Temporal Relationship between Impairment of Cerebellar Motor Learning and Deterioration of Ataxia in Patients with Cerebellar Degeneration
Temporal Relationship between Impairment of Cerebellar Motor Learning and Deterioration of Ataxia in Patients with Cerebellar Degeneration
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DOI：
10.1007/s12311-023-01545-1
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发表时间：
2023-04-28
期刊：
影响因子：
3.5
通讯作者：
Ishikawa，Kinya
中科院分区：
文献类型：
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作者：
Honda，Takeru;Matsumura，Ken;Ishikawa，Kinya
共济失调和运动学习障碍都是影响小脑的疾病的基本特征。然而，目前尚不清楚运动学习是否仅在共济失调明显表现时受损，也不知道是否可以通过检查运动学习来监测共济失调的进展，共济失调的进展速度在患有相同疾病的患者中通常不同。我们评估了40例退行性疾病患者的运动学习和共济失调，多系统萎缩（MSA）、Machado-Joseph病（MJD）/脊髓小脑共济失调3型（SCA 3）、SCA 6和SCA 31]。在棱镜适应任务中将运动学习量化为适应性指数（AI），并使用共济失调评估和评级量表（SARA）对共济失调进行评分。我们发现，MSA-C和MSA-P的AI下降最明显，MJD中度下降，SCA 6和SCA 31轻度下降。总的来说，AI下降比SARA评分增加更快。有趣的是，在纯帕金森病MSA-P患者（n= 4）中AI保持正常，但当这些患者开始表现出共济失调时，AI下降到共济失调范围。与SARA评分≥ 10.5的患者相比，SARA评分< 10.5的患者在随访期间AI（dAI/dt）显著降低，表明AI对诊断早期小脑变性特别有用。我们的结论是，AI是小脑疾病进展的一个有用的标志物，并且评估患者的运动学习对于检测小脑损伤特别有价值，小脑损伤通常被帕金森综合征和其他体征所掩盖。
Ataxia and impaired motor learning are both fundamental features in diseases affecting the cerebellum. However, it remains unclarified whether motor learning is impaired only when ataxia clearly manifests, nor it is known whether the progression of ataxia, the speed of which often varies among patients with the same disease, can be monitored by examining motor learning. We evaluated motor learning and ataxia at intervals of several months in 40 patients with degenerative conditions [i.e., multiple system atrophy (MSA), Machado–Joseph disease (MJD)/spinocerebellar ataxia type 3 (SCA3), SCA6, and SCA31]. Motor learning was quantified as the adaptability index (AI) in the prism adaptation task and ataxia was scored using the Scale for the Assessment and Rating of Ataxia (SARA). We found thatAIdecreased most markedly in both MSA-C and MSA-P, moderately in MJD, and mildly in SCA6 and SCA31. Overall, theAIdecrease occurred more rapidly than the SARA score increase. Interestingly,AIs remained normal in purely parkinsonian MSA-P patients (n= 4), but they dropped into the ataxia range when these patients started to show ataxia. The decrease inAIduring follow-up (dAI/dt) was significant in patients with SARA scores < 10.5 compared with patients with SARA scores ≥ 10.5, indicating thatAIis particularly useful for diagnosing the earlier phase of cerebellar degeneration. We conclude thatAIis a useful marker for progressions of cerebellar diseases, and that evaluating the motor learning of patients can be particularly valuable for detecting cerebellar impairment, which is often masked by parkinsonisms and other signs.