Graduate Institute of Integrated Medicine, College of Chinese Medicine, China Medical University, Taichung, Taiwan.
Department of Chinese Medicine, Changhua Christian Hospital, Changhua, Taiwan.
Orphanet J Rare Dis. 2023 Oct 11;18(1):317. doi: 10.1186/s13023-023-02948-3.
Spinocerebellar ataxia type 3 (SCA3) is an inherited, autosomal, and rare neurodegenerative disease. Serum/plasma biomarkers or functional magnetic resonance imaging used to assess progression, except for neurological examinations, is either inconvenient or expensive. Handgrip strength (HGS) may be considered as a biomarker to predict the progress of SCA3 and align with the alteration of plasma neurofilament light chain (NfL) and Scale for the Assessment and Rating of Ataxia (SARA).
Patients with SCA3 and healthy subjects were recruited from Changhua Christian Hospital. SARA, body mass index (BMI), and NfL were obtained for both groups. HGS was measured using a Jamar Plus + hand dynamometer.
This study recruited 31 patients and 36 controls. HGS in the SCA3 group revealed a profound decrease (P < 0.001) compared with normal subjects. HGS also had a negative correlation with SARA (r = - 0.548, P = 0.001), NfL (r = - 0.359, P = 0.048), and a positive correlation with BMI (r = 0.680, P < 0.001). Moreover, HGS/BMI ratio correlated with SARA (r = - 0.441, P = 0.013). Controlling for gender and age, HGS still correlated with the above clinical items. The initial hypothesis was also proved in SCA3 84Q transgenic mice, showing grip strength weakness compared to normal mice.
HGS can be an alternative tool to assess the clinical severity of SCA3. Further research is needed to investigate the underlying mechanisms.
脊髓小脑性共济失调 3 型（SCA3）是一种遗传性、常染色体隐性、罕见的神经退行性疾病。除了神经检查外，用于评估进展的血清/血浆生物标志物或功能磁共振成像既不方便也昂贵。握力（HGS）可被视为预测 SCA3 进展的生物标志物，与血浆神经丝轻链（NfL）和共济失调评估量表（SARA）的改变一致。
本研究从彰化基督教医院招募了 SCA3 患者和健康受试者。为两组受试者均获得 SARA、体重指数（BMI）和 NfL。使用 Jamar Plus + 握力计测量 HGS。
本研究共纳入 31 例 SCA3 患者和 36 例健康对照者。SCA3 组的 HGS 明显下降（P<0.001）。HGS 与 SARA（r= - 0.548，P= 0.001）、NfL（r= - 0.359，P= 0.048）呈负相关，与 BMI（r= 0.680，P<0.001）呈正相关。此外，HGS/BMI 比值与 SARA（r= - 0.441，P= 0.013）相关。控制性别和年龄后，HGS 仍与上述临床项目相关。最初的假设也在 SCA3 84Q 转基因小鼠中得到了证明，与正常小鼠相比，它们的握力较弱。
HGS 可作为评估 SCA3 临床严重程度的替代工具。需要进一步研究以探讨其潜在机制。
