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遗传性共济失调与多系统萎缩小脑型强化康复治疗影响的差异。

Differences in the Impact of Intensive Rehabilitation on Hereditary Ataxias and the Cerebellar Subtype of Multiple System Atrophy.

机构信息

Department of Rehabilitation, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan.

Department of Rehabilitation, National Center of Neurology and Psychiatry, 4-1-1 Ogawahigashi-cho, Kodaira-shi, Tokyo, 187-8551, Japan.

出版信息

Cerebellum. 2024 Dec;23(6):2447-2456. doi: 10.1007/s12311-024-01744-4. Epub 2024 Oct 4.

Abstract

Multiple system atrophy-cerebellar type (MSA-C) exhibits faster disease progression than does hereditary spinocerebellar degeneration (hSCD). In this study, we aimed to investigate the differences in the treatment effects and sustainability of intensive rehabilitation between patients with hSCD and those with MSA-C. Forty-nine patients (hSCD = 30, MSA-C = 19) underwent a 2- or 4-week intensive rehabilitation program. Balance function was evaluated using the scale for the assessment and rating of ataxia (SARA) and the balance evaluation systems test (BESTest) at pre-intervention, post-intervention, and 6-month follow-up. Notably, both groups demonstrated beneficial effects from the rehabilitation intervention. However, differences were observed in the magnitude and duration of these effects. In the hSCD group, the SARA scores at follow-up remained similar to those at baseline, indicating sustained benefits. However, the MSA-C group showed some deterioration in SARA scores compared with baseline scores but maintained improvements on the BESTest, demonstrating partial sustainability. Differences, mainly in sustainability, were observed between the hSCD and MSA-C groups. This may be due to varying rates of symptom progression. The findings of this study are significant when considering the frequency of follow-ups based on disease type.

摘要

多系统萎缩-小脑型(MSA-C)的疾病进展速度比遗传性脊髓小脑变性(hSCD)更快。本研究旨在探讨 hSCD 与 MSA-C 患者强化康复治疗效果和可持续性的差异。49 名患者(hSCD=30,MSA-C=19)接受了为期 2 或 4 周的强化康复计划。在干预前、干预后和 6 个月随访时,使用共济失调评估量表(SARA)和平衡评估系统测试(BESTest)评估平衡功能。值得注意的是,两组患者均从康复干预中获益。然而,这些效果的幅度和持续时间存在差异。在 hSCD 组,随访时的 SARA 评分与基线相似,表明持续获益。然而,MSA-C 组的 SARA 评分与基线相比有所下降,但 BESTest 仍有改善,表明部分可持续性。hSCD 和 MSA-C 组之间观察到差异,主要表现在可持续性方面。这可能是由于症状进展速度不同。考虑到基于疾病类型的随访频率,本研究的结果具有重要意义。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/54bd/11585490/65d694bfecae/12311_2024_1744_Fig1_HTML.jpg

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