Simon Podnar1, Matija Barbic. 1. Institute of Clinical Neurophysiology, Division of Neurology, University Medical Centre Ljubljana, Slovenia. simon.podnar@kclj.si
Abstract
AIMS: To report for the first time occurrence of obstructed voiding due to excessive activity of the urethral sphincter (US) muscle in two sisters with polycystic ovaries (Fowler's syndrome). METHODS: In both patients precise micturition history was obtained. In addition, clinical neurological and gynecological examinations, cystometry, urethral pressure profile measurements, gynecological ultrasound, measurement of gonadotropic hormone levels, and concentric needle electromyography (EMG) of the US muscle were performed. RESULTS: Both sisters reported symptoms of severely obstructed voiding. Clinical examination, and filling cystometries were normal. Urethral pressures were increased (99-134 cm water). The first sister was not able to void, and the urinary flow was slow and intermittent in the second on voiding studies. Profuse complex repetitive discharges and decelerating burst activity were found on concentric needle EMG of the US in both of them. Both sisters had increased LH/FSH ratio (2.96 and 2.64), and ultrasonographic abnormalities compatible with polycystic ovaries. CONCLUSIONS: Diagnosis of Fowler's syndrome was made in both sisters. Due to very low incidence rate of this syndrome (0.2/100.000 per year), we think that it is highly unlikely to find it in two sisters just by chance. We suggest that the probable explanation is a genetic predisposition to polycystic ovaries, with which this condition has been shown to be associated. (c) 2006 Wiley-Liss, Inc.
AIMS: To report for the first time occurrence of obstructed voiding due to excessive activity of the urethral sphincter (US) muscle in two sisters with polycystic ovaries (Fowler's syndrome). METHODS: In both patients precise micturition history was obtained. In addition, clinical neurological and gynecological examinations, cystometry, urethral pressure profile measurements, gynecological ultrasound, measurement of gonadotropic hormone levels, and concentric needle electromyography (EMG) of the US muscle were performed. RESULTS: Both sisters reported symptoms of severely obstructed voiding. Clinical examination, and filling cystometries were normal. Urethral pressures were increased (99-134 cm water). The first sister was not able to void, and the urinary flow was slow and intermittent in the second on voiding studies. Profuse complex repetitive discharges and decelerating burst activity were found on concentric needle EMG of the US in both of them. Both sisters had increased LH/FSH ratio (2.96 and 2.64), and ultrasonographic abnormalities compatible with polycystic ovaries. CONCLUSIONS: Diagnosis of Fowler's syndrome was made in both sisters. Due to very low incidence rate of this syndrome (0.2/100.000 per year), we think that it is highly unlikely to find it in two sisters just by chance. We suggest that the probable explanation is a genetic predisposition to polycystic ovaries, with which this condition has been shown to be associated. (c) 2006 Wiley-Liss, Inc.