Abstract

Multiple sclerosis neuropsychological questionnaire (MSNQ) is a brief questionnaire useful forscreening patient's and informant's self-perception of cognitive dysfunctions in daily life activities. Our study aims toevaluate the MSNQ validity in Huntington's disease (HD) mutation carriers and to correlate MSNQ scores withneurological, cognitive, and behavioral variables. The study was conducted on a sample of 107 subjects from presymptomatic to the middle stage of HDrecruited at LIRH Foundation and C.S.S. Mendel Institute in Rome. Unified Huntington's Disease Rating Scale(UHDRS), an internationally standardized and validated scale, was used to evaluate motor, functional cognitive,and behavioral domains. Our results showed that in HD subjects, MSNQ has a unidimensional factor structure. Correlationalanalyses indicated a good correlation between the MSNQ-patient version (MSNQ-p) and clinical variables,specifically with cognitive dysfunction and behavioral alterations. Moreover, higher scores in MSNQ-p wereassociated with higher motor disease and functional impairment showing that patients in advanced stage of HDperceive a greater cognitive impairment. These results confirm the questionnaire's reliability. The present study demonstrates the validity and adaptability of MSNQ in the HD populationproposing it as a cognitive tool during routine clinical follow-ups, although further research is needed to determinean optimal cut-off score for this measure.

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