Neurochemical Differences in Spinocerebellar Ataxia Type 14 and 1.

ABSTRACT: Autosomal-dominant spinocerebellar ataxias (SCA) are neurodegenerative diseases characterized by progressive ataxia. Here, we report on neurometabolic alterations in spinocerebellar ataxia type 1 (SCA1; SCA-ATXN1) and 14 (SCA14; SCA-PRKCG) assessed by non-invasive ¹H magnetic resonance spectroscopy. Three Tesla ¹H magnetic resonance spectroscopy was performed in 17 SCA14, 14 SCA1 patients, and in 31 healthy volunteers. We assessed metabolites in the cerebellar vermis, right cerebellar hemisphere, pons, prefrontal, and motor cortex. Additionally, clinical characteristics were obtained for each patient to correlate them with metabolites. In SCA14, metabolic changes were restricted to the cerebellar vermis compared with widespread neurochemical alterations in SCA1. In SC

SUBMITTER: Grosch AS 

PROVIDER: S-EPMC8004522 | biostudies-literature | 2021 Apr

REPOSITORIES: biostudies-literature