Pulmonary pathology of amyotrophic lateral sclerosis with non-invasive ventilation

Abstract

We performed retrospective hospital-based post-mortem study to clarify the clinical features and pulmonary pathology of amyotrophic lateral sclerosis (ALS) patients with non-invasive ventilation (NIV) support. To elucidate the clinical features and lung pathology of ALS with NIV support (ALS-NIV), 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. From 96 autopsied cases, we identified 8 ALS-NIV patients. Average age of death was 66.6 y.o. and mean survival time was 57.4 months. According to initial symptom, 4 cases were upper limb onset, 3 cases were bulbar onset, and a case was lower limb onset. Age of onset ranged from 50 to 77 years old, and age of death ranged from 52 to 80 years old. Right lung weight ranged from 270 g to 935 g, and left lung weight were from 300 g to 870 g. Seven out of 8 ALS-NIV patients had bronchopneumonia, and 5 of these 7 patients had severe bronchopneumonia. Lung abscess were found in 1 case. From this institute-based retrospective study, NIV support could improve prognosis of ALS patients. However, it would be difficult to avoid bronchopneumonia in the end stage of ALS-NIV. Pulmonary pathology which we showed by this post-mortem study could be useful findings to improve respiratory management of ALS-NIV.