Abstract Details

Title Prognosis of Pathologically Confirmed Japanese Amyotrophic Lateral Sclerosis, a Retrospective Institute-based Study

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) Neuromuscular and Clinical Neurophysiology (EMG) Posters (7:00 AM-5:00 PM)

Poster/Presentation
Number 048

Objective

We performed retrospective institute-based prognosis study to clarify the clinical course and features of pathologically confirmed amyotrophic lateral sclerosis (ALS) patients.

Background We provide long-term medical care for neuromuscular disease patients as a neuromuscular-disease center hospital in Hokuriku area of Japan. We have also developed multidisciplinary care team for ALS and other neuromuscular diseases. It is necessary to understand the prognosis for a better patient’s QOL and to know common clinical features to estimate our team intervention method.

Design/Methods To elucidate the clinical characteristics of pathologically confirmed ALS, we retrospectively reviewed medical records of 120 consecutive autopsied patients between 2008 and 2019. 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.

Results We identified 54 patients of pathologically diagnosed ALS in this period. Female to male ratio was 16 to 38. Average age of death was 69.6 y.o. (SD=10.6) and median survival time was 45.5 months (SD=50.0). Fifteen patients (28%) chose tracheostomy invasive ventilation (TIV) and 9 patients (17%) decided using non-invasive ventilation (NIV). Female to male ratio of TIV patients was 4 to 11, and that of NIV patients was 1 to 8. TIV prolonged median survival time (105 months), as did NIV (48 months) when compared to non-ventilation supported patients (36 months). Female patients tended to choose non-ventilation support. Most common cause of death was respiratory failure due to bronchopneumonia and/or hypoventilation.

Conclusions

From this retrospective study, TIV and NIV support could improve prognosis of pathologically proved ALS patients. Respiratory failures due to airway infection were the main cause of death in patients with ALS. Female patients might tend to decide not to choose NIV nor TIV.

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

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Abstract Details