Urinary incontinence in familial dysautonomia.

The aim of this study was to determine the prevalence of urinary incontinence in women with familial dysautonomia (FD). A telephone survey was conducted on 68 known surviving female FD patients over 13 years of age registered with the Dysautonomia Centers in the USA and Israel. The mean age of the surveyed group was 27.1+/-9.8 years and 99% of the patients were nulliparous. The overall reported prevalence of urinary incontinence was 82% (n=56). Of the patients with incontinence, 59% (n=33) reported stress incontinence, 11% (n=6) reported urge incontinence, and 30% (n=17) reported symptoms of both, or mixed incontinence. In most women urinary loss was both small and infrequent, but 36% of women (n=20) with incontinence experienced a loss sufficient to necessitate the use of protection (panty liners, pads or diapers); in 7% (n=4) such loss occurred daily. Twelve per cent of all women with FD surveyed experienced primary nocturnal enuresis and 26% experienced nocturia. The prevalence of urinary incontinence is high in young female patients with familial dysautonomia. Neurophysiologic testing in this population may provide a better understanding of the role of the autonomic nervous system in urinary incontinence.