Introduction: Lower urinary tract symptoms (LUTS) and gastrointestinal (GI) problems are common in Duchenne muscular dystrophy (DMD), but not systematically assessed in regular care. We aimed to... Show moreIntroduction: Lower urinary tract symptoms (LUTS) and gastrointestinal (GI) problems are common in Duchenne muscular dystrophy (DMD), but not systematically assessed in regular care. We aimed to determine the prevalence of bladder and bowel dysfunction (BBD) in DMD patients compared with healthy controls (HC). Methods: The Childhood Bladder and Bowel Dysfunction Questionnaire (CBBDQ) based on the International Rome III criteria and the International Children's Continence Society was filled out by 57 DMD patients and 56 HC. Additionally, possible associations of BBD with, for example, medication use or quality of life were evaluated in an additional questionnaire developed by experts. Results: In 74% of patients versus 56% of HC >= 1 LUTS (n.s.) were reported, 68% of patients versus 39% of HC reported >= 1 bowel symptom (p = 0.002) and 53% of patients versus 30% of HC reported combined LUTS and bowel symptoms (p = 0.019). A negative impact of BBD on daily life functioning was reported by 42% of patients. Conclusions: These data underscore that standard screening for BBD is needed and that the CBBDQ could be of added value to optimize DMD care. Show less
Vaganee, D.; Voorham, J.; Borne, S. van de; Voorham-van der Zalm, P.; Fransen, E.; Wachter, S. de 2020
Purpose To assess the activation of the different parts of the pelvic floor muscles (PFM) upon electrical stimulation of the sacral spinal nerves while comparing the different lead electrode... Show morePurpose To assess the activation of the different parts of the pelvic floor muscles (PFM) upon electrical stimulation of the sacral spinal nerves while comparing the different lead electrode configurations. Material and Methods PFM electromyography (EMG) was recorded using an intravaginal multiple array probe with 12 electrodes pairs, which allows to make a distinction between the different sides and depths of the pelvic floor. In addition concentric needle EMG of the external anal sphincter was performed to exclude far-field recording. A medtronic InterStim tined lead (model 3889) was used as stimulation source. Standard SNM parameters (monophasic pulsed square wave, 210 microseconds, 14 Hz) were used to stimulate five different bipolar electrode configurations (3+0-/3+2-/3+1-/0+3-/1+3-) up to and around the sensory threshold. Of each EMG signal the stimulation intensity needed to evoke the EMG signals as well as its amplitude and latency were determined. Linear mixed models was used to analyse the data. Results Twenty female patients and 100 lead electrode configurations were stimulated around the sensory response threshold resulting in 722 stimulations and 12 times as many (8664) EMG recordings. A significant increase in EMG amplitude was seen upon increasing stimulation intensity (P < .0001). Large differences were noted between the EMG amplitude recorded at the different sides (ipsilateral>posterior>anterior>contralateral) and depths (deep>center>superficial) of the pelvic floor. These differences were noted for all lead electrodes configurations stimulated (P < .0001). Larger EMG amplitudes were measured when the active electrode was located near the entry point of the sacral spinal nerves through the sacral foramen (electrode #3). No differences in EMG latency could be withheld, most likely due to the sacral neuroanatomy (P > .05). Conclusions A distinct activation pattern of the PFM could be identified for all stimulated lead electrode configurations. Electrical stimulation with the most proximal electrode (electrode #3) as the active one elicited the largest PFM contractions. Show less
