Progressive Spinal Cord Degeneration in Friedreich's Ataxia: Results from ENIGMA-Ataxia.

ABSTRACT:

Background

Spinal cord damage is a hallmark of Friedreich's ataxia (FRDA), but its progression and clinical correlates remain unclear.

Objective

The objective of this study was to perform a characterization of cervical spinal cord structural damage in a large multisite FRDA cohort.

Methods

We performed a cross-sectional analysis of cervical spinal cord (C1-C4) cross-sectional area (CSA) and eccentricity using magnetic resonance imaging data from eight sites within the ENIGMA-Ataxia initiative, including 256 individuals with FRDA and 223 age- and sex-matched control subjects. Correlations and subgroup analyses within the FRDA cohort were undertaken based on disease duration, ataxia severity, and onset age.

Results

Individuals with FRDA, relative to control subje

SUBMITTER: Rezende TJR 

PROVIDER: S-EPMC9852007 | biostudies-literature | 2023 Jan

REPOSITORIES: biostudies-literature