Abstract Details

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

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P2 - Poster Session 2 (11:45 AM-12:45 PM)

Poster/Presentation
Number 11-007

Objective

We performed retrospective institute-based outcome study to clarify factors influencing prognosis of pathologically confirmed amyotrophic lateral sclerosis (ALS) patients with tracheostomy invasive mechanical ventilation (TIV).

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 outcome for a better patient’s QOL. Several clinical factors have been reported as influencing prognosis of ALS. But there are few reports of the factor which can influence the prognosis of ALS-TIV.

Design/Methods To elucidate the clinical characteristics of pathologically confirmed ALS with TIV (ALS-TIV), 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 TIV. Female to male ratio of TIV patients was 4 to 11. TIV prolonged median survival time (105 months) when compared to non-ventilation supported patients (36 months). There was positive correlation between the pre-TIV period which was duration from motor-symptom onset to starting TIV support and the post-TIV period which was duration from starting TIV support to patient’s death (r=0.577). The shorter pre-TIV significantly related to shorter post-TIV (Mann-Whitney U test, p<0.01).

Conclusions

From this retrospective study, TIV support could improve prognosis of pathologically proved ALS patients. However, rapid progression related to shorter prognosis even in ALS-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
	No disclosure on file

��ɫ��

��ɫ��