The cause of death in pathologically confirmed amyotrophic lateral sclerosis with invasive tracheostomy ventilation, a retrospective institute-based study

Abstract

We performed retrospective institute-based prognosis study to clarify the cause of death and the clinical course in pathologically confirmed amyotrophic lateral sclerosis (ALS) patients with tracheostomy invasive ventilation (TIV). To elucidate the clinical characteristics of pathologically confirmed ALS, we retrospectively reviewed medical records of 96 consecutive autopsied patients between 2008 and 2016. Pathological diagnosis was confirmed when the following two histopathological features were observed. First of them was systematic both upper and lower motor neuronal degeneration, and second was the existence of TDP-43 inclusion body pathology and/or Bunina bodies. We identified 11 patients of pathologically diagnosed ALS with TIV (ALS-TIV) in this period. Female to male ratio was 4 to 7. Average age of death was 71.1 y.o. and median survival time was 123 months. Age of onset ranged from 43 to 82 years old, and age of death ranged from 56 to 90 years old. According to initial symptom, 5 cases were upper limb onset, 3 cases were bulbar onset, 1 case was respiratory onset, and a case was lower limb onset. All ALS-TIV patients had bronchopneumonia at their autopsied time. Respiratory insufficiency was the main cause of death in 6 of these patients. Sepsis was another main cause of death in 4 patients. There were no pulmonary embolism in this series. From this retrospective study, TIV could markedly improve prognosis of pathologically proved ALS patients. Sepsis and respiratory failures were the main cause of death in ALS-TIV.