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Spinal and bulbar muscular atrophy (SBMA) progressively impairs gait function, resulting in the need for patients to use a wheelchair approximately 20 years after onset. No reports have investigated the effects of long-term exercise training using the Hybrid Assisted Limb (HAL) in patients with multiple SBMA. This study investigated the effects of long-term exercise training using HAL in patients with SBMA and its effects on the quality of life (QoL). Six courses of HAL treatment were administered to three males with SBMA, and leuprorelin was administered to each patient. Each course had a 4-5 week duration, during which the treatment was performed nine times, with a rest period of at least 2 months between each course. A 2-minute walk test (2MWT) and a 10-m walk test (10MWT) were performed to measure gait ability, and a blood test to measure the serum creatine kinase (CK) and creatinine (CRE) levels was performed before and after each course of treatment. We evaluated QoL using the Medical Outcomes Study 36-Item Short Form Health Survey (SF-36). The average 2MWT distance improved over 2 years (p = 0.038), and the 10MWT showed neither improvement nor decline. No increase or decrease in serum CK or CRE levels was observed. There were no significant changes in the SF-36 physical, mental, or social summary scores. In combination with leuprorelin therapy, robot-assisted training using HAL maintained gait ability and QoL in patients with SBMA for 2 years., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024. Published by Elsevier Ltd.)

Disaster preparation is an important issue for patients with amyotrophic lateral sclerosis (ALS). However, to the best of our knowledge, no studies have investigated disaster preparedness among patients with ALS. In this study, we aimed to investigate disaster preparation in patients with ALS and their caregivers, including their families, in Japan. We conducted a nationwide webinar in September 2022 titled "ALS Café" and distributed a self-report questionnaire to participants with questions about awareness of disaster preparedness, social countermeasures, stockpiles, and electricity demand. Forty-eight patients with ALS (27 male; average age 60.0 ± 9.3 years) and 23 caregivers (8 male; 55.7 ± 9.9 years) responded. The median revised ALS Functional Rating Scale score was 30.5, and 25% of the patients with ALS were on a ventilator. More than 70% of the respondents answered that they were not prepared for disasters, increasing to 89% in patients not using ventilators. In the event of their phones being down, 86% of the respondents had no plans for alternative means of communication. <30% of the respondents, including ventilator users, had secured human resources for transportation. Twenty-five percent of the respondents did not stockpile food and beverages, and 12% of the ventilator users had no government-recommended ventilator preparation equipment. Thus, although patients with ALS and their families with ventilators have a high awareness of disaster preparedness, their awareness remains insufficient. Furthermore, patients with ALS and their families without ventilators have a low awareness of disaster preparedness. Therefore, better education regarding disaster preparedness is necessary for these groups., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Objective: The Hybrid Assistive Limb (HAL; CYBERDYNE, Inc., Japan) is a wearable robot device that provides effective gait assistance according to voluntary intention by detecting weak bioelectrical signals of neuromuscular activity on the surface of the skin. We used HAL for patients with amyotrophic lateral sclerosis (ALS) to determine whether HAL training had an effect on their gait ability., Methods: We conducted a single-center, single-arm, observational study. Patients with ALS underwent HAL training once per day (20-40 min per session) for 9-10 days for at least 4 weeks. Gait ability was evaluated using the 2-minute walk test, the 10-meter walk test without the assistance of HAL, and activities of daily living (ADL) using the Barthel Index and Functional Independence Measures before and after a full course of HAL training., Results: There were no dropouts or adverse events during the observation period. Gait function improved after HAL training. The 2-minute walk test revealed a mean gait distance of 73.87 m (36.65) at baseline and 89.9m (36.70) after HAL training (p = 0.004). The 10-meter walk test showed significantly improved cadence, although gait speed, step length on the 10-m walk, or ADL measurements did not change significantly., Conclusions: Although HAL is not a curative treatment for ALS, our data suggest that HAL may be effective in ameliorating and preserving gait ability in patients with ALS., (Copyright © 2022 The Authors. Published by Elsevier Ltd.. All rights reserved.)