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Peripheral polyneuropathy and female sexual dysfunction--familial amyloidotic polyneuropathy as an example besides diabetes mellitus.

INTRODUCTION: Female sexual dysfunction (FSD) in peripheral polyneuropathies besides diabetes mellitus is still a poorly studied subject. Little is known about sexual function in women with amyloidosis, Guillain-Barré syndrome, or porphyria. Even for the world's most common peripheral polyneuropathies such as diabetes mellitus, knowledge and consensus are still lacking. Familial amyloidotic polyneuropathy (FAP) is the most common cause of genetic systemic amyloidosis, with neurological clinical manifestations similar to diabetes mellitus. Until today, no study on the sexual function of these young female patients has been published.

AIM: To evaluate FSD in female FAP patients and to compare the results with those of healthy, non-FAP females.

METHODS: A questionnaire-based, observational study comprising 94 nonmenopausal women with a sexual partner (51 FAP and 43 non-FAP as the control group) was conducted. The Female Sexual Function Index (FSFI)--Portuguese-validated version was used to assess FSD.

MAIN OUTCOME MEASURES: Total and subscales scores of the FSFI.

RESULTS: FSD was reported by 42% (95% confidence intervals [CI] 28.3-55.7) of FAP patients compared to 12% of healthy controls. Of all the FAP patients, 39.2% reported problems with desire (95% CI 25.6-52.4), 72.5% reported problems with arousal (95% CI 60.2-84.8), 68% reported lubrication problems (95% CI 55.1-80.9), 62% reported orgasm problems (95% CI 48.5-75.5), 39.2% experienced pain (95% CI, 25.8-52.6), and 49% experienced sexual dissatisfaction (95% CI, 35.3-62.7). Even after multiple logistic regression analysis, FAP is associated with sexual dysfunction in women (OR 4.3, 95% CI 1.2-15.5, P < 0.03), and the affected domains are desire (OR 5.1, 95% CI 1.3-19.7, P < 0.02), arousal (OR 4.7, 95% CI 1.5-14.1, P < 0.007), orgasm (OR 5, 95% CI 1.6-16, P < 0.007), and sexual satisfaction (OR 4.8, 95% CI 1.4-16.9, P < 0.02). Only the use of medication with potential for sexual dysfunction was found as a significant predictor of orgasm disorder (OR 4.2, 95% CI 1.1-15.6, P < 0.03), as did age for sexual dissatisfaction (OR 1.1, 95% CI 1.0-1.2, P < 0.04).

CONCLUSIONS: FAP as a peripheral polyneuropathy results in FSD, presenting a risk factor four times greater and related to disease severity in terms of desire, arousal, and orgasm disorders, as well as sexual dissatisfaction.

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