Frequency distribution in intraoperative stimulation-evoked EMG responses during selective dorsal rhizotomy in children with cerebral palsy-part 1: clinical setting and neurophysiological procedure.

INTRODUCTION: Selective dorsal rhizotomy (SDR) consists of microsurgical partial deafferentation of sensory nerve roots (L1-S2). It is primarily used today in decreasing spasticity in young cerebral palsy (CP) patients. Intraoperative monitoring (IOM) is an essential part of the surgical decision-making process, aimed at improving functional results. The role played by SDR-IOM is examined, while realizing that connections between complex EMG responses to nerve-root stimulation and a patient's individual motor ability remain to be clarified. METHODS: We conducted this retrospective study, analyzing EMG responses in 146 patients evoked by dorsal-root and rootlet stimulation, applying an objective response-classification system, and investigating the prevalence and distribution of the assessed grades. Part1 describes the clinical setting and SDR procedure, reintroduced in Germany by the senior author in 2007. RESULTS: Stimulation-evoked EMG response patterns revealed significant differences along the segmental levels. More specifically, a comparison of grade 3+4 prevalence showed that higher-graded rootlets were more noticeable at lower nerve root levels (L5, S1), resulting in a typical rostro-caudal anatomical distribution. CONCLUSIONS: In view of its prophylactic potential, SDR should be carried out at an early stage in all CP patients suffering from severe spasticity. It is particularly effective when used as an integral part of a coordinated, comprehensive spasticity program in which a team of experts pool their information. The IOM findings pertaining to the anatomical grouping of grades could be of potential importance in adjusting the SDR-IOM intervention to suit the specific individual constellation, pending further validation. TRIAL REGISTRATION: ClinicalTrials.gov ID: NCT03079362.

Frequency distribution in intraoperative stimulation-evoked EMG responses during selective dorsal rhizotomy in children with cerebral palsy-part 2: gender differences and left-biased asymmetry.

INTRODUCTION: Spinal reflexes reorganize in cerebral palsy (CP), producing hyperreflexia and spasticity. CP is more common among male infants, and gender might also influence brain and spinal-cord reorganization. This retrospective study investigated the frequency of higher-graded EMG responses elicited by electrical nerve-root stimulation during selective dorsal rhizotomy (SDR), prior to partial nerve- root deafferentation, considering not only segmental level and body side, but also gender. METHODS: Intraoperative neuromonitoring (IOM) was used in SDR to pinpoint the rootlets most responsible for exacerbated stimulation-evoked EMG patterns recorded from lower-limb muscle groups. Responses were graded according to an objective response-classification system, ranging from no abnormalities (grade 0) to highly abnormal (grade 4+), based on ipsilateral spread and contralateral involvement. Non-parametric analysis of data with repeated measures was primarily used in investigating the frequency distribution of these various EMG response grades. Over 7000 rootlets were stimulated, and the results for 65 girls and 81 boys were evaluated, taking changes in the composition of patient groups into account when considering GMFCS levels. RESULTS: The distribution of graded EMG responses varied according to gender, laterality, and level. Higher-graded EMG responses were markedly more frequent in the boys and at lower segmental levels (L5, S1). Left-biased asymmetry in higher-graded rootlets was also more noticeable in the boys and in patients with GMFCS level I. A close link was observed between higher-grade assessments and left-biased asymmetry. CONCLUSIONS: Detailed insight into the patient's initial spinal-neurofunctional state prior to deafferentation suggests that differences in asymmetrical spinal reorganization might be attributable to a hemispheric imbalance.

MR imaging features in Marinesco-Sjögren syndrome: severe cerebellar atrophy is not an obligatory finding.

BACKGROUND AND PURPOSE: Cerebellar atrophy is considered the most prominent neuroradiologic finding in Marinesco-Sjögren syndrome (MSS). Our purpose was to investigate this neuroradiologic feature in a series of patients with MSS. METHODS: Five patients with MSS (age range, 5-19 years) underwent native MR imaging of the brain. The findings were assessed with particular attention to the cerebellum and the supratentorial structures. RESULTS: Only two patients had slight cerebellar atrophy; the cerebellum was normal in size and configuration in the other patients. Additional supratentorial findings were present in some of the patients, with an apparently small anterior pituitary gland in two and the absence of the posterior pituitary bright spot in three of the patients. CONCLUSION: Cerebellar atrophy is not an obligatory finding in MSS, and almost normal cranial MR imaging results are compatible with the diagnosis. Morphologic changes of the pituitary gland seem to be common in patients with MSS and are not associated with endocrine dysfunction.

Urodynamic effects of propiverine in children and adolescents with neurogenic bladder: results of a prospective long-term study.

OBJECTIVE: To evaluate prospectively the efficacy and tolerability of propiverine for long-term treatment of neurogenic detrusor overactivity (NDO) in children. MATERIALS AND METHODS: 17 children and adolescents with NDO (10 female, 7 male; average age at last consultation 13.0 years) were evaluated during long-term treatment with propiverine (0.8 mg/kg body weight/day). Outcome measurements included urodynamic parameters, continence, hydronephrosis and tolerability of propiverine. RESULTS: Average follow-up was 3.6 years (range 2.0-5.9). The average maximum detrusor pressure was 33.2 ± 4.8 cmH(2)O and bladder compliance was 20.0 ± 5.4 ml/cmH(2)O at the last follow-up visit. Maximum cystometric bladder capacity (MCBC) within the normal range was attained in 11 patients; it was still reduced (average of 61% of expected MCBC) in the remaining 6. Incontinence occurred on average once per day. Hydronephrosis was classified for each renal unit separately: grade 0 was measured in 26 and 22 cases, grade 1 or 2 in 6 and 8 cases, grade 3 or 4 in 2 and 4 cases pre and post treatment, respectively. In 6/17 patients adjuvant intravesical oxybutynin was applied, in 4 out of these 6 patients more invasive procedures, such as untethering, augmentation cystoplasty or botulinum toxin injections, were necessitated. Propiverine monotherapy was well tolerated in 11/17 patients. No serious adverse events were encountered during the study period. CONCLUSION: Long-term efficacy and tolerability of propiverine for NDO in children and adolescents is promising: clinically relevant improvements in key urodynamic outcomes were paralleled by improvements in incontinence score.

Longevity of implantable cardioverter-defibrillators, influencing factors, and comparison to industry-projected longevity.

BACKGROUND: Because the best possible device longevity is crucial (i.e., risk of infection with premature device exchange, current cost-effectiveness calculations depending on reasonable longevity, patient comfort), industry-independent real-life data are fundamental. However, only limited independent data on the longevity of implantable cardioverter-defibrillators (ICDs) are available. OBJECTIVE: The purpose of this study was to determine ICD device longevity and influencing factors. METHODS: From a prospective database, we studied overall device longevity and identified those devices with replacement for battery depletion or prolonged charge time. For every device, we determined factors that included averaged shocks, pacing percentage, pacing mode, device size, and time of implant. Survival probabilities at different time intervals were calculated, and Kaplan-Meier and Cox regression analyses were used. Observed longevity was compared to industry-projected longevity obtained from product performance reports. RESULTS: A total of 644 ICDs (Medtronic 317, Guidant 189, St. Jude 118, Intermedics 20) were implanted in 499 patients. During follow-up, 163 (25.3%) ICDs were replaced. Manufacturer, time of implant, pacing mode, pacing percentage, and capacitor reformation interval influenced longevity, whereas device size and number of shocks did not. Median longevity was 7.6 years for Medtronic devices, 5.0 years for Guidant devices, and 3.8 years for St. Jude devices. After 5 years, only 70% of ICDs were still in service compared to the 80% projected by industry. CONCLUSION: Marked differences in device longevity among manufacturers cannot be explained by pacing mode, number of shocks, or pacing percentage only. Overall, device performance requires further improvement for the sake of patient health and cost.

Urodynamic effects of propiverine hydrochloride in children with neurogenic detrusor overactivity: a prospective analysis.

OBJECTIVES: To evaluate prospectively the efficacy and tolerability of propiverine for treating neurogenic detrusor overactivity (NDO) in children. PATIENTS AND METHODS: Twenty children (mean age 8.9 years; median 5.6) with NDO due to an upper motor neurone lesion were enrolled (17 had myelomeningocele). In the urodynamic examination, reflex volume (RV), maximum detrusor pressure (MDP), maximum cystometric bladder capacity (MCBC) and bladder compliance (BC) before and after a twice-daily propiverine hydrochloride regimen were determined. The urodynamic follow-up was after 3-6 months. Incontinence was assessed by an incontinence score. RESULTS: The mean (sem) RV increased from 103.8 (21.3) to 174.5 (33.7) mL (P < 0.005), MDP decreased from 52.5 (7.9) to 40.1 (6.2) cmH(2)O (P < 0.05), MCBC increased from 166 (28.8) to 231.9 (34.8) mL (P < 0.005), and BC improved from 11.2 (2.8) to 30.6 (9.7) mL/cmH(2)O (P < 0.01), with propiverine treatment. The incontinence score (scale 0-3) improved from 2.4 (0.2) to 1.6 (0.3) (P < 0.05). Propiverine was well tolerated, although some children were given higher doses than recommended. CONCLUSIONS: Propiverine hydrochloride is effective and well tolerated in the treatment of children with NDO. Because of its dual mode of action, it is well tolerated even in children who need higher doses. Propiverine hydrochloride is a preferable alternative to oxybutynin, the anticholinergic most frequently used in children with NDO to date.

Repeated botulinum-A toxin injections in treatment of children with neurogenic detrusor overactivity.

OBJECTIVES: To assess the long-term success of treatment with repeated botulinum-A toxin (BTX-A; Botox) injections into the detrusor muscle for neurogenic detrusor overactivity in children. METHODS: We reviewed the charts of 10 children (average age at first injection 11.2 years) with neurogenic detrusor overactivity who had received at least three BTX-A detrusor injections; four had received five or more injections. The total dose of BTX-A was 85 to 300 U. We measured the urodynamic outcomes 6 months after each injection and compared the results after the first injection with the results after the third and fifth injections (in the children who had five or more injections). RESULTS: The relative changes--each in comparison with the value before injection therapy--after the first versus the fifth injection were as follows: the reflex volume increased by 81% versus 88%, maximal detrusor pressure decreased by 7% versus 39%, maximal cystometric bladder capacity increased by 88% versus 72%, and bladder compliance showed no change at the 6-month follow-up visit after the first injection and an increase of 109% after the fifth injection. The results after the third injection were generally similar to those after the fifth injection. No major side effects occurred. CONCLUSIONS: Although we surveyed only a few patients, the efficacy seems clear in all the urodynamic measures after repeated BTX-A detrusor injections in children with neurogenic bladder. Also, we found no evidence of drug tolerance. Thus, BTX-A detrusor injection has gained additional importance in the treatment of these difficult-to-treat patients.