Evaluation of neuropsychological functions in patients with Friedreich ataxia before and after cognitive therapy

Friedreich ataxia (FA) is a neurodegenerative disease characterized by progressive nervous system damage resulting in severe disability. Cognitive functions and mood disorders in FA have been studied little and with conflicting results. The aim of this study was to investigate cognitive functions and mood disorders in FA subjects and the role of cognitive rehabilitation therapy (sequential treatments) performed during a scheduled study period. The executive functions of 24 subjects with FA were evaluated over one year during three separate periods of in-hospital rehabilitation. The neuropsychological evaluations performed before and after cognitive therapy did not reveal differences in the mean test scores of the MMSE, the Rey 15-item Memorization Test for long-term memory, Raven's Colored Progressive Matrices, the Phonemic Verbal Fluency Test, the Symbol Digit Modalities Test, or the Zung scale. The mean scores of the Stroop color-word interference task and of the Rey 15-item Memorization Test for short-term memory were increased at the final evaluation. This finding of long-lasting stability of neuropsychological test scores is noteworthy, as it suggests that one-year cognitive rehabilitation therapy (sequential treatments) may at least contribute to reducing cognitive decline. A cognitive rehabilitation therapy in addition to the conventional neuromotor rehabilitation treatment may improve the management of subjects with FA.