Preoperative MRI findings and functional outcome after selective dorsal rhizotomy in children with bilateral spasticity

PURPOSE: To identify MRI characteristics that may predict the functional effect of selective dorsal rhizotomy (SDR) in children with bilateral spastic paresis. METHODS: We performed SDR in a group of 36 patients. The gross motor functioning measure-66 (GMFM-66) was applied before and after SDR. Available cerebral MRIs were retrospectively classified into three diagnostic groups: periventricular leucomalacia (PVL; n = 10), hydrocephalus (n = 2), and normal (n = 6). In patients with PVL, we scored the severity of the MR abnormalities. We compared the changes in the GMFM-66 after SDR in the diagnostic groups. In patients with PVL, we correlated the severity of the MR abnormalities with the changes in the GMFM-66. RESULTS: The mean follow-up period was 5 years and 4 months (range, 1 year and 1 month to 9 years). The best improvement in gross motor function was observed in patients with normal MRI, and the slightest improvement was observed in patients with hydrocephalus. The severity of the PVL did correlate with the GMFM-66 score before SDR but not with the functional effect of SDR. CONCLUSION: We conclude that with respect to gross motor skills, the improvements after SDR are good in patients with no MRI abnormalities. In the patients with hydrocephalus, the improvements after SDR were insignificant. In patients with PVL, the improvements were intermediate and did not correlate with the degree of PVL.

The updated European Consensus 2009 on the use of Botulinum toxin for children with cerebral palsy

An interdisciplinary European group of clinical experts in the field of movement disorders and experienced Botulinum toxin users has updated the consensus for the use of Botulinum toxin in the treatment of children with cerebral palsy (CP). A problem-orientated approach was used focussing on both published and practice-based evidence. In part I of the consensus the authors have tabulated the supporting evidence to produce a concise but comprehensive information base, pooling data and experience from 36 institutions in 9 European countries which involves more than 10,000 patients and over 45,000 treatment sessions during a period of more than 280 treatment years. In part II of the consensus the Gross Motor Function Measure (GMFM) and Gross Motor Function Classification System (GMFCS) based Motor Development Curves have been expanded to provide a graphical framework on how to treat the motor disorders in children with CP. This graph is named "CP(Graph) Treatment Modalities - Gross Motor Function" and is intended to facilitate communication between parents, therapists and medical doctors concerning (1) achievable motor function, (2) realistic goal-setting and (3) treatment perspectives for children with CP. The updated European consensus 2009 summarises the current understanding regarding an integrated, multidisciplinary treatment approach using Botulinum toxin for the treatment of children with CP.

Cognition and driving in older persons

In Switzerland, approximately 350,000 people aged 70 years or older own a valid driving license. By law, these drivers are medically assessed every other year, most commonly by their general practitioner, to exclude that a medical condition is interfering with their driving skills. A prerequisite for driving is the integration of high-level cognitive functions with perception and motor function. Ageing, per se, does not necessarily impair driving or increase the crash risk. However, medical conditions, such as cognitive impairment and dementia, become more prevalent with advancing age and may contribute to poor driving and an increased crash risk. The extent to which driving skills are impaired depends on the cause of dementia, disease severity, other co-morbidities and individual compensation strategies. Dementia often remains undiagnosed and therefore general practitioners (GPs) can find themselves in the difficult situation to disclose a suspicion about cognitive impairment and queries about medical fitness to drive, at the same time. In addition, the literature suggests that cognitive screening tests, most commonly used by GPs, have a limited role in judging whether an older person remains fit to drive. Further specialist assessment, for example in a memory clinic or on the road testing (ORT), may be helpful when the diagnosis or its implication for driving remain unclear. Here, we review the literature about cognition and driving, for GPs who advise older drivers who wish to continue driving.

Placebo effect characteristics observed in a single, international, longitudinal study in Huntington's disease

BACKGROUND Classically, clinical trials are based on the placebo-control design. Our aim was to analyze the placebo effect in Huntington's disease. METHODS Placebo data were obtained from an international, longitudinal, placebo-controlled trial for Huntington's disease (European Huntington's Disease Initiative Study Group). One-hundred and eighty patients were evaluated using the Unified Huntington Disease Rating Scale over 36 months. A placebo effect was defined as an improvement of at least 50% over baseline scores in the Unified Huntington Disease Rating Scale, and clinically relevant when at least 10% of the population met it. RESULTS Only behavior showed a significant placebo effect, and the proportion of the patients with placebo effect ranged from 16% (first visit) to 41% (last visit). Nondepressed patients with better functional status were most likely to be placebo-responders over time. CONCLUSIONS In Huntington's disease, behavior seems to be more vulnerable to placebo than overall motor function, cognition, and function

Outcome at two years of age in a Swiss national cohort of extremely preterm infants born between 2000 and 2008

Background While survival rates of extremely preterm infants have improved over the last decades, the incidence of neurodevelopmental disability (ND) in survivors remains high. Representative current data on the severity of disability and of risk factors associated with poor outcome in this growing population are necessary for clinical guidance and parent counselling. Methods Prospective longitudinal multicentre cohort study of preterm infants born in Switzerland between 240/7 and 276/7 weeks gestational age during 2000–2008. Mortality, adverse outcome (death or severe ND) at two years, and predictors for poor outcome were analysed using multilevel multivariate logistic regression. Neurodevelopment was assessed using Bayley Scales of Infant Development II. Cerebral palsy was graded after the Gross Motor Function Classification System. Results Of 1266 live born infants, 422 (33%) died. Follow-up information was available for 684 (81%) survivors: 440 (64%) showed favourable outcome, 166 (24%) moderate ND, and 78 (11%) severe ND. At birth, lower gestational age, intrauterine growth restriction and absence of antenatal corticosteroids were associated with mortality and adverse outcome (p < 0.001). At 360/7 weeks postmenstrual age, bronchopulmonary dysplasia, major brain injury and retinopathy of prematurity were the main predictors for adverse outcome (p < 0.05). Survival without moderate or severe ND increased from 27% to 39% during the observation period (p = 0.02). Conclusions In this recent Swiss national cohort study of extremely preterm infants, neonatal mortality was determined by gestational age, birth weight, and antenatal corticosteroids while neurodevelopmental outcome was determined by the major neonatal morbidities. We observed an increase of survival without moderate or severe disability.

To stent or not to stent perioperatively the ureteroileal anastomosis of ileal orthotopic bladder substitutes and ileal conduits? Results of a prospective randomized trial

PURPOSE: We evaluated the impact of stenting the ureteroileal anastomosis on its competence, upper urinary tract dilatation, gastrointestinal recovery, metabolic parameters and patency rate after cystectomy with ileal bladder substitution or ileal conduit. MATERIALS AND METHODS: A total of 54 patients (37 with an ileal bladder substitute and 17 with an ileal conduit) were prospectively randomized into 2 groups, with (29) or without (25) perioperative stenting of the ureteroileal anastomosis. In all cases an end-to-side ureteroileal refluxing anastomosis was performed. The stents were removed after 5 to 10 days. The parameters assessed postoperative days 1, 3 and 7 were creatinine concentration from the wound drains, upper urinary tract dilatation, time to bowel function recovery, serum creatinine, as well as urea and incidence of metabolic acidosis. RESULTS: Median patient age was 68 years (range 45 to 85). Urine leak on postoperative day 1 was more frequent in those anastomoses without stents, and on postoperative days 3 and 7 the values were comparable. Stenting of the ureteroileal anastomosis resulted in significantly decreased early postoperative upper urinary tract dilatation, improved recovery of bowel function and decreased metabolic acidosis. In either group no patient had clinical evidence of ureteroileal anastomotic stricture during the early postoperative period. Three patients with perioperative stenting required surgical or endoscopic treatment for a stricture of the ureteroileal anastomosis during the 12-month followup. CONCLUSIONS: Stenting of the ureteroileal anastomosis allows for significantly less frequent incidence of early postoperative dilatation of the pelvicaliceal system, bowel activity resumes significantly earlier and metabolic acidosis is significantly less frequent.

Impaired gesture performance in schizophrenia: particular vulnerability of meaningless pantomimes

Schizophrenia patients frequently present with subtle motor impairments, including higher order motor function such as hand gesture performance. Using cut off scores from a standardized gesture test, we previously reported gesture deficits in 40% of schizophrenia patients irrespective of the gesture content. However, these findings were based on normative data from an older control group. Hence, we now aimed at determining cut-off scores in an age and gender matched control group. Furthermore, we wanted to explore whether gesture categories are differentially affected in Schizophrenia. Gesture performance data of 30 schizophrenia patients and data from 30 matched controls were compared. Categories included meaningless, intransitive (communicative) and transitive (object related) hand gestures, which were either imitated or pantomimed, i.e. produced on verbal command. Cut-off scores of the age matched control group were higher than the previous cut-off scores in an older control group. An ANOVA tested effects of group, domain (imitation or pantomime), and semantic category (meaningless, transitive or intransitive), as well as their interaction. According to the new cut-off scores, 67% of the schizophrenia patients demonstrated gestural deficits. Patients performed worse in all gesture categories, however meaningless gestures on verbal command were particularly impaired (p = 0.008). This category correlated with poor frontal lobe function (p < 0.001). In conclusion, gestural deficits in schizophrenia are even more frequent than previously reported. Gesture categories that pose higher demands on planning and selection such as pantomime of meaningless gestures are predominantly affected and associated with the well-known frontal lobe dysfunction.

Correlations between severity of clinical signs and histopathological changes in 60 dogs with spinal cord injury associated with acute thoracolumbar intervertebral disc disease

The outcome of spinal surgery in dogs with absent voluntary motor function and nociception following intervertebral disc (IVD) herniation is highly variable, which likely attests to differences in the severity of spinal cord damage. This retrospective study evaluated the extent to which neurological signs correlated with histologically detected spinal cord damage in 60 dogs that were euthanased because of thoracolumbar IVD herniation. Clinical neurological grades correlated significantly with the extent of white matter damage (P<0.001). However, loss of nociception also occurred in 6/31 (19%) dogs with relatively mild histological changes. The duration of clinical signs, Schiff-Sherrington posture, loss of reflexes and pain on spinal palpation were not significantly associated with the severity of spinal cord damage. Although clinical-pathological correlation was generally good, some clinical signs frequently thought to indicate severe cord injury did not always correlate with the degree of cord damage, suggesting functional rather than structural impairment in some cases.

Testing visual perception in dementia with Lewy bodies and Alzheimer disease

OBJECTIVE Visuoperceptual deficits are common in dementia with Lewy bodies (DLB) and Alzheimer disease (AD). Testing visuoperception in dementia is complicated by decline in other cognitive domains and extrapyramidal features. To overcome these issues, we developed a computerized test, the Newcastle visuoperception battery (NEVIP), which is independent of motor function and has minimal cognitive load.We aimed to test its utility to identify visuoperceptual deficits in people with dementia. PARTICIPANTS AND MEASUREMENTS We recruited 28 AD and 26 DLB participants with 35 comparison participants of similar age and education. The NEVIP was used to test angle, color, and form discrimination along with motion perception to obtain a composite visuoperception score. RESULTS Those with DLB performed significantly worse than AD participants on the composite visuoperception score (Mann-Whitney U = 142, p = 0.01). Visuoperceptual deficits (defined as 2 SD below the performance of comparisons) were present in 71% of the DLB group and 40% of the AD group. Performance was not significantly correlated with motor impairment, but was significantly related to global cognitive impairment in DLB (rs = -0.689, p <0.001), but not in AD. CONCLUSION Visuoperceptual deficits can be detected in both DLB and AD participants using the NEVIP, with the DLB group performing significantly worse than AD. Visuoperception scores obtained by the NEVIP are independent of participant motor deficits and participants are able to comprehend and perform the tasks.

Three-dimensional, task-specific robot therapy of the arm after stroke: a multicentre, parallel-group randomised trial

BACKGROUND: Arm hemiparesis secondary to stroke is common and disabling. We aimed to assess whether robotic training of an affected arm with ARMin--an exoskeleton robot that allows task-specific training in three dimensions-reduces motor impairment more effectively than does conventional therapy. METHODS: In a prospective, multicentre, parallel-group randomised trial, we enrolled patients who had had motor impairment for more than 6 months and moderate-to-severe arm paresis after a cerebrovascular accident who met our eligibility criteria from four centres in Switzerland. Eligible patients were randomly assigned (1:1) to receive robotic or conventional therapy using a centre-stratified randomisation procedure. For both groups, therapy was given for at least 45 min three times a week for 8 weeks (total 24 sessions). The primary outcome was change in score on the arm (upper extremity) section of the Fugl-Meyer assessment (FMA-UE). Assessors tested patients immediately before therapy, after 4 weeks of therapy, at the end of therapy, and 16 weeks and 34 weeks after start of therapy. Assessors were masked to treatment allocation, but patients, therapists, and data analysts were unmasked. Analyses were by modified intention to treat. This study is registered with ClinicalTrials.gov, number NCT00719433. FINDINGS: Between May 4, 2009, and Sept 3, 2012, 143 individuals were tested for eligibility, of whom 77 were eligible and agreed to participate. 38 patients assigned to robotic therapy and 35 assigned to conventional therapy were included in analyses. Patients assigned to robotic therapy had significantly greater improvements in motor function in the affected arm over the course of the study as measured by FMA-UE than did those assigned to conventional therapy (F=4.1, p=0.041; mean difference in score 0.78 points, 95% CI 0.03-1.53). No serious adverse events related to the study occurred. INTERPRETATION: Neurorehabilitation therapy including task-oriented training with an exoskeleton robot can enhance improvement of motor function in a chronically impaired paretic arm after stroke more effectively than conventional therapy. However, the absolute difference between effects of robotic and conventional therapy in our study was small and of weak significance, which leaves the clinical relevance in question.

Effects of arm training with the robotic device ARMin I in chronic stroke: three single cases

BACKGROUND: Several clinical studies on chronic stroke conducted with end-effector-based robots showed improvement of the motor function in the affected arm. Compared to end-effector-based robots, exoskeleton robots provide improved guidance of the human limb and are better suited to train task-oriented movements with a large range of motions. OBJECTIVE: To test whether intensive arm training with the arm exoskeleton ARMin I is feasible with chronic-stroke patients and whether it improves motor function in the paretic arm. METHODS: Three single cases with chronic hemiparesis resulting from unilateral stroke (at least 14 months after stroke). A-B design with 2 weeks of multiple baseline measurements (A), 8 weeks of training (B) with repetitive measurements and a follow-up measurement 8 weeks after training. The training included shoulder and elbow movements with the robotic rehabilitation device ARMin I. Two subjects had three 1-hour sessions per week and 1 subject received five 1-hour sessions per week. The main outcome measurement was the upper-limb part of the Fugl-Meyer Assessment (FMA). RESULTS: The ARMin training was well tolerated by the patients, and the FMA showed moderate, but significant improvements for all 3 subjects (p < 0.05). Most improvements were maintained 8 weeks after discharge. CONCLUSIONS: This study indicates that intensive training with an arm exoskeleton is feasible with chronic-stroke patients. Moderate improvements were found in all 3 subjects, thus further clinical investigations are justified.

Effects of intensive arm training with the rehabilitation robot ARMin II in chronic stroke patients: four single-cases

BACKGROUND: Robot-assisted therapy offers a promising approach to neurorehabilitation, particularly for severely to moderately impaired stroke patients. The objective of this study was to investigate the effects of intensive arm training on motor performance in four chronic stroke patients using the robot ARMin II. METHODS: ARMin II is an exoskeleton robot with six degrees of freedom (DOF) moving shoulder, elbow and wrist joints. Four volunteers with chronic (>or= 12 months post-stroke) left side hemi-paresis and different levels of motor severity were enrolled in the study. They received robot-assisted therapy over a period of eight weeks, three to four therapy sessions per week, each session of one hour.Patients 1 and 4 had four one-hour training sessions per week and patients 2 and 3 had three one-hour training sessions per week. Primary outcome variable was the Fugl-Meyer Score of the upper extremity Assessment (FMA), secondary outcomes were the Wolf Motor Function Test (WMFT), the Catherine Bergego Scale (CBS), the Maximal Voluntary Torques (MVTs) and a questionnaire about ADL-tasks, progress, changes, motivation etc. RESULTS: Three out of four patients showed significant improvements (p < 0.05) in the main outcome. The improvements in the FMA scores were aligned with the objective results of MVTs. Most improvements were maintained or even increased from discharge to the six-month follow-up. CONCLUSION: Data clearly indicate that intensive arm therapy with the robot ARMin II can significantly improve motor function of the paretic arm in some stroke patients, even those in a chronic state. The findings of the study provide a basis for a subsequent controlled randomized clinical trial.

Selection criteria for selective dorsal rhizotomy in children with spastic cerebral palsy: a systematic review of the literature

AIM Information regarding the selection procedure for selective dorsal rhizotomy (SDR) in children with spastic cerebral palsy (CP) is scarce. Therefore, the aim of this study was to summarize the selection criteria for SDR in children with spastic CP. METHOD A systematic review was carried out using the following databases: MEDLINE, CINAHL, EMBASE, PEDro, and the Cochrane Library. Additional studies were identified in the reference lists. Search terms included 'selective dorsal rhizotomy', 'functional posterior rhizotomy', 'selective posterior rhizotomy', and 'cerebral palsy'. Studies were selected if they studied mainly children (<18y of age) with spastic CP, if they had an intervention of SDR, if they had a detailed description of the selection criteria, and if they were in English. The levels of evidence, conduct of studies, and selection criteria for SDR were scored. RESULTS Fifty-two studies were included. Selection criteria were reported in 16 International Classification of Functioning, Disability and Health model domains including 'body structure and function' (details concerning spasticity [94%], other movement abnormalities [62%], and strength [54%]), 'activity' (gross motor function [27%]), and 'personal and environmental factors' (age [44%], diagnosis [50%], motivation [31%], previous surgery [21%], and follow-up therapy [31%]). Most selection criteria were not based on standardized measurements. INTERPRETATION Selection criteria for SDR vary considerably. Future studies should describe clearly the selection procedure. International meetings of experts should develop more uniform consensus guidelines, which could form the basis for selecting candidates for SDR.

Superior functional outcome after radical cystectomy and orthotopic bladder substitution with restrictive intraoperative fluid management: a followup study of a randomized clinical trial

PURPOSE Continuous intraoperative norepinephrine infusion combined with restrictive deferred hydration improves surgical field visibility, and significantly decreases intraoperative blood loss and postoperative complications in patients undergoing radical cystectomy and urinary diversion. We determined whether the intraoperative fluid regimen would affect functional results (continence and erectile function) 1 year after orthotopic ileal bladder substitution. MATERIALS AND METHODS We analyzed a subgroup of 93 patients who received an ileal orthotopic bladder substitute. The subgroup was part of a randomized trial in 167 patients initially allocated to continuous norepinephrine administration starting with 2 μg/kg per hour combined with 1 ml/kg per hour initially and 3 ml/kg per hour crystalloid infusion after cystectomy (norepinephrine/low volume group of 51) or a standard crystalloid infusion of 6 ml/kg per hour throughout surgery (42 controls). We prospectively assessed daytime and nighttime continence, and erectile function 1 year postoperatively in the 93-patient subgroup. RESULTS Daytime continence was reported by 44 of 51 patients (86%) in the norepinephrine/low volume group and by 27 of 42 controls (64%) (p = 0.016), and nighttime continence was reported by 38 (75%) and 25 (60%), respectively (p = 0.077). Erectile function recovery was reported by 26 of 33 preoperatively potent patients (79%) in the norepinephrine/low volume group and by 11 of 29 controls (38%) (p = 0.002). CONCLUSIONS Patients who undergo radical cystectomy and orthotopic bladder substitution with continuous norepinephrine infusion and restrictive hydration during surgery have significantly better daytime continence and erectile function 1 year postoperatively.

Parental reports of the oral health-related quality of life of children with cerebral palsy

BACKGROUND The severity of physical and mental impairments and oral problems, as well as socioeconomic factors, may have an impact on quality of life of children with cerebral palsy (CP). The aim of this research was to assess the impact of impairments and oral health conditions, adjusted by socioeconomic factors, on the Oral Health-Related Quality of Life (OHRQoL) of children with CP using their parents as proxies. METHODS Sixty children, between 6-14 years of age were selected. Their parents answered a children's OHRQoL instrument (5 domains) which combines the Parental-Caregivers Perception Questionnaire (P-CPQ) and Family Impact Scale (FIS). The severity of dental caries, type of CP, communication ability, gross motor function, seizures and socioeconomic conditions were assessed. RESULTS Considering the total score of the OHRQoL instrument, only the reduction of communication ability and dental caries severity had a negative impact on the OHRQoL (p < 0.05). Considering each domain of the instrument, the severity of the type of CP and its reduction of communication ability showed a negative impact on oral symptoms and functional limitations domains (p < 0.05). Seizures have a negative impact on oral symptoms domain (p = 0.006). The multivariate fitted model showed that the severity of dental caries, communication ability and low family income were negatively associated with the impact on OHRQoL (p = 0.001). CONCLUSIONS The severity of dental caries, communication ability, and family income are conditions strongly associated with a negative impact on OHRQoL of children with CP.