Cognitive and functional connectivity alterations in Friedreich's ataxia.

Objective: The aim of this study was to perform the first resting-state functional MRI (RS-fMRI) analysis in Friedreich's ataxia (FRDA) patients to assess possible brain functional connectivity (FC) differences in these patients, and test their correlations with neuropsychological performances.

Methods: In total, 24 FRDA patients (M/F: 15/9, mean age 31.3 ± 15.

Emotion Recognition and Psychological Comorbidity in Friedreich's Ataxia.

Friedreich's ataxia (FRDA) is an autosomal recessive disease presenting with ataxia, corticospinal signs, peripheral neuropathy, and cardiac abnormalities. Little effort has been made to understand the psychological and emotional burden of the disease. The aim of our study was to measure patients' ability to recognize emotions using visual and non-verbal auditory hints, and to correlate this ability with psychological, neuropsychological, and neurological variables.

Normalization of timed neuropsychological tests with the PATA rate and nine-hole pegboard tests.

Introduction: Despite neurological patients show frequent physical impairment, timed neuropsychological tests do not take this into account during scoring procedures.

Objective: We propose a normalization method based on the PATA Rate Task (PRT) and on the nine-hole pegboard test (9HPT) as a measure of dysarthria and upper limb dysfunction.

Methods: We tested 65 healthy controls on timed neuropsychological tests (Attentional Matrices [AM], Trail Making Test, Symbol Digit Modalities Test, Verbal Fluencies) to determine the time spent on phonation or on hand movement during test execution.