Clinical Motor Function Research Articles

Ulnar Nerve Injury in Pediatric Midshaft Forearm Fractures: A Case Series.

To describe a midshaft forearm fracture pattern that places the ulnar nerve at risk in the pediatric population and provide 7 clinical case examples describing the injury pattern and treatment methods. Retrospective observational case series, review of literature, cadaver dissection, and treatment recommendations. Multi-institutional, Southeast United States. Seven pediatric patients (5 male and 2 female) with mean age of 8.7 years (range, 3-14) who sustained a significantly displaced closed, or grade I open, middle to distal one-third both-bone forearm fracture with subsequent ulnar nerve dysfunction. Manual reduction and casting of both-bone forearm shaft fractures, operative debridement, fracture fixation, nerve exploration, neurolysis, nerve repair, and nerve grafting. Radiographic fracture union, clinical ulnar nerve motor and sensory function testing, along with selective electric nerve testing and advanced imaging were monitored throughout follow-up postinjury. Five of 7 patients underwent surgical treatment and 2 others were treated with conservative measures. The ulnar nerve was entrapped within the fracture site of one patient with an open fracture along with partial nerve transection, and 4 patients were found to have the nerve encased in hypertrophic scar tissue or bony callus upon surgical exploration at 3-12 months postinjury. The ulnar nerve lies in a precarious position in the middle to distal one-third forearm and is bound by anatomic constraints that place the nerve at risk of injury. This article offers a treatment algorithm that includes conservative treatment, acute exploration, early exploration (≤3 months), and late exploration (>3 months). Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Correlation of phenotype with genotype and protein structure in RYR1-related disorders

Variants in the skeletal muscle ryanodine receptor 1 gene (RYR1) result in a spectrum of RYR1-related disorders. Presentation during infancy is typical and ranges from delayed motor milestones and proximal muscle weakness to severe respiratory impairment and ophthalmoplegia. We aimed to elucidate correlations between genotype, protein structure and clinical phenotype in this rare disease population. Genetic and clinical data from 47 affected individuals were analyzed and variants mapped to the cryo-EM RyR1 structure. Comparisons of clinical severity, motor and respiratory function and symptomatology were made according to the mode of inheritance and affected RyR1 structural domain(s). Overall, 49 RYR1 variants were identified in 47 cases (dominant/de novo, n = 35; recessive, n = 12). Three variants were previously unreported. In recessive cases, facial weakness, neonatal hypotonia, ophthalmoplegia/paresis, ptosis, and scapular winging were more frequently observed than in dominant/de novo cases (all, p < 0.05). Both dominant/de novo and recessive cases exhibited core myopathy histopathology. Clinically severe cases were typically recessive or had variants localized to the RyR1 cytosolic shell domain. Motor deficits were most apparent in the MFM-32 standing and transfers dimension, [median (IQR) 85.4 (18.8)% of maximum score] and recessive cases exhibited significantly greater overall motor function impairment compared to dominant/de novo cases [79.7 (18.8)% vs. 87.5 (17.7)% of maximum score, p = 0.03]. Variant mapping revealed patterns of clinical severity across RyR1 domains, including a structural plane of interest within the RyR1 cytosolic shell, in which 84% of variants affected the bridging solenoid. We have corroborated genotype-phenotype correlations and identified RyR1 regions that may be especially sensitive to structural modification.

P54. Differences in neuronal representation of mental rotation in CRPS patients and healthy controls

Spatial integration of the body matrix, especially representation of the affected side is reduced in patients suffering from complex regional pain syndrome (CRPS; Moseley et al., 2009 ). The parietal sulcus integrates multimodal sensory input to spatial aspects of the own body and is therefore essential for processing mental rotation (MR) (Lebon et al., 2012). Since the training of MR has turned out to be among the effective therapy strategies for CRPS (Graded motor imagery; GMI; Moseley et al., 2006), MR is also important for the pathophysiological understanding of CRPS. The aim of this study was to point out differences in neural representation of MR in a group of CRPS patients and healthy controls. Therefore we included 15 CRPS patients (DASH-Avg.: 51.54; CSS: 12.39; pain intensity 4.36 of 10 VAS; age: 58 years; CRPS duration 21 ± 20 months) and 15 age-/gender matched healthy controls. We assessed behavioral (reaction time in MR for both hands), clinical (CRPS-severity, somatosensory and motor function) and MRI (T1, fMRI during MR, connectivity during rest) data. Our data of reaction time in patient group showed a bilateral MR delay compared to healthy controls (t(28) = 2.39; p = 0.02). There was no lateralization effect. As expected (Berneiser et al., 2017) for healthy controls fMRI demonstrated an active network from visual recognition (occipital cortex (OC)), parietal sulcus (PS), and primary motor cortex (M1). The same network was less expressed in activation magnitude in CRPS patients (pFWE corrected for ROI-analysis). Correlation analysis between clinical aspects, MRI data and connectivity measures (DCM: OCG-PS-M1) are still being evaluated.

Determinants of difficulty in activities of daily living in ambulatory patients undergoing hemodialysis

BackgroundPatients undergoing hemodialysis (HD) have difficulty performing activities of daily living (ADL) compared to healthy people. ADL difficulty is an early predictor of loss of independence and mortality in older community-living people. However, determinants of ADL difficulty in HD patients have not been clarified. This study aimed to identify factors associated with ADL difficulty in ambulatory HD patients.MethodsSubjects were 216 Japanese outpatients (130 men, 86 women; mean age, 67 years) undergoing maintenance HD three times a week. Clinical characteristics, depressive symptoms, motor function (leg strength, balance, and walking speed), and ADL difficulty related to lower-limb function such as mobility issues were compared across three difficulty levels (higher, middle, and lower) as classified according to the percentages of patients with perceived difficulty. Multivariate logistic regression analysis was performed to examine whether clinical characteristics, depressive symptoms, and motor function could discriminate ADL difficulty at each level. Receiver operating characteristic curve analysis was performed to determine cut-off values of motor function for predicting ADL difficulty at each level.ResultsADL difficulty was independently associated with age (odds ratio (OR) = 1.05, 95% confidence interval (CI) 1.00–1.10; P = 0.039), presence of depressive symptoms (OR = 4.24, 95%CI 1.13–15.95; P = 0.033), and usual walking speed (OR = 0.94, 95%CI 0.90–0.97; P < 0.001) for higher level difficulty; age (OR = 1.06, 95%CI 1.02–1.10; P = 0.006), maximum leg strength (OR = 0.97, 95%CI 0.94–1.00; P = 0.043), and usual walking speed (OR = 0.96, 95%CI 0.93–0.98; P = 0.001) for middle level difficulty; and age (OR = 1.06, 95%CI 1.02–1.10; P = 0.006) and usual walking speed (OR = 0.93, 95%CI 0.90–0.6; P < 0.001) for lower level difficulty. Cut-off values of usual walking speed for predicting ADL difficulty for higher, middle, and lower level difficulty were 83.7, 75.5, and 75.1 m/min, respectively.ConclusionsA slow walking speed and old age were significantly and independently associated with ADL difficulty in ambulatory HD patients. Presence of depressive symptoms was significantly and independently associated with ADL difficulty at the higher level of difficulty in ambulatory HD patients. These findings provide useful data for planning effective therapeutic regimens to prevent ADL difficulty in ambulatory HD patients.

Corticospinal tract diffusion properties and robotic visually guided reaching in children with hemiparetic cerebral palsy.

Perinatal stroke is the leading cause of hemiparetic cerebral palsy (CP), resulting in life-long disability. In this study, we examined the relationship between robotic upper extremity motor impairment and corticospinal tract (CST) diffusion properties. Thirty-three children with unilateral perinatal ischemic stroke (17 arterial, 16 venous) and hemiparesis were recruited from a population-based research cohort. Bilateral CSTs were defined using diffusion tensor imaging (DTI) and four diffusion metrics were quantified: fractional anisotropy (FA), mean (MD), radial (RD), and axial (AD) diffusivities. Participants completed a visually guided reaching task using the KINARM robot to define 10 movement parameters including movement time and maximum speed. Twenty-six typically developing children underwent the same evaluations. Partial correlations assessed the relationship between robotic reaching and CST diffusion parameters. All diffusion properties of the lesioned CST differed from controls in the arterial group, whereas only FA was reduced in the venous group. Non-lesioned CST diffusion measures were similar between stroke groups and controls. Both stroke groups demonstrated impaired reaching performance. Multiple reaching parameters of the affected limb correlated with lesioned CST diffusion properties. Lower FA and higher MD were associated with greater movement time. Few correlations were observed between non-lesioned CST diffusion and unaffected limb function though FA was associated with reaction time (R = -0.39, p < .01). Diffusion properties of the lesioned CST are altered after perinatal stroke, the degree of which correlates with specific elements of visually guided reaching performance, suggesting specific relevance of CST structural connectivity to clinical motor function in hemiparetic children.

Kinematic evaluation for impairment of skilled hand function in chemotherapy-induced peripheral neuropathy

IntroductionChemotherapy-induced peripheral neuropathy (CIPN) usually affects both sensory and motor function of hands and feet, resulting in impaired skilled hand function (e.g., typing a keyboard). However, quantitative and objective evaluations for this condition have not been established. Purpose of the StudyWe evaluated skilled hand function using a kinematic analysis and investigated relationships among hand kinematic function and the clinical sensory and motor features of CIPN. Study DesignsClinical measurement. MethodsTwelve CIPN patients and 12 age-matched control participants were enrolled. We recorded their reach and grasp movements using a three-dimensional measurement system, and calculated the normalized jerk of these movements as quantitative indexes of skilled hand function. Additionally, we used the number of sequential hand grip–release cycles in 10 seconds as an evaluation of clinical motor function. ResultsOur kinematic analyses revealed significant difference in normalized jerk of grasp movement (CIPN: 3.7 ± 0.2, control: 3.4 ± 0.1; P = .005), but this was not the case for reach movement (CIPN: 2.5 ± 0.1, control: 2.5 ± 0.2; P = .43), indicating that the distal part of the forearm is particularly affected in CIPN. Such disturbed grasp movement was directly correlated with poor scores on the hand grip–release test and the sensory tests. DiscussionWe revealed deficit impaired hand function objectively and quantitatively in CIPN patients using a kinematic analysis. Further, the hand grip test could represent such kinematic abnormality and could be useful for evaluating skilled hand function of CIPN patients. ConclusionsOur kinematic and clinical measurements objectively and quantitatively evaluate skilled hand function in individuals with CIPN in clinical settings. Level of EvidenceCross-sectional observational study.

Adipose Stromal Vascular Fraction-Mediated Improvements at Late-Stage Disease in a Murine Model of Multiple Sclerosis.

Multiple sclerosis (MS) is a common neurodegenerative disease and remains an unmet clinical challenge. In MS, an autoimmune response leads to immune cell infiltration, inflammation, demyelination, and lesions in central nervous system (CNS) tissues resulting in tremors, fatigue, and progressive loss of motor function. These pathologic hallmarks are effectively reproduced in the murine experimental autoimmune encephalomyelitis (EAE) model. The stromal vascular fraction (SVF) of adipose tissue is composed of adipose-derived stromal/stem cells (ASC), adipocytes, and various leukocytes. The SVF can be culture expanded to generate ASC lines. Clinical trials continue to demonstrate the safety and efficacy of ASC therapies for treating several diseases. However, little is known about the effectiveness of the SVF for neurodegenerative diseases, such as MS. At late-stage disease, EAE mice show severe motor impairment. The goal for these studies was to test the effectiveness of SVF cells and ASC in EAE mice after the onset of neuropathology. The clinical scoring, behavior, motor function, and histopathologic analyses revealed significant improvements in EAE mice treated with the SVF or ASC. Moreover, SVF treatment mediated more robust improvements to CNS pathology than ASC treatment based on significant modulations of inflammatory factors. The most pronounced changes following SVF treatment were the high levels of interleukin-10 in the peripheral blood, lymphoid and CNS tissues along with the induction of regulatory T cells in the lymph nodes which indicate potent immunomodulatory effects. The data indicate SVF cells effectively ameliorated the EAE immunopathogenesis and supports the potential use of SVF for treating MS. Stem Cells 2017;35:532-544.

Determinants of Slow Walking Speed in Ambulatory Patients Undergoing Maintenance Hemodialysis.

Walking ability is significantly lower in hemodialysis patients compared to healthy people. Decreased walking ability characterized by slow walking speed is associated with adverse clinical events, but determinants of decreased walking speed in hemodialysis patients are unknown. The purpose of this study was to identify factors associated with slow walking speed in ambulatory hemodialysis patients. Subjects were 122 outpatients (64 men, 58 women; mean age, 68 years) undergoing hemodialysis. Clinical characteristics including comorbidities, motor function (strength, flexibility, and balance), and maximum walking speed (MWS) were measured and compared across sex-specific tertiles of MWS. Univariate and multivariate logistic regression analyses were performed to examine whether clinical characteristics and motor function could discriminate between the lowest, middle, and highest tertiles of MWS. Significant and common factors that discriminated the lowest and highest tertiles of MWS from other categories were presence of cardiac disease (lowest: odds ratio [OR] = 3.33, 95% confidence interval [CI] = 1.26–8.83, P<0.05; highest: OR = 2.84, 95% CI = 1.18–6.84, P<0.05), leg strength (OR = 0.62, 95% CI = 0.40–0.95, P<0.05; OR = 0.57, 95% CI = 0.39–0.82, P<0.01), and standing balance (OR = 0.76, 95% CI = 0.63–0.92, P<0.01; OR = 0.81, 95% CI = 0.68–0.97, P<0.05). History of fracture (OR = 3.35, 95% CI = 1.08–10.38; P<0.05) was a significant factor only in the lowest tertile. Cardiac disease, history of fracture, decreased leg strength, and poor standing balance were independently associated with slow walking speed in ambulatory hemodialysis patients. These findings provide useful data for planning effective therapeutic regimens to prevent decreases in walking ability in ambulatory hemodialysis patients.

Multiple rodent models and behavioral measures reveal unexpected responses to FTY720 and DMF in experimental autoimmune encephalomyelitis

Experimental autoimmune encephalomyelitis (EAE) is a widely-used rodent model for multiple sclerosis (MS), but a single model can hardly capture all features of MS. We investigated whether behavioral parameters in addition to clinical motor function scores could be used to assess treatment efficacy during score-free intervals in the relapsing-remitting EAE model in SJL/J mice. We studied the effects of the clinical reference compounds FTY720 (fingolimod, 0.5mg/kg/day) and dimethyl fumarate (DMF, 20–30mg/kg/day) on clinical scores in several rodent EAE models in order to generate efficacy profiles. SJL/J mice with relapsing-remitting EAE were studied using behavioral tests, including rotarod, gait analysis, locomotor activity and grip strength. SJL/J mice were also examined according to Crawley’s sociability and preference for social novelty test. Prophylactic treatment with FTY720 prevented clinical scores in three of the four EAE rodent models: Dark Agouti (DA) and Lewis rats and C57BL/6J mice. Neither prophylactic nor late-therapeutic treatment with FTY720 reduced clinical scores or reversed deficits in the rotarod test in SJL/J mice, but we observed effects on motor functions and sociability in the absence of clinical scores. Prophylactic treatment with FTY720 improved the gait of SJL/J mice whereas late-therapeutic treatment improved manifestations of reduced social (re)cognition or preference for social novelty. DMF was tested in three EAE models and did not improve clinical scores at the dose used. These data indicate that improvements in behavioral deficits can occur in absence of clinical scores, which indicate subtle drug effects and may have translational value for human MS.

Improving activity recognition using a wearable barometric pressure sensor in mobility-impaired stroke patients.

BackgroundStroke survivors often suffer from mobility deficits. Current clinical evaluation methods, including questionnaires and motor function tests, cannot provide an objective measure of the patients’ mobility in daily life. Physical activity performance in daily-life can be assessed using unobtrusive monitoring, for example with a single sensor module fixed on the trunk. Existing approaches based on inertial sensors have limited performance, particularly in detecting transitions between different activities and postures, due to the inherent inter-patient variability of kinematic patterns. To overcome these limitations, one possibility is to use additional information from a barometric pressure (BP) sensor.MethodsOur study aims at integrating BP and inertial sensor data into an activity classifier in order to improve the activity (sitting, standing, walking, lying) recognition and the corresponding body elevation (during climbing stairs or when taking an elevator). Taking into account the trunk elevation changes during postural transitions (sit-to-stand, stand-to-sit), we devised an event-driven activity classifier based on fuzzy-logic.Data were acquired from 12 stroke patients with impaired mobility, using a trunk-worn inertial and BP sensor. Events, including walking and lying periods and potential postural transitions, were first extracted. These events were then fed into a double-stage hierarchical Fuzzy Inference System (H-FIS). The first stage processed the events to infer activities and the second stage improved activity recognition by applying behavioral constraints. Finally, the body elevation was estimated using a pattern-enhancing algorithm applied on BP. The patients were videotaped for reference. The performance of the algorithm was estimated using the Correct Classification Rate (CCR) and F-score. The BP-based classification approach was benchmarked against a previously-published fuzzy-logic classifier (FIS-IMU) and a conventional epoch-based classifier (EPOCH).ResultsThe algorithm performance for posture/activity detection, in terms of CCR was 90.4 %, with 3.3 % and 5.6 % improvements against FIS-IMU and EPOCH, respectively. The proposed classifier essentially benefits from a better recognition of standing activity (70.3 % versus 61.5 % [FIS-IMU] and 42.5 % [EPOCH]) with 98.2 % CCR for body elevation estimation.ConclusionThe monitoring and recognition of daily activities in mobility-impaired stoke patients can be significantly improved using a trunk-fixed sensor that integrates BP, inertial sensors, and an event-based activity classifier.

Quantitative assessment of non-motor fluctuations in Parkinson's disease using the Non-Motor Symptoms Scale (NMSS).

Data on frequency, severity and correlations of NMS with motor complications are only available for a limited number of NMS. The NMS Scale (NMSS) is a validated tool to assess a broad range of NMS, which has not been used in NMS fluctuations. We assessed fluctuations of a broad range of non-motor symptom (NMS) for a 1-month time period in fluctuating Parkinson's disease (PD) in a multicenter cross-sectional study using the NMSS assessing NMS in motor On (NMSSOn) and Off state (NMSSOff) combined with clinical NMS and motor function scoring in 100 fluctuating PD patients. ΔNMSSOn/Off was defined as the differences of NMSS scores between On and Off. Complete NMSS datasets were available from 73 patients (53 % men; age: 68.2 ± 9.7 years) with mean total NMSS score in On state of 41.5 ± 37.6 and in Off state of 75.6 ± 42.3 (P < 0.001). Scores were higher in Off compared to On state for all domains except for domain "perceptual problems/hallucinations" (P = 0.608). Clinimetric properties of the NMSS were similar to those reported previously for NMS assessments independent of motor oscillations. NMSSOn, NMSSOff and ΔNMSSOn/Off showed weak to moderate correlations with demographics, indicators of motor symptom severity as well as with other measures of NMS, depression and quality of life. Correlations of NMSS items/domains with independent measures of related constructs were weak to moderate. In conclusion, when assessed with the NMSS, a broad range of NMS fluctuate with motor oscillations, but these fluctuations do neither correlate with motor function nor with measures of disease progression.

Cerebral palsy in children in Kampala, Uganda: clinical subtypes, motor function and co-morbidities.

BackgroundCerebral palsy (CP) is a common chronic childhood disorder worldwide. There is limited information about the CP panorama in sub-Saharan Africa. Our aim was to describe the clinical subtypes, gross and fine motor functions and presence of co-morbidities in a group of children with CP attending a tertiary hospital in Uganda.MethodsChildren with CP in the age range of 2-12 years visiting the paediatric CP clinic at Mulago Hospital, Kampala, were enrolled. Screening and inclusion were based on a three-stage procedure: i) Two screening questions from the Ten Question Screen; ii) Clinical assessments adapted from the Surveillance for Cerebral Palsy in Europe (SCPE); iii) Clinical examinations and diagnoses of subtype, severity level and co-morbidities. Caregivers were interviewed using questionnaires to provide information on child’s medical history and co-morbidities. Co-morbidity scores were calculated for each child.ResultsOne hundred and thirty five children with CP were enrolled (72 males, 63 females, median age 3 years 5 months, IQR-2 years 4 months-5 years 6 months). Bilateral spastic type was commonest (45%); moderate impairment in gross motor function was present in 43%, with comparable numbers (37%) in the mild and severely impaired fine motor function groups. The severe gross and fine motor function levels were seen in the bilateral spastic and dyskinetic CP subtypes.Signs of learning disability (75%) and epilepsy (45%) were the commonest co-morbidities. Higher co-morbidity scores were obtained in children with dyskinetic CP and severe levels of gross and fine motor function. There was a significant difference in distribution of the co-morbidity scores between the CP subtypes, gross motor and fine motor function levels (p <0.001). Signs of speech and language impairments were associated with bilateral spastic CP and severe gross and fine motor dysfunction (p < 0.05).ConclusionsBilateral spastic CP was the main clinical subtype, with signs of learning disability and epilepsy as major causes of co-morbidity. The severity of gross and fine motor function levels was related to severity of clinical CP subtypes. Our findings imply a higher occurrence of birth asphyxia or post natally acquired infections. Improvement in emergency obstetric and postnatal care may reduce this burden.Electronic supplementary materialThe online version of this article (doi:10.1186/s13104-015-1125-9) contains supplementary material, which is available to authorized users.

Toward tract-specific fractional anisotropy (TSFA) at crossing-fiber regions with clinical diffusion MRI.

White matter fractional anisotropy (FA), a measure suggesting microstructure, is significantly underestimated with single diffusion tensor model at crossing-fiber regions (CFR). We propose a tract-specific FA (TSFA), corrected for the effects of crossing-fiber geometry and free water at CFR, and adapted for tract analysis with diffusion MRI (dMRI) in clinical research. At CFR voxels, the proposed technique estimates free water fraction (fiso ) as a linear function of mean apparent diffusion coefficient (mADC), fits the dual tensors and estimates TSFA. Digital phantoms were designed for testing the accuracy of fiso and fitted dual-anisotropies at CFR. The technique was applied to clinical dMRI of normal subjects and hereditary spastic paraplegia (HSP) patients to test the effectiveness of TSFA. Phantom simulation showed unbiased estimates of dual-tensor anisotropies at CFR and high accuracy of fiso as a linear function of mADC. TSFA at CFR was highly consistent to the single tensor FA at non-CFR within the same tract with normal human dMRI. Additional HSP imaging biomarkers with significant correlation to clinical motor function scores could be identified with TSFA. Results suggest the potential of the proposed technique in estimating unbiased TSFA at CFR and conducting tract analysis in clinical research.

T.P.7: Prediction equation to estimate 6-Minute-Walk-Distance from motor function tests in patients with Duchenne Muscular Dystrophy

6-Minute Walk Distance (6MWD) has been used as the primary outcome measure for clinical trials in ambulatory patients with Duchenne Muscular Dystrophy (DMD). Unlike the Gower's Maneuver (G), 10-m walk/run (W/R) and North Star Ambulatory Assessment (NSAA) tests, the 6-min Walk Test (6MWT) is not routinely performed in clinic. Prediction of 6MWD with clinical motor function tests would facilitate in-clinic pre-screening of DMD patients for clinical trials, reduce screening failure rates and costs for clinical trials. To predict 6MWD from G, W/R and NSAA data Retrospective review of motor screening data (6WMD, G, W/R and NSAA) for the IGF1, EIM, ataluren, drisapersen and DMD natural history studies at our center. Velocity (<i>V</i>) was calculated from the <i>W</i>/<i>R</i> test. Linear regression was use to fit the 6MWD vs the other variables separately. SAS version 9.3 was used for statistical analyses. Study population: <i>N</i>=107; mean age of subjects is 9.28±2.96years; all subjects are on corticosteroid treatment (CS); mean age of starting CS is 5.55±1.69years; mean duration of CS is 3.78 ±2.68years; mean 6MWD for this cohort is 399.4±78.2. There are significant correlations between the clinical motor function test results and 6MWD. The correlation coefficients are −0.54, −0.77, 0.76 and 0.75 for <i>G</i>, <i>W</i>/<i>R</i>, <i>V</i> and NSAA vs 6MWD respectively (all <i>p</i>< 0,0001). <i>W</i>/<i>R</i>, <i>V</i> and NSAA could be used independently to predict 6MWD: 6MWD=575–34∗<i>W</i>/<i>R</i> (R2=0.60); 6MWD=172 +107*V (R2=0.57) and 6MWD=208 +8*NSAA (R2=0.56). Using NSAA and either <i>W</i>/<i>R</i> (R2=0.62) or <i>V</i> (R2=0.61) in a two-variable regression improves the regression fit slightly. Overall, <i>G</i>, <i>V</i> and NSAA are highly correlated with 6MWD; with greater correlations with <i>W</i>/<i>R</i>, <i>V</i> and NSAA. The NSAA and 10m walk/run test may be useful tools in clinics to predict 6MWD, thereby enabling time and cost efficient pre-screening of clinic patients for ambulatory DMD trials.

Plasticity and Response to Action Observation

Background. Action observation has been suggested as a possible gateway to retraining arm motor function post stroke. However, it is unclear if the neuronal response to action observation is affected by stroke and if it changes during the course of recovery. Objective. To examine longitudinal changes in neuronal activity in a group of patients with subacute stroke when observing and executing a bimanual movement task. Methods. Eighteen patients were examined twice using 3-T functional magnetic resonance imaging; 1 to 2 weeks and 3 months post stroke symptom onset. Eighteen control participants were examined once. Image time series were analyzed (SPM8) and correlated with clinical motor function scores. Results. During action observation and execution, an overlap of neuronal activation was observed in the superior and inferior parietal lobe, precentral gyrus, insula, and inferior temporal gyrus in both control participants and patients (P < .05; false discovery rate corrected). The neuronal response in the observation task increased from 1 to 2 weeks to 3 months after stroke. Most activated clusters were observed in the inferior temporal gyrus, the thalamus and movement-related areas, such as the premotor, supplementary and motor cortex (BA4, BA6). Increased activation of cerebellum and premotor area correlated with improved arm motor function. Most patients had regained full movement ability. Conclusions. Plastic changes in neurons responding to action observation and action execution occurred in accordance with clinical recovery. The involvement of motor areas when observing actions early and later after stroke may constitute a possible access to the motor system.

Frameless stereotactic deep brain stimulation for Parkinson′s disease: A case report and technical note

Because deep brain stimulation (DBS) implantations and other stereotactic and functional surgical procedures require accurate, precise, and safe targeting of the brain structure, the technical aids for preoperative planning, intervention, and postoperative follow-up have become increasingly important. In this paper, we introduce a case of advanced Parkinson's disease with 10 years of medical control in which the patient received subthalamic nuclei (STN) DBS therapy through frameless surgery. A preliminary outcomes analysis is also provided. The STN DBS was implanted using a frameless stereotaxy protocol. After identifying the STN by microelectrode recording (MER), the DBS electrodes were implanted and connected to an implanted programmable generator. Programming started 1 month after the operation, and the patient was followed up on regularly and 12 months of post-STN DBS unified Parkinson's disease rating scale were recorded. After 12 months of follow-up, the patient who received the frameless surgery showed a significant improvement in clinical motor functions compared with his preoperative scores. The frameless system has the advantage of providing accuracy in postoperative lead position survey and target deviation measurements with comparison to the preoperative planning image. The outcomes of frameless DBS surgery are similar to those of frame-based surgery, with the advantages being that frameless surgery can reduce the patient's discomfort, shorten the operation and MER time, and decrease the MER trajectory number.

Hemispheric surgery for refractory epilepsy in children and adolescents: Outcome regarding seizures, motor skills and adaptive function

PurposeThe aim of the study was to report the seizure outcome, motor skills and adaptive motor functions in a series of children and adolescents who underwent hemispheric surgery, analysing the risk-benefits of surgery. MethodsThe clinical course, seizure and motor function outcomes of 15 patients who underwent hemispheric surgery were reviewed. ResultsThe mean age at surgery was 9.5, with 1–9 years follow-up. The underlying pathologies were Rasmussen encephalitis, vascular disorders, and hemimegalencephaly. All the patients presented with severe epilepsy and different degrees of hemiparesis, although motor functionality was preserved in 80% of the patients. At last follow-up, 67% were seizure free, and 20% rarely experienced seizures. Antiepileptic drugs were reduced in 60%, and complete withdrawal from such drugs was successful in 20% of the patients. The motor outcome following the surgery varied between the patients.Despite the motor deficit after surgery, the post-operative motor function showed unchanged for gross motor function in most (60%), while 27% improved. Similar results were obtained for the ability to handle objects in daily life activities. Sixty percent of the children were capable of handling objects, with somewhat reduced coordination and/or motor speed. ConclusionPre-surgical motor function continues to play a role in the pre-surgical evaluation process in order to provide a baseline for outcome. Hemispheric surgery, once regarded as a radical intervention and last treatment resource, may become routinely indicated for refractory hemispheric epilepsy in children and adolescents, with oftentime favourable motor outcomes.

Probable rapid eye movement sleep behavior disorder, nocturnal disturbances and quality of life in patients with Parkinson’s disease: a case-controlled study using the rapid eye movement sleep behavior disorder screening questionnaire

BackgroundIncreasing evidence provides a clear association between rapid eye movement sleep behavior disorders (RBD) and Parkinson’s disease (PD), but the clinical features that determine the co-morbidity of RBD and PD are not yet fully understood.MethodsWe evaluated the characteristics of nocturnal disturbances and other motor and non-motor features related to RBD in patients with PD and the impact of RBD on their quality of life. Probable RBD (pRBD) was evaluated using the Japanese version of the RBD screening questionnaire (RBDSQ-J).ResultsA significantly higher frequency of pRBD was observed in PD patients than in the controls (RBDSQ-J ≥ 5 or ≥ 6: 29.0% vs. 8.6%; 17.2% vs. 2.2%, respectively). After excluding restless legs syndrome and snorers in the PD patients, the pRBD group (RBDSQ-J≥5) showed higher scores compared with the non-pRBD group on the Parkinson’s disease sleep scale-2 (PDSS-2) total and three-domain scores. Early morning dystonia was more frequent in the pRBD group. The Parkinson’s Disease Questionnaire (PDQ-39) domain scores for cognition and emotional well-being were higher in the patients with pRBD than in the patients without pRBD. There were no differences between these two groups with respect to the clinical subtype, disease severity or motor function. When using a cut-off of RBDSQ-J = 6, a similar trend was observed for the PDSS-2 and PDQ-39 scores. Patients with PD and pRBD had frequent sleep onset insomnia, distressing dreams and hallucinations. The stepwise linear regression analysis showed that the PDSS-2 domain “motor symptoms at night”, particularly the PDSS sub-item 6 “distressing dreams”, was the only predictor of RBDSQ-J in PD.ConclusionOur results indicate a significant impact of RBD co-morbidity on night-time disturbances and quality of life in PD, particularly on cognition and emotional well-being. RBDSQ may be a useful tool for not only screening RBD in PD patients but also predicting diffuse and complex clinical PD phenotypes associated with RBD, cognitive impairment and hallucinations.

Neurophysiological Correlates of Motor and Working Memory Performance following Subthalamic Nucleus Stimulation

Subthalamic nucleus (STN) deep brain stimulation (DBS) has become an accepted treatment for the motor manifestations of Parkinson disease (PD). The beneficial motor effects of STN DBS are likely due to modulation of BG output to frontal cortical regions associated with motor control, but the underlying neurophysiology of STN DBS effects, especially at the level of the cortex, is not well understood. In this study, we examined the effects of STN DBS on motor disability and visual working memory, a cognitive process supported by pFC. We tested 10 PD participants off medications, ON and OFF stimulation, along with 20 normal controls on a visual working memory task while simultaneously recording cortical EEG. In the OFF state, PD patients had poor motor function, were slower and less accurate in performing the working memory task, and had greater amplitudes and shorter latencies of the N200 ERP response. DBS improved clinical motor function, reduced N200 amplitudes, and increased N200 latencies but had little effect on working memory performance. We conclude that STN DBS normalizes neurophysiological activity in fronto striatal circuits and this may independently affect motor and cognitive function.

Does intraoperative neurophysiological monitoring have predictive value for functional recovery following spinal cord injury? A case report

To present a case report of a patient with an ASIA B spinal cord injury with partially intact baseline IONM who made a complete functional recovery postoperatively. A thirty-three year old male presented after a motor vehicle accident. Imaging studies revealed a C4-C5 bilateral facet dislocation. On presentation the patient had 4/5 strength in bilateral biceps and wrist extensors, 3/5 strength in bilateral triceps, and 0/5 strength in the finger flexors, intrinsics and all lower extremity muscles. Motor level was C7. Sensation was grossly intact to light touch throughout all extremities, intact to pinprick from C2 to T7, and absent to pinprick caudal to T7. Rectal tone and contraction were absent. After attempts at closed reduction failed the patient underwent an open reduction and posterior C4-C5 fusion. Intraoperative neurophysiologic monitoring (IONM) revealed the presence of baseline responses to the posterior tibial nerve using somatosensory evoked potentials and to the right abductor hallucis using transcranial motor evoked potentials. At the 6 weeks postoperative visit the patient had full 5/5 motor strength to all muscles except the left deltoid that was 4/5 due to a rotator cuff injury. This case illustrates a potential prognostic value of IONM. Despite lack of clinical motor function at the time of surgery, IONM was able to illicit a motor response in the right lower extremity. Further prospective studies are needed for further investigation.