Zonta Marise Bueno, Teive Hélio A G, Camargo Carlos Henrique F, Meira Alex T, Lopes Neto Francisco Diego Negrão, Tensini Fernando Spina, Braga Cláudia Bonfim, Ashizawa Tetsuo, Munhoz Renato P
Movement Disorder Unit, Neurology Service, Department of Internal Medicine, Hospital de Clínicas, Federal University of Paraná, Rua General Carneiro 181, 4th Floor, Alto da Glória, Curitiba, PR 80060-900, Brazil; Neurological Disease Group, Graduate Program in Internal Medicine, Department of Internal Medicine, Hospital de Clínicas, Federal University of Paraná, Rua General Carneiro 181, 11th Floor, Alto da Glória, Curitiba, PR 80060-900, Brazil.
Neurological Disease Group, Graduate Program in Internal Medicine, Department of Internal Medicine, Hospital de Clínicas, Federal University of Paraná, Rua General Carneiro 181, 11th Floor, Alto da Glória, Curitiba, PR 80060-900, Brazil.
Clin Neurol Neurosurg. 2022 Mar;214:107150. doi: 10.1016/j.clineuro.2022.107150. Epub 2022 Feb 1.
Spinocerebellar ataxia (SCA) presents different rates of functional decline depending on the type of ataxia.
To compare the progression of disability, imbalance and severity of ataxia in patients with the three most common types of SCA in southern Brazil.
126 patients (31-SCA2, 58-SCA3 and 37-SCA10) were stratified into four groups based on disease duration. Progression rates were calculated in each group for ataxia severity (SARA), functioning (FIM-ADL and Lawton-IADL), and balance (Berg Balance Scale).
Differences across groups in terms of disease severity revealed a linear pattern of decline in SCA3, with a faster rate over time (p = 0.039) compared to SCA2 and SCA10. The pattern was nonlinear for SCA2 and SCA10, with a twofold faster rate in patients with up to seven years of disease compared to all other periods in SCA10 (p < 0.001) and to the longer follow up period in SCA2 (p = 0.049). Differences across groups regarding worsening of balance scores was significantly faster in SCA3 compared to SCA10 (p = 0.028) and SCA2 (p = 0.028). The rate of loss of independence of ADLs tended to diminish over time in the three types of ataxia and was faster in SCA3. Similarly, the rate for loss of independence (IADLs) was faster in SCA3 compared to SCA2 (p = 0.057) and significantly faster compared to SCA10 (p = 0.028).
The present findings suggest that the progression of the disease (severity/functioning/balance) varies according to the SCA subtype and the period in disease course. Progression is more linear and aggressive in patients with SCA3.
脊髓小脑共济失调（SCA）根据共济失调类型呈现出不同的功能衰退速率。
比较巴西南部三种最常见类型SCA患者的残疾进展、失衡情况和共济失调严重程度。
126例患者（31例SCA2型、58例SCA3型和37例SCA10型）根据病程分为四组。计算每组共济失调严重程度（SARA）、功能（FIM-ADL和Lawton-IADL）及平衡（伯格平衡量表）的进展率。
各疾病严重程度组间差异显示，SCA3呈线性衰退模式，与SCA2和SCA10相比，随时间推移衰退速率更快（p = 0.039）。SCA2和SCA10的模式为非线性，SCA10中病程达七年的患者衰退速率比所有其他时期快两倍（p < 0.001），SCA2中与更长随访期相比衰退速率快两倍（p = 0.049）。SCA3组平衡评分恶化速度显著快于SCA10（p = 0.028）和SCA2（p = 0.028）。三种类型共济失调中，日常生活活动（ADL）独立性丧失率随时间趋于降低，SCA3中更快。同样，SCA3中工具性日常生活活动（IADL）独立性丧失率比SCA2快（p = 0.057），比SCA10显著快（p = 0.028）。
目前研究结果表明，疾病进展（严重程度/功能/平衡）因SCA亚型和病程阶段而异。SCA3患者的进展更呈线性且更具侵袭性。
