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Abstract Details

The Cause of Death in Pathologically Confirmed Amyotrophic Lateral Sclerosis with Mechanical Ventilation Assist, a Retrospective Hospital-based Study
Neuromuscular and Clinical Neurophysiology (EMG)
P7 - Poster Session 7 (5:30 PM-6:30 PM)
1-002

We performed retrospective institute-based prognosis study to clarify the cause of death and the clinical course in pathologically confirmed amyotrophic lateral sclerosis (ALS) with mechanical ventilation assist (MVA).
In the advanced stage of ALS, most patients with ALS develop respiratory insufficiency. MVA is commonly regarded to be effective to treat respiratory insufficiency in ALS and improves survival and quality of life. But there are few autopsy reports which described cause of death in ALS patients with MVA (ALS-MVA).
To elucidate the clinical characteristics of pathologically confirmed ALS, we retrospectively reviewed medical records of 103 consecutive autopsied patients between 2008 and 2017.
We identified 17 patients of pathologically diagnosed ALS-MVA which included both tracheostomy invasive ventilation (TIV) and noninvasive ventilation (NIV). The number of patients with TIV (ALS-TIV) was 11. Average age of death was 69.1 years old and median survival time was 102 months. Age of onset ranged from 43 to 82, and age of death ranged from 52 to 90. According to initial symptom, 9 cases were upper limb onset, 4 cases were bulbar onset, 2 cases were lower limb onset, and one case was respiratory onset. All ALS-MVA patients had bronchopneumonia at their autopsied time. Respiratory insufficiency was the main cause of death in 6 of 11 patients with ALS-TIV. Sepsis was another main cause of death in 4 patients with ALS-TIV. Five of 6 patients with ALS-NIV had severe bronchopneumonia. Lung abscess were found in 1 case.

From this retrospective study, respiratory failures due to respiratory infection were the main cause of death in patients with ALS-MVA. Especially in ALS-NIV, severe bronchopneumonia was unavoidable on their advanced stage. In ALS-TIV, we emphasize that urosepsis or biliary infection often could be cause of death.
Authors/Disclosures
Kiyonobu Komai, MD (NHO Iou National Hospital)
PRESENTER 		Dr. Komai has nothing to disclose.
No disclosure on file
No disclosure on file
No disclosure on file
No disclosure on file
No disclosure on file