A pilot study: handgrip as a predictor in the disease progression of SCA3

Orphanet Journal of Rare Diseases

Research

Yujun Chang¹  Huiju Chang²  Chiaju Lee³  Chungmin Chiu⁴  Henhong Chang⁵  Tatsung Lin⁶  Wenling Cheng⁶  Yongshiou Lin⁶  Chinsan Liu⁷ 

[1] Big Data Center, Changhua Christian Hospital, Changhua, Taiwan;Center of Regenerative Medicine and Tissue Repair, Institute of ATP, Changhua Christian Hospital, Changhua, Taiwan;Department of Neurology, Changhua Christian Hospital, 7F., No.235, Syuguang Rd., 500, Changhua, Taiwan;Graduate Institute of Integrated Medicine, College of Chinese Medicine, China Medical University, Taichung, Taiwan;Department of Chinese Medicine, Changhua Christian Hospital, Changhua, Taiwan;Graduate Institute of Integrated Medicine, College of Chinese Medicine, China Medical University, Taichung, Taiwan;Department of Chinese Medicine, China Medical University Hospital, No.91, Xueshi Rd., North District, 404, Taichung, Taiwan;Chinese Medicine Research Center, China Medical University, Taichung, Taiwan;Vascular and Genomic Center, Institute of ATP, Changhua Christian Hospital, Changhua, Taiwan;Vascular and Genomic Center, Institute of ATP, Changhua Christian Hospital, Changhua, Taiwan;Department of Neurology, Changhua Christian Hospital, 7F., No.235, Syuguang Rd., 500, Changhua, Taiwan;Department of Post-Baccalaureate Medicine, College of Medicine, National Chung Hsing University, Taichung, Taiwan;

关键词: Spinocerebellar ataxia type 3;    Handgrip strength;    Neurofilament light chain;    Scale for the assessment and rating of ataxia;    Body mass index;   

DOI  :  10.1186/s13023-023-02948-3

 received in 2023-06-16, accepted in 2023-10-05,  发布年份 2023

来源: Springer

【 摘 要 】

BackgroundSpinocerebellar 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).MethodsPatients 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.ResultsThis 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.ConclusionsHGS can be an alternative tool to assess the clinical severity of SCA3. Further research is needed to investigate the underlying mechanisms.

【 授权许可】

CC BY   
© Institut National de la Santé et de la Recherche Médicale (INSERM) 2023

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