No abstract

Exercise for patients with amyotrophic lateral sclerosis (ALS) can be expected to improve function at the early stage of disease, but improvement cannot be expected at the late stage. However, no report has investigated the correlation between the effect of exercise and time since onset. This study examined the relevance between the effect of muscle strengthening exercise and time since onset in patients with ALS.This study is a retrospective nonconsecutive case series study at a single university hospital. We included 2 patients with ALS who were admitted twice. Case 1 was a 60-year-old man with the bulbar type. He was hospitalized 10 months (ALS functional rating scale-revised, 42 points) and 1 year and 8 months (33 points) after onset. Case 2 was a 52-year-old man with the lower limb type. He was hospitalized 1 year and 3 months (44 points) and 1 year and 8 months (33 points) after onset. Physical therapy was implemented daily on weekdays for approximately 30 minutes. The intervention period was 2 weeks. Lower limb muscle strengthening exercises were mainly performed and exercise intensity was adjusted to a modified Borg Scale score of 5 (lower limbs). The study investigated knee extension muscle strength (KEMS) by using a hand-held dynamometer and Functional Ambulation Categories (FAC) at the start and end of physical therapy during each hospitalization.KEMS improved during the initial hospitalization in both patients, and FAC improved in Case 2; neither KEMS nor FAC improved during rehospitalization in either patient. In Case 1, KEMS was maintained for 10 months.The current results suggest that the positive effect of muscle strengthening exercise is greater at the early stage and may be maintained in patients with bulbar type ALS. In addition, improvement can be achieved approximately 1 year after onset and in patients with an ALSFRS-R score of 40 points or more. Therefore, it is necessary to initiate and continue exercise earlier after onset.

The muscle volume analyzer (MVA) can predict limb muscle weight based on bioelectric impedance analysis, whereas the conventional handheld dynamometer (HHD) measures muscle strength. In this study, a 26-year-old female on invasive ventilation due to late-onset Pompe disease was treated with enzyme replacement therapy (ERT) for 12 months. MVA measurements demonstrated time-dependent improvement from the baseline compared to HHD measurements, showing remarkably fluctuating muscle strength. Thus, the MVA can be used as an alternative, particularly for patients suffering from severe limb muscle weakness.

Rationale:Rehabilitation for paraneoplastic cerebellar degeneration (PCD) has been indicated as necessary. However, there are only a few reports on rehabilitation in PCD. We describe the course of physical and cognitive functions and activities of daily living (ADL) in a patient with PCD and examine the effect of rehabilitation, along with a review of relevant literature.Patient concerns:A 42-year-old woman experienced rapid deterioration in cerebellar symptoms and was unable to walk. The cerebellar symptoms improved after mastectomy, which was performed 3 months after the onset of symptoms. However, the cerebellar symptoms exacerbated 11 months after the onset of symptoms. She underwent immunotherapy because the level of anti-Yo antibodies was high, and anti-glutamic acid decarboxylase antibodies were detected.Diagnoses:She was diagnosed with anti-Yo antibody-positive PCD caused by breast cancer.Interventions:Rehabilitation was performed preoperatively, postoperatively, and at 1 year after the onset of symptoms.Outcomes:Her physical function and ADL improved after the surgery and at 1 year after the onset of symptoms. Finally, she regained the ability to walk between parallel bars supported with one hand and a walking frame.Lessons:Given the results of this case and the relevant literature, it appears that rehabilitation improves physical function and ADL after oncotherapy. Particularly, initiating treatment within three months of onset of symptoms may enable patients to walk without assistance.

[Purpose] We investigated whether patients with early-stage amyotrophic lateral sclerosis can improve their voluntary strength with a physical therapy program. [Participants and Methods] This retrospective case series study at a single university hospital included 13 patients with amyotrophic lateral sclerosis (amyotrophic lateral sclerosis functional rating scale-revised ≥35, modified functional ambulation categories score ≥4). Physical therapy was performed for 3 weeks. We investigated knee extension muscle strength and modified functional ambulation categories scores at the start and end of the therapy and calculated the improvement rate of knee extension muscle strength. We performed a regression analysis of the relationship between knee extension muscle strength at the start of the study and the improvement rate. [Results] The knee extension muscle strength improved significantly; however, the effect size was small (0.13). The modified functional ambulation categories scores did not improve significantly. The knee extension muscle strength at the start of the therapy was negatively correlated with the improvement rate (logarithmic transformed linear regression: adjusted R 2 =0.27). [Conclusion] A short-duration exercise program improved lower limb muscle strength in patients with early-stage amyotrophic lateral sclerosis. Additionally, we found that patients with weaker lower limb muscle strength at the start of the therapy demonstrated greater improvement at the end of the therapy.