Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2025 Jan 25;24(2):37.
doi: 10.1007/s12311-024-01769-9.

Assessment of Peripheral Neuropathy Using Current Perception Threshold Measurement in Patients with Spinocerebellar Ataxia Type 3

Xia-Hua Liu  1   2 , Wei Lin  3   4   5 , Hao-Ling Xu  5 , Mao-Lin Cui  6 , Zhuo-Ying Huang  3   4   5 , Ying Li  7 , Nan-Nan Zhang  1   2 , Ning Wang  8   9   10 , Zhi-Yong Wang  11   12 , Shi-Rui Gan  13   14   15 ; OSCCAR Investigators
Affiliations

Assessment of Peripheral Neuropathy Using Current Perception Threshold Measurement in Patients with Spinocerebellar Ataxia Type 3

Xia-Hua Liu et al. Cerebellum. .

Abstract

Peripheral neuropathy (PN) identified as a significant contributor to disability in Spinocerebellar ataxia type 3 (SCA3) patients. This study seeks to assess the utility of current perception threshold (CPT) measurements in evaluating PN in individuals with SCA3 and aims to identify factors influencing CPT values in SCA3 and ascertain whether these values correlate with the severity of ataxia. Ninety-four patients diagnosed with SCA3 and 44 healthy controls were recruited for this investigation. All participants were performed standard CPT assessments. Comparative analysis was conducted on CPT variables between the groups. Multivariable linear regression models were employed to identify potential risk factors influencing CPT values, and to investigate the association between CPT values and the severity of ataxia in SCA3. The case group exhibited significantly higher values across all CPT variables compared to the control group (P < 0.01). Peripheral neuropathy was prevalent among SCA3 patients, with lower limb nerves demonstrating greater susceptibility than upper limb nerves. Increasing age (β = 1.813, P = 0.012) and heightened ataxia severity (β = 3.763, P = 0.013) as predictors of poorer CPT values. Gender also emerged as a predictor of CPT values. Furthermore, CPT values (β = 0.003, P = 0.013) and disease duration (β = 0.118, P < 0.001) were associated with more severe ataxia. Our findings suggest that the CPT test holds promise for assessing peripheral neuropathy in SCA3 patients and that CPT values may serve as indicators of disease severity in this population.

Keywords: Current perception threshold; Peripheral neuropathy; Spinocerebellar ataxia type 3.

PubMed Disclaimer

Conflict of interest statement

Declarations. Ethical Approval: The study was approved by the ethics committee of the First Affiliated Hospital of Fujian Medical University. Written informed consent forms were signed by all subjects. Competing Interests: The authors declare no competing interests.

References

    1. Klockgether T, Mariotti C, Paulson HL. Spinocerebellar ataxia. Nat Rev Dis Primers. 2019;5(1):24. Published 2019 Apr 11. https://doi.org/10.1038/s41572-019-0074-3 .
    1. Kawaguchi Y, Okamoto T, Taniwaki M, et al. CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1. Nat Genet. 1994;8(3):221–8. https://doi.org/10.1038/ng1194-221 . - DOI - PubMed
    1. Gan SR, Ni W, Dong Y, Wang N, Wu ZY. Population genetics and new insight into range of CAG repeats of spinocerebellar ataxia type 3 in the Han Chinese population. PLoS One. 2015;10(8):e0134405. Published 2015 Aug 12. https://doi.org/10.1371/journal.pone.0134405 .
    1. Coutinho P, Guimarães A, Pires MM, Scaravilli F. The peripheral neuropathy in Machado-Joseph disease. Acta Neuropathol. 1986;71(1–2):119–24. https://doi.org/10.1007/BF00687972 . - DOI - PubMed
    1. Colding-Jørgensen E, Sørensen SA, Hasholt L, Lauritzen M. Electrophysiological findings in a Danish family with Machado-Joseph disease. Muscle Nerve. 1996;19(6):743–50. https://doi.org/10.1002/(SICI)1097-4598(199606)19:6%3c743::AID-MUS9%3e3.... . - DOI - PubMed

LinkOut - more resources