Spinocerebellar ataksi 2 ve 7'de nöromelanin duyarlı görüntüleme kullanılarak substantia nigra dejenerasyonu.

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ObjectiveSpinocerebellar ataxias (SCA) are neurodegenerative diseases with widespread lesions across the central nervous system. Ataxia and spasticity are usually predominant, but patients may also present with parkinsonism. We aimed to characterize substantia nigra pars compacta (SNc) degeneration in SCA2 and 7 using neuromelanin-sensitive imaging.MethodsAtaxic and preataxic expansion carriers with SCA2 (n=15) and SCA7 (n=15) and healthy controls (n=10) were prospectively recruited. Volume and signal-to-noise ratio (SNR) values of the SNc were extracted from neuromelanin-sensitive images. ROC curves were used to determine the metrics that best differentiated SCA participants. Correlations between imaging measurements, clinical variables, and plasma neurofilaments light chain (NfL) levels were investigated.ResultsSCA2 participants had lower SNR values in the SNc than controls (110.2 ± 1.3 versus 113.2 ± 1.4; p ConclusionsNeuromelanin-sensitive imaging provides biomarkers of nigral degeneration in SCAs, detectable from the preataxic stage in SCA2, which could potentially serve as outcome measures in clinical trials.

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