Sexual Functioning in Women with Mild and Severe Symptoms of Charcot-Marie-Tooth Disease

Abstract

Introduction 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. Aim 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. Methods 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. Main Outcome Measure The main outcome measures are the factors MFSQ-SEX and MFSQ-PARTNER, which describe sexual functioning and sexual satisfaction with a partner, respectively. Results 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. Conclusions 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.

Urogenital dysfunction in male patients with Charcot-Marie-Tooth: a systematic review

“…A total of 131 records were initially identified though database searching. Among them, nine [11,12,14,[16][17][18][19][20][21] were assessed for eligibility and five were included in the qualitative analysis [11,12,17,19,21]. The flow chart of the study is shown in Figure 1.…”

“…In fact, despite the lack of literature data investigating sexuality in CMT patients, this review provides evidence for the occurrence of LUTS, erectile and ejaculation disorders in men affected by this syndrome. However, many studies used questionnaires to evaluate the presence of urogenital dysfunction [16,17], resulting in contradictory data. Therefore, the presence of ED and anejaculation needs to be investigated more in depth and through more objective methods, such as penile Doppler evaluation, basal and post-orgasm prostate vesicular ultrasound examination, and penile biothesiometry.…”

“…Such disturbances could heavily impact on the low QoL of these patients. Unfortunately, disorders of the urogenital system are rarely investigated and may have been underestimated in patients with CMT [14,16,17].…”

Anejaculation in a patient with Charcot–Marie–Tooth

“…The presence of urogenital and sexual dysfunction has been already shown in women with CMT. 5 6 Lower urinary tract dysfunction (LUTS), 7 neurogenic bladder, ED, and sexual dysfunctions have been reported in male patients with CMT, 6 7 8 but the prevalence of such disturbances in patients with CMT is unknown. Unfortunately, the literature lacks of studies describing ejaculatory disorders in these patients.…”

“…Unfortunately, the literature lacks of studies describing ejaculatory disorders in these patients. Furthermore, it should be kept in mind that the major of studies used questionnaires to evaluate the presence of urogenital dysfunction, 6 7 resulting in contradictory data. The presence of ED and anejaculation needs to be investigated through more objective methods, such as penile Doppler evaluation, basal and postorgasm prostate vesicular ultrasound examination, and penile biothesiometry.…”