hyperglycemic nonketotic coma (HHNC) that develop in ALS are very rare in clinical settings

  • A pilot study indicated that hyperosmolar hyperglycemic state (HHS) is in a pre-DIC state during coma, and intra-arterial thrombus is likely to occur for several hours to several days [17]

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Summary

Introduction

Amyotrophic lateral sclerosis (ALS) is a progressive disease that affects the motor neurons and results in the development of non-neuronal comorbidities in addition to neuromuscular symptoms [1,2]. A 61-year-old man was diagnosed with amyotrophic lateral sclerosis (ALS) 21 years prior He developed quadriplegia and respiratory muscle paralysis and introduced tracheostomy positive pressure ventilation five years ago. In order to reduce consciousness during home-visit nursing care, the patient was moved by ambulance. His blood pressure was 55/31 mmHg, and his heart rate was 137 bpm. His level of consciousness was calculated using the Glasgow coma scale (GCS), giving the score of E4VTM1. The calculated plasma osmolality was 397 mOsm/ kgH2O His level of consciousness later deteriorated to a GCS score of E1VTM1. His limb was rescued from amputation, and he returned home 7 weeks after operation with antiplatelet drugs

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