The novel Next Step test is a reliable measure of anticipatory postural adjustments made by children with cerebral palsy prior to taking a step.

BACKGROUND: Children with cerebral palsy (CP) make smaller medio-lateral anticipatory postural adjustments (APAs) than typically developing peers when stepping forward to a medial target. They are also less accurate at reaching the stepping target. The Next Step test involves the biomechanical measurement of APAs and foot placement error. These may be useful outcome measures to evaluate dynamic balance in a clinical trial. The reliability of the measures must be assessed to establish their reliability as research tools. RESEARCH QUESTION: What is the inter-rater and intra-rater reliability of stepping accuracy and measures of APAs made by children prior to taking a step? METHODS: Typically developing (TD) (n = 14) or children with CP (n = 16) were recruited from local clinics. Children stepped to electro-luminescent targets placed medially and laterally to each foot. Stepping responses were measured using a force plate and 3D motion analysis of markers placed on the feet and pelvis. The APA was defined as the movement of the centre of pressure (COP) and the centre of mass (COM) estimated via pelvic markers, prior to lifting the lead leg. Stepping accuracy was defined as the absolute distance between the target and end foot position. Participants undertook two data collection sessions separated by at least one week. In session one, the test was measured by rater 1 who repeated this in session two, along with another data collection by a rater 2 or rater 3, after a rest period. Where data were normally distributed, they were assessed for inter-rater and intra-rater reliability using an intra-class correlation coefficient (ICC) and Bland-Altman plots. The standard error of measurement was calculated to determine the minimum difference needed to detect true change. RESULTS: There was no between-group differences in group characteristics (age, weight, height) or in stepping velocity. We found good to excellent reliability when measuring the amplitude and velocity of medio-lateral APAs (ICC range 0.73-0.89). The reliability of antero-posterior APAs was more variable (ICC range 0.08-0.92). The minimum difference to detect a true change for peak medio-lateral motion of COP ranges from 23.7 mm to 29.6 mm and for peak velocity of medio-lateral COM estimate 41-61.9 mm. Stepping accuracy was not normally distributed. SIGNIFICANCE: The Next Step test is a reliable measure of dynamic balance. The peak medio-lateral motion of the COP and medio-lateral velocity of the COM estimate are reliable when measured during a constrained stepping task in ambulant children with cerebral palsy.

A cross sectional study investigating dynamic balance when stepping to targets in children with cerebral palsy compared to typically developing children.

BACKGROUND: Children with Cerebral Palsy (CP) have altered anticipatory postural adjustments (APAs) during gait initiation. These APAs may affect dynamic balance in tasks such as stepping. RESEARCH QUESTIONS: How are APAs in children with CP affected during stepping to precise targets? How do children with CP modulate APAs when stepping to medial and lateral targets? What is the association between APAs and symptom severity, movement quality and impairment profile? METHOD: Children undertook a stepping task to laterally and medially placed targets with either leg, in a randomised order. Movement of the centre of pressure (COP) and markers at the pelvis and foot were measured via a force plate and 3D motion analysis. Motion of the centre of mass (COM) was estimated via pelvic markers. APAs were assessed prior to leading leg lift-off in medio-lateral and antero-posterior directions. Stepping error was calculated. Baseline characteristics of children with CP included Gross Motor Function Measure (GMFM), Quality Function Measure (QFM), leg muscle hypertonia (Tardieu test) and strength (manual dynamometry). RESULTS: Sixteen ambulant children with CP (12.2 years ± 2.2) and 14 typically developing (TD) children (11.6 years ± 2.9) were assessed. In children with CP, APAs in the medio-lateral direction were 20-30% smaller. Children with CP were less able to modulate their APAs with steps to medial and laterally placed targets, than TD children. Medio-lateral COP motion was associated with movement quality assessed by QFM subsections, GMFM (correlation coefficient r = 0.66-0.80) and hip abductor strength (r = 0.75). Antero-posterior APAs were significantly smaller when stepping with the non-paretic leg in children with CP. APA size was positively related to the length of the contralateral, paretic gastrocnemius (r = 0.77). Stepping error was higher in children with CP and inversely correlated to the size of the medio-lateral APA. DISCUSSION: Children with CP show smaller medio-lateral APAs especially when stepping to medially placed targets. APA size may be limited by proximal muscle strength and gastrocnemius length.

Exploratory Randomized Double-Blind Placebo-Controlled Trial of Botulinum Therapy on Grasp Release After Stroke (PrOMBiS).

Background. OnabotulinumtoxinA injections improve upper-limb spasticity after stroke, but their effect on arm function remains uncertain. Objective. To determine whether a single treatment with onabotulinumtoxinA injections combined with upper-limb physiotherapy improves grasp release compared with physiotherapy alone after stroke. Methods. A total of 28 patients, at least 1 month poststroke, were randomized to receive either onabotulinumtoxinA or placebo injections to the affected upper limb followed by standardized upper-limb physiotherapy (10 sessions over 4 weeks). The primary outcome was time to release grasp during a functionally relevant standardized task. Secondary outcomes included measures of wrist and finger spasticity and strength using a customized servomotor, clinical assessments of stiffness (modified Ashworth Scale), arm function (Action Research Arm Test [ARAT], Nine Hole Peg Test), arm use (Arm Measure of Activity), Goal Attainment Scale, and quality of life (EQ5D). Results. There was no significant difference between treatment groups in grasp release time 5 weeks post injection (placebo median = 3.0 s, treatment median = 2.0 s; t(24) = 1.20; P = .24; treatment effect = -0.44, 95% CI = -1.19 to 0.31). None of the secondary measures passed significance after correcting for multiple comparisons. Both groups achieved their treatment goals (placebo = 65%; treatment = 71%), and made improvements on the ARAT (placebo +3, treatment +5) and in active wrist extension (placebo +9°, treatment +11°). Conclusions. In this group of stroke patients with mild to moderate spastic hemiparesis, a single treatment with onabotulinumtoxinA did not augment the improvements seen in grasp release time after a standardized upper-limb physiotherapy program.

Cerebellar ataxia.

The cerebellum plays an integral role in the control of limb and ocular movements, balance, and walking. Cerebellar disorders may be classified as sporadic or hereditary with clinical presentation varying with the extent and site of cerebellar damage and extracerebellar signs. Deficits in balance and walking reflect the cerebellum's proposed role in coordination, sensory integration, coordinate transformation, motor learning, and adaptation. Cerebellar dysfunction results in increased postural sway, hypermetric postural responses to perturbations and optokinetic stimuli, and postural responses that are poorly coordinated with volitional movement. Gait variability is characteristic and may arise from a combination of balance impairments, interlimb incoordination, and incoordination between postural activity and leg movement. Intrinsic problems with balance lead to a high prevalence of injurious falls. Evidence for pharmacologic management is limited, although aminopyridines reduce attacks in episodic ataxias and may have a role in improving gait ataxia in other conditions. Intensive exercises targeting balance and coordination lead to improvements in balance and walking but require ongoing training to maintain/maximize any effects. Noninvasive brain stimulation of the cerebellum may become a useful adjunct to therapy in the future. Walking aids, orthoses, specialized footwear and seating may be required for more severe cases of cerebellar ataxia.

Effects of superficial heating and insulation on walking speed in people with hereditary and spontaneous spastic paraparesis: A randomised crossover study.

OBJECTIVES: Cooling of the lower limb in people with Hereditary and Spontaneous Spastic Paraparesis (pwHSSP) has been shown to affect walking speed and neuromuscular impairments. The investigation of practical strategies, which may help to alleviate these problems is important. The potential of superficial heat to improve walking speed has not been explored in pwHSSP. Primary objective was to explore whether the application of superficial heat (hot packs) to lower limbs in pwHSSP improves walking speed. Secondary objective was to explore whether wearing insulation after heating would prolong any benefits. METHODS: A randomised crossover study design with 21 pwHSSP. On two separate occasions two hot packs and an insulating wrap (Neo-G™) were applied for 30minutes to the lower limbs of pwHSSP. On one occasion the insulating wrap was maintained for a further 30minutes and on the other occasion it was removed. Measures of temperature (skin, room and core), walking speed (10 metre timed walk) and co-ordination (foot tap time) were taken at baseline (T1), after 30 mins (T2) and at one hour (T3). RESULTS: All 21 pwHSSP reported increased lower limb stiffness and decreased walking ability when their legs were cold. After thirty minutes of heating, improvements were seen in walking speed (12.2%, P<0.0001, effect size 0.18) and foot tap time (21.5%, P<0.0001, effect size 0.59). Continuing to wear insulation for a further 30minutes gave no additional benefit; with significant improvements in walking speed maintained at one hour (9.9%, P>0.001) in both conditions. CONCLUSIONS: Application of 30minutes superficial heating moderately improved walking speed in pwHSSP with effects maintained at 1hour. The use of hot packs applied to lower limbs should be the focus of further research for the clinical management of pwHSSP who report increased stiffness of limbs in cold weather and do not have sensory deficits.

Humeral elevation reduces the dynamic control ratio of the shoulder muscles during internal rotation.

OBJECTIVES: To determine the differences in the dynamic control ratio of the glenohumeral joint rotators, during internal rotation at 20° and 60° of humeral elevation in the scapular plan. Dynamic control ratio (DCR) is defined as the ratio between eccentric action of the lateral rotators and the concentric action of the medial rotators. DESIGN: A cross-sectional laboratory study. METHODS: Thirty asymptomatic participants (men n=14, women n=16, mean age=29.4±8.9years, BMI: 24.1±5.4) were tested. Peak torque generated by the concentric action of the MR and the eccentric action of the LR of the shoulder joint and the DCR were evaluated on the dominant arm using an isokinetic dynamometer at 20° and 60° of humeral elevation at a speed of 20°/s. RESULTS: There was a significant decrease in the DCR at 60° humeral elevation when compared to 20° humeral elevation (p<0.05). This decrease was due to the significant decrease in eccentric peak torques at 60° humeral elevation when compared to 20° (p<0.05). However, there was no significant difference in the concentric peak torques between 20° and 60° (p>0.05). CONCLUSIONS: The significant decrease in the DCR as a consequence of a decrease in the eccentric peak torque of the LR when the humerus is in a more elevated position suggests that the introduction of humeral elevation can be used as a progression for improving the eccentric action of the shoulder LR and subsequently the dynamic control of the shoulder.

Sensorimotor processing for balance in spinocerebellar ataxia type 6.

BACKGROUND: We investigated whether balance impairments caused by cerebellar disease are associated with specific sensorimotor processing deficits that generalize across all sensory modalities. Experiments focused on the putative cerebellar functions of scaling and coordinate transformation of balance responses evoked by stimulation of single sensory channels. METHODS: Vestibular, visual, and proprioceptive sensory channels were stimulated in isolation using galvanic vestibular stimulation, moving visual scenery, and muscle vibration, respectively, in 16 subjects with spinocerebellar ataxia type 6 (SCA6) and 16 matched healthy controls. Two polarities of each stimulus type evoked postural responses of similar form in the forward and backward directions. Disease severity was assessed using the Scale for Assessment and Rating of Ataxia. RESULTS: Impaired balance of SCA6 subjects during unperturbed stance was reflected in faster than normal body sway (P = 0.009), which correlated with disease severity (r = 0.705, P < 0.001). Sensory perturbations revealed a sensorimotor processing abnormality that was specific to response scaling for the visual channel. This manifested as visually evoked postural responses that were approximately three times larger than normal (backward, P < 0.001; forward P = 0.005) and correlated with disease severity (r = 0.543, P = 0.03). Response direction and habituation properties were no different from controls for all three sensory modalities. CONCLUSION: Cerebellar degeneration disturbs the scaling of postural responses evoked by visual motion, possibly through disinhibition of extracerebellar visuomotor centers. The excessively high gain of the visuomotor channel without compensatory decreases in gains of other sensorimotor channels provides a potential mechanism for instability of the balance control system in cerebellar disease.

Training balance with opto-kinetic stimuli in the home: a randomized controlled feasibility study in people with pure cerebellar disease.

OBJECTIVE: To investigate the feasibility of a randomized controlled trial of a home-based balance intervention for people with cerebellar ataxia. DESIGN: A randomized controlled trial design. SETTING: Intervention and assessment took place in the home environment. PARTICIPANTS: A total of 12 people with spinocerebellar ataxia type 6 were randomized into a therapy or control group. Both groups received identical assessments at baseline, four and eight weeks. INTERVENTIONS: Therapy group participants undertook balance exercises in front of optokinetic stimuli during weeks 4-8, while control group participants received no intervention. MAIN MEASURES: Test-retest reliability was analysed from outcome measures collected twice at baseline and four weeks later. Feasibility issues were evaluated using daily diaries and end trial exit interviews. RESULTS: The home-based training intervention with opto-kinetic stimuli was feasible for people with pure ataxia, with one drop-out. Test-retest reliability is strong (intraclass correlation coefficient >0.7) for selected outcome measures evaluating balance at impairment and activity levels. Some measures reveal trends towards improvement for those in the therapy group. Sample size estimations indicate that Bal-SARA scores could detect a clinically significant change of 0.8 points in this functional balance score if 80 people per group were analysed in future trials. CONCLUSIONS: Home-based targeted training of functional balance for people with pure cerebellar ataxia is feasible and the outcome measures employed are reliable.

Stance instability in spinocerebellar ataxia type 6.

Balance impairment is a principal symptom of cerebellar disease, but is poorly understood partly because subjects with heterogenous cerebellar and extracerebellar lesions have often been studied. Spinocerebellar ataxia type 6 (SCA6) provides an opportunity to understand balance dysfunction associated with a relatively homogenous cerebellar lesion. This study investigated stance instability in SCA6 and how it is affected by varying stance width. Body sway, as well as its directional preponderance and distribution across joints, was measured three-dimensionally in 17 SCA6 and 17 matched healthy control subjects. Subjects stood for 40 seconds on a stable surface with their eyes open and feet positioned at various stance widths (32, 16, 8, 4, and 0 cm). SCA6 subjects swayed faster than controls at every stance width. Decreasing the stance width produced a disproportionate increase in sway speed in SCA6 subjects, compared to controls. Directional preponderance of sway was dependent on stance width, but did not differ between groups. Joint instability was increased by reducing stance width in both groups, but there was greater instability of the ankle joint in the roll plane in the SCA6 group. Measures of global instability correlated strongly with disease severity measured with the Scale for the Assessment and Rating of Ataxia (r = 0.79). The sway characteristics suggest a disruption of sensorimotor processing for balance control in SCA6. The correlation with disease severity implies that balance impairment is a feature of progression of SCA6 clinical syndrome. With stance width standardized, the instability measures employed could provide sensitive, continuous outcome measures of longitudinal or therapeutic change.

Identification of risk factors for falls in multiple sclerosis: a systematic review and meta-analysis.

BACKGROUND: Falls are a significant issue in people with multiple sclerosis (MS), with research demonstrating fall rates of more than 50%. PURPOSE: The purpose of this study was to evaluate the risk factors associated with falling in people with MS. DATA SOURCES: Mixed search methods were used, including computer-based and manual searches. Additionally, hand searches of reference lists and conference abstracts were performed. All literature published from the source's earliest date to January 2012 was included; only full-text English-language sources (or those where a translation was available) were included. STUDY SELECTION: Eligibility criteria specified articles evaluating any aspect of fall risk in adults with a confirmed MS diagnosis, where the incidence of falling as determined by prospective or retrospective participant report was included. DATA EXTRACTION: Data were extracted independently by 2 reviewers using a written protocol and standardized extraction documentation. Detailed assessment of each article was independently undertaken by both reviewers, including assessment of study quality using an adaptation of the Newcastle Ottawa Scale plus extraction of key data (participant characteristics, fall incidence, and outcomes). DATA SYNTHESIS: The final review comprised 8 articles with a total of 1,929 participants; 1,037 (53.75%) were classified as fallers. Eighteen different risk factors were assessed within the included studies. Meta-analysis demonstrated an increase in fall risk associated with impairments of balance and cognition, progressive MS, and use of a mobility aid. Narrative review of the qualitative articles and those factors where meta-analysis was not possible also was undertaken. LIMITATIONS: Variation in assessment, analysis, and reporting methods allowed meta-analysis for only 4 factors. CONCLUSION: There is limited evidence of the factors associated with fall risk in people with MS. Further methodologically robust studies are needed.

Exploring the experience of fatigue in people with Charcot-Marie-Tooth disease.

This qualitative study explored the phenomenon of fatigue for people with Charcot-Marie-Tooth disease, while acknowledging the triggers, impact and strategies people have developed to manage this symptom in daily life. A phenomenological approach was used to explore the experience of fatigue. Participants were recruited from a support group. Twenty-five people participated in three focus groups. Group interviews were tape recorded and independently transcribed. Transcripts were coded and emerging themes were highlighted. Four areas were explored and themes identified within them. Fatigue descriptions: energy depletion, overwhelming nature, variations in fatigue and fatigue as an abnormal experience; triggers of fatigue: activity, stress and mental concentration; impact of fatigue on: physical abilities, mood, guilt and frustration, isolation and concerns about how they were perceived by others; strategies to manage fatigue: coping with symptoms; planning ahead; deception; and reducing symptoms. This study revealed that fatigue is an overwhelming experience for people with Charcot-Marie-Tooth disease that impacts on many areas of their lives. People may require significant adjustment that could be facilitated by fatigue management approaches. In addition, exercise may have potential to reduce the symptoms of fatigue in some people.

Foot drop splints improve proximal as well as distal leg control during gait in Charcot-Marie-Tooth disease.

INTRODUCTION: During walking, people with Charcot-Marie-Tooth (CMT) disease may compensate for distal weakness by using proximal muscles. We investigated the effect of different AFOs on distal leg control and proximal compensatory actions. METHODS: Fourteen people with CMT were tested while wearing 3 types of ankle-foot orthosis (AFO) bilaterally compared with shoes alone. Walking was assessed using three-dimensional gait analysis. Stiffness of the splints was measured by applying controlled 5-degree ankle stretches using a motor. RESULTS: The results showed that each AFO significantly stiffened the ankle and increased ankle dorsiflexion at foot clearance compared with shoes alone. At push off, peak ankle power generation was reduced, but only with 1 type of AFO. A significant decrease in hip flexion amplitude during the swing phase was observed with all 3 AFOs. CONCLUSIONS: These results indicate that AFOs reduce foot drop and remove the need for some proximal compensatory action.

Theta burst stimulation in the rehabilitation of the upper limb: a semirandomized, placebo-controlled trial in chronic stroke patients.

BACKGROUND: Noninvasive cortical stimulation could represent an add-on treatment to enhance motor recovery after stroke. However, its clinical value, including anticipated size and duration of the treatment effects, remains largely unknown. OBJECTIVE: The authors designed a small semi-randomized clinical trial to explore whether long-lasting clinically important gains can be achieved by adding theta burst stimulation (TBS), a form of repetitive transcranial magnetic stimulation (TMS), to a rehabilitation program for the hand. METHODS: A total of 41 chronic stroke patients received excitatory TBS to the ipsilesional hemisphere or inhibitory TBS to the contralesional hemisphere in 2 centers; each active group was compared with a group receiving sham TBS. TBS was followed by physical therapy for 10 working days. Patients and therapists were blinded to the type of TBS. Primary outcome measures (9-hole Peg Test [9HPT], Jebsen Taylor Test [JTT], and grip and pinch-grip dynamometry) were assessed 4, 30, and 90 days post treatment. The clinically important difference was defined as 10% of the maximum score. RESULTS: There were no differences between the active treatment and sham groups in any of the outcome measures. All patients achieved small sustainable improvements--9HPT, 5% of maximum (confidence interval [CI] = 3%-7%); JTT, 5.7% (CI = 3%-8%); and grip strength, 6% (CI = 2%-10%)--all below the defined clinically important level. CONCLUSIONS: Cortical stimulation did not augment the gains from a late rehabilitation program. The effect size anticipated by the authors was overestimated. These results can improve the design of future work on therapeutic uses of TMS.

Comparing gait performance of people with Charcot-Marie-Tooth disease who do and do not wear ankle foot orthoses.

BACKGROUND AND PURPOSE: Ankle foot orthoses (AFOs) are commonly prescribed for people with Charcot-Marie-Tooth (CMT) disease. Scant evidence exists to guide the type and timing of orthotic prescription. This study explores the latter issue by investigating the differences in presentation and gait function of people with CMT disease who wore AFOs for daily mobility (n = 11) and a group who did not (n = 21). The aim was to see if there was a difference in the characteristics in people who regularly wear AFOs. METHODS: Primary measures of gait function were a 10-m timed walk (comfortable and maximum speed) and a 6-minute walk test. Means of the variables were compared using independent t-tests. Secondary measures included disease severity, lower limb muscle strength, sensory impairment, walking effort, fatigue severity and perceived walking ability. RESULTS: AFO wearers walked slower with higher effort. They also had greater disease severity, weaker leg muscles and perceived greater walking difficulty. Subjects not wearing AFOs showed significant relationships between gait variables and muscle strength, whereas AFO wearers showed significant relationships between gait variables and perceived walking ability, fatigue severity and effort. CONCLUSIONS: People who regularly wore AFOs were more severely affected, had a slower maximum walking speed, higher energy cost of walking and worse perceived walking ability. Walking ability in this group was related to fatigue, perceived exertion during walking and perceived walking ability. Gait function of people not using AFOs was determined by lower limb muscle function. People prescribed AFOs, those who do not wear them and those not prescribed AFOs were similar in presentation, suggesting that people choose to wear orthoses when their condition becomes sufficiently severe.

Non-linear vector summation of left and right vestibular signals for human balance.

The left and right vestibular organs always transduce the same signal of head movement, and with natural stimuli can only be activated simultaneously. To investigate how signals from the left and right vestibular organs are integrated to control human balance we electrically modulated the firing of vestibular afferents from each labyrinth independently and measured the resulting balance responses. Stimulation of one side at a time (monaural) showed that individual leg muscles receive equal inputs from the two labyrinths even though a single labyrinth appeared capable of signalling 3-D head motion. To deduce principles of left-right integration, balance responses to simultaneous stimulation of both sides (binaural) were compared with responses to monaural stimuli. The binaural whole-body response direction was compatible with vector summation of the left and right monaural responses. The binaural response magnitude, however, was only 64-74% that predicted by the monaural sum. This probably reflects a central non-linearity between vestibular input and motor output because stimulation of just one labyrinth revealed a power law relationship between stimulus current and response size with exponents 0.56 (force) and 0.51 (displacement). Thus, doubling total signal magnitude either by doubling monaural current or by binaural stimulation produced equivalent responses. We conclude that both labyrinths provide independent estimates of head motion that are summed vectorially and transformed non-linearly into motor output. The former process improves signal-to-noise and reduces artifactual common-mode changes, while the latter enhances responses to small signals, all critical for detecting the small head movements needed to control human balance.

Hip flexor fatigue limits walking in Charcot-Marie-Tooth disease.

Charcot-Marie-Tooth (CMT) disease results in distal lower limb weakness that affects walking. In this study we assess the role of the hip flexors in compensating for distal weakness while walking and the effects of prolonged walking on these putative compensatory strategies. Eighteen subjects with CMT disease were compared with 14 matched controls while they walked on a treadmill to a predetermined point of perceived effort. A significant reduction was observed in peak hip flexor velocity during walking and hip flexor maximal voluntary contraction. In a second session following selective fatigue of the hip flexors, hip flexor velocity decreased immediately on walking, and walking duration was greatly reduced. This study suggests that hip flexors compensate for distal weakness and that fatigue in the hip flexors can limit walking duration. Treatments directed toward improving proximal muscle strength may therefore help to delay onset of hip flexor fatigue and thus prolong walking duration.

Spinally generated electromyographic oscillations and spasms in a low-thoracic complete paraplegic.

We have measured some oscillatory properties of severe lower limb spasms experienced by a low-thoracic complete paraplegic during assisted standing. Electromyograms (EMG) were recorded from the leg muscles while the patient stood passively in a standing frame. The patient also stood using functional electrical stimulation (FES) while ground and handle reaction force vectors were measured together with EMG activity. During passive standing, spasms appeared simultaneously in all leg muscle groups on one side. The interval between spasms varied between 3 and 30 seconds. Within the spasms, there was a tendency of repetitive grouped discharge of motor units as well as a strong 10-Hz component in the EMG that was coherent across ipsilateral muscle groups. Thus, the spasms were inherently oscillatory. During FES-assisted standing, clinically similar spasms were observed. However, the interspasm interval became relatively fixed at around 16 seconds, which may indicate entraining of the spasm cycle by FES. There are similarities between this patient's spasms and the pathological motor activities seen in other movement disorders that may also be of spinal origin.