Abstract

Charcot-Marie-Tooth (CMT) disease is one of the most frequently inherited neurological disorders, and while it is known that individuals suffering from this condition have low quality of life, little is known about their sexual function and satisfaction. To describe the functioning on different domains of sexuality in a relatively large sample of women with CMT, provide comparisons between mildly and severely affected patients and between women with the two different types of CMT (demyelinating vs. axonal), and assess the relationship between sexual function and age of onset. Fifty-seven women (age: 18-60 years) were approached in a CMT rehabilitation clinic by a psychologist and administered the Italian version of the McCoy Female Sexuality Questionnaire (MFSQ). Data from 40 patients who had had sexual intercourse in the previous 4 weeks were analyzed. The main outcome measures are the factors MFSQ-SEX and MFSQ-PARTNER, which describe sexual functioning and sexual satisfaction with a partner, respectively. Almost 30% of women did not engage in sexual intercourse with a partner. Overall sexual problems were more prominent in younger women and tended to be lower as age increased: this pattern was different from what was reported in previous studies in comparable samples of healthy Italian women. Severity of CMT was associated with better sexual functioning in the areas of desire, arousal, orgasm, and satisfaction, with women with more severe symptoms reporting greater functioning. Women with more severe CMT symptoms reported more pain during intercourse. Age of CMT onset and type of CMT (demyelinating vs. axonal) were not associated with differences in sexual functioning. Findings point to the importance of including assessment of sexual dysfunction in young women with mild CMT symptoms and the importance of providing sex therapy or counseling to these patients.

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