Movement Disorders Unit, Neurology Service, Internal Medicine Department, Hospital de Clínicas, Curitiba, Paraná, Brazil.
Neurological Diseases Group, Postgraduate Program in Internal Medicine, Internal Medicine Department, Hospital de Clínicas, Federal University of Paraná, Curitiba, Paraná, Brazil.
Acta Neurol Scand. 2021 Apr;143(4):458-463. doi: 10.1111/ane.13384. Epub 2020 Dec 10.
Limitations of functional capacity and balance are common features of the natural history of spinocerebellar ataxias (SCA). However, their onset and progression patterns differ according to subtype. The aim of our study was to compare physical functionality and balance parameters in SCA10 and SCA3 patients, correlating with clinical variables.
MATERIALS & METHODS: Cross-sectional study evaluating ninety-five SCA patients (60 with SCA3 and 35 with SCA10) with validated scales for functional independence, balance and the severity of signs and symptoms.
The groups were similar in terms of age and gender, and results were adjusted for age at symptom onset. The SCA10 patients had better results for balance and functional independence (p < 0.007). They also had lower scores for disease severity (p < 0.0002) and the subitems gait (p < 0.0005), posture (p < 0.0021) and sitting balance (p < 0.0008). Symptom progression in both groups was similar for patients with a disease duration of up to ten years, but there was a more marked decline in SCA3 patients after this period.
We have shown that disease progression as assessed by balance and physical functioning is slower in SCA10 patients than SCA3 patients, particularly after 10 years of disease. These findings are important as they can help to characterize the disease, assisting in the development of new therapies and rehabilitation programs.
运动功能和平衡能力的受限是脊髓小脑性共济失调(SCA)自然病史的共同特征。然而,根据亚型的不同,其发病和进展模式也有所不同。我们的研究目的是比较 SCA10 和 SCA3 患者的身体功能和平衡参数,并与临床变量相关联。
这是一项横断面研究,共纳入 95 例 SCA 患者(60 例 SCA3 和 35 例 SCA10),采用经过验证的量表评估其功能独立性、平衡和体征与症状严重程度。
两组患者在年龄和性别方面无差异,且结果经过症状发病年龄的调整。SCA10 患者的平衡和功能独立性结果更好(p<0.007)。他们的疾病严重程度评分也较低(p<0.0002),以及步态(p<0.0005)、姿势(p<0.0021)和坐姿平衡(p<0.0008)亚项评分也较低。两组患者在疾病持续时间长达十年内的症状进展相似,但在此之后,SCA3 患者的病情恶化更为明显。
我们发现,与 SCA3 患者相比,SCA10 患者的平衡和身体功能的疾病进展更为缓慢,特别是在疾病发生 10 年后。这些发现非常重要,因为它们有助于对疾病进行特征描述,有助于新疗法和康复方案的开发。