Quadriceps strength is a sensitive marker of disease progression in sporadic inclusion body myositis

There are currently no effective treatments to restore the muscle function in sporadic inclusion body myositis. Natural history studies of this disease are scarce. The goal of this study consisted in defining the functional pattern of patients with sporadic inclusion body myositis and to follow its change over a 9-month period to determine the most sensitive outcome measures for future clinical trials. Twenty-two patients with definite sporadic inclusion body myositis were assessed using clinical and functional scales. Dynamometry was used to evaluate the strength for hand grip and wrist, elbow, ankle and knee flexion and extension. Among the patients, 16 were reassessed 9 months later. The mean whole composite index was at 43.3 ± 16.5% of the predicted normal values. The weakest muscle functions were hand grip, wrist flexion and elbow flexion at the upper limbs and knee extension and ankle flexion at the lower limbs. Muscle weakness was generally asymmetrical, especially for upper limbs where all tested functions were significantly stronger at the dominant side. The patient strength was correlated with the disease duration only for knee extension, which was also the only muscle function to change significantly over 9 months. Knee extension strength seems to be the most relevant marker of disease progression in sporadic inclusion body myositis when measured with suitable dynamometry.     

Tonic stretch reflex threshold as a measure of spasticity after stroke: Reliability,minimal detectable change and responsiveness

ObjectiveTo determine inter-rater reliability, minimal detectable change and responsiveness of Tonic Stretch Reflex Threshold (TSRT) as a quantitative measure of elbow flexor spasticity.MethodsElbow flexor spasticity was assessed in 55 patients with sub-acute stroke by determining TSRT, the angle of spasticity onset at rest (velocity = 0°/s). Elbow flexor muscles were stretched 20 times at different velocities. Dynamic stretch-reflex thresholds, the elbow angles corresponding to the onset of elbow flexor EMG at each velocity, were used for TSRT calculation. Spasticity was also measured with the Modified Ashworth Scale (MAS). In a sub-group of 44 subjects, TSRT and MAS were measured before and after two weeks of an upper-limb intervention.ResultsThe intraclass correlation coefficient was 0.65 and the 95% minimal detectable change was 32.4°. In the treated sub-group, TSRT, but not MAS significantly changed. TSRT effect size and standardized response mean were 0.40 and 0.35, respectively. Detection of clinically meaningful improvements in upper-limb motor impairment by TSRT change scores ranged from poor to excellent.ConclusionsEvaluation of stroke-related elbow flexor spasticity by TSRT has good inter-rater reliability. Test responsiveness is low, but better than that of the MAS.SignificanceTSRT may be used to complement current scales of spasticity quantification.     

Strength deficits in primary focal hand dystonia.

Cortical activation is reduced when patients with focal dystonia perform movements that do not induce dystonic posturing. This finding suggests that the cortical drive to muscles may in some circumstances actually be reduced not increased, as suggested by basal ganglia models of dystonia as a hyperkinetic disorder. The purpose of this study was to examine flexor and extensor strength at the wrist (a clinically affected joint) and elbow (a nonclinically affected joint) in 18 patients with primary focal hand dystonia compared to matched control subjects. We measured peak torque from maximum voluntary contractions, and agonist and antagonist muscle activation by means of surface electromyograms. Patients were significantly weaker than controls at both the elbow and wrist joints and in both flexors and extensors compared to controls. Peak elbow flexion torque was, on average, 14.4% lower in the dystonic compared to the control group, elbow extensor peak torque was 28.6% lower, wrist flexor peak torque was 17.4% lower, and wrist extensor peak torque was 20.7% lower. Strength did not differ as a function of clinical severity. Reductions in peak torque were accompanied by reduced agonist activation, although this finding only reached statistical significance at the elbow. The amount of co-contraction of antagonistic muscles was not significantly different between the two groups. These results are discussed in the context of dystonia as a disorder resulting from dysfunction of basal ganglia output.     

Prospective study examining remote effects of botulinum toxin a in children with cerebral palsy

We examined the remote effects on muscle strength and functional decline of lower-extremity botulinum toxin A injections in children with cerebral palsy. This prospective study enrolled 34 children (19 boys, 15 girls; mean age, 7.7 years) diagnosed with spastic cerebral palsy. Patients were examined at baseline and 1 month to determine if they experienced a change in upper-extremity strength (handheld dynamometry) or function (Pediatric Outcomes Data Collection Instrument). Subjects were analyzed in aggregate and by dosing group (low dose, 0-10 U/kg body weight; high dose, 11-25 U/kg) to determine if injection dose was associated with a change in remote muscle strength or function. We measured baseline and 1-month postinjection strength in shoulder flexor, shoulder abductor, elbow flexor, elbow extensor, and finger flexor muscles. None of these remote muscle groups was significantly weaker at 1 month after injection. No correlation was evident between change in muscle strength and toxin dose. These findings indicate that doses of botulinum toxin A in the lower extremities, at up to 21 U/kg, do not affect upper-extremity strength. This information can help guide dosages of botulinum toxin A in the management of spasticity in children with cerebral palsy.     

Blefarospasmo y espasmo hemifacial: tratamiento a largo plazo con toxina botulínica

Introduction

Our purpose is to describe the demographic, clinical and therapeutic characteristics of patients with blepharospasm (BS) and hemifacial spasm (HFS) in treatment with botulinum toxin type A (BtA).

Patients and methods

Retrospective analysis of patients diagnosed with BS or HFS and treated with BtA in the Neurology Department at Complejo Asistencial de Segovia between March 1991 and December 2009.

Results

Different variables were collected from 34 patients with BS and 55 with HFS, of whom 44.1% and 32.7% respectively had been undergoing treatment with BtA for more than 10 years. Elapsed time from symptom onset to the first visit was 24 months in the BS group and 59.7 months in the HFS group. Diagnosis was given on the first visit for 76.5% of the BS patients and 90.7% of the HFS patients. Patients were referred by their primary care centres in 34.6% of the cases with BS and in 77.6% of the cases with HFS. The most commonly used BtA preparation was BOTOX^® in both groups, and there were no cases of primary or secondary resistance. The median dose of BtA was raised gradually in both groups, and the increase was statistically significant during the early years of treatment. The most common side effect was ptosis (47.1% in BS, 32.5% in HFS).

Conclusions

BS and HFS are the most common facial movement disorders. The demographic and clinical characteristics and therapeutic findings from this study show that treatment with BtA is both effective and safe over the long term.     

Injection with corticosteroids (ultrasound guided) in patients with an ulnar neuropathy at the elbow,feasibility study

Introduction and purpose: Unlike carpal tunnel syndrome, little is known about injection with corticosteroids in patients with an ulnar neuropathy at the elbow (UNE). The purpose of this feasibility study is to see whether injection with corticosteroids is safe in patients with UNE and whether there are grounds to launch a prospective placebo‐controlled study on the effects of corticosteroids. Methods: Patients with clinical symptoms of UNE and a nerve conduction study compatible with UNE or thickened ulnar nerve at the elbow (> 10 mm²) by ultrasonography were included. All included patients received an ultrasound‐guided injection of 1 ml containing 40 mg methylprednisoloneacetate and 10 mg lidocainhydrochloride (Depo‐Medrol^®). Complications of the injection were monitored. After 3 months, nerve conduction studies and ultrasonography were repeated and a clinical outcome determined. Results: Eight patients with nine UNE were included. None of the patients mentioned increase in the symptoms directly after the injection nor had an infection on the injection site or haematoma. After 3 months, there was improvement of the symptoms in five patients. One patient deteriorated and three had no change of the symptoms at all. Overall, there was no significant change of the thickness of the ulnar nerve with mean difference ?0.056 mm² (95% CI ?2.56 to 2.45 mm²). Conclusion: We showed that injection with corticosteroids in patients with UNE is easy and safe, and based on this result, we found enough arguments to launch a prospective, placebo‐controlled trial to explore the effectiveness of corticosteroids in patients with UNE.     

Long-term effects of injection of botulinum toxin type A combined with home-based functional training for post-stroke patients with spastic upper limb hemiparesis

The aim of this study was to assess the effects of botulinum toxin type A (BoNT-A) injection with home-based functional training for passive and active motor function, over a 6-month period in post-stroke patients with upper limb spasticity. We studied 190 patients with at least 6-month history of stroke. They received injections of BoNT-A in upper limb muscles and detailed one-to-one instructions for home-based functional training. At baseline (before therapy), and at 1-, 3- and 6-month follow-up, Fugl-Meyer assessment (FMA) and Wolf motor function test (WMFT) were used to assess active motor function, the modified Ashworth scale (MAS) for assessment of spasticity, and the range of motion (ROM) for passive motor function. The total score for upper limb and scores of categories A and B of the FMA increased significantly at 1, 3 and 6 months, while the FMA score for category D increased significantly at 3 and 6 months, but not at 1 month. Significant decreases in the MAS scores were noted in all muscles examined at 1, 3 and 6 months, compared with baseline. The ROM for elbow joint extension significantly improved at 1, 3, and 6 months, while that for wrist joint extension increased significantly at 1 month, but not 3 or 6 months, compared with baseline. The results suggest that comprehensive improvement of motor function requires improvement of motor function in the proximal part of the upper limb and that BoNT-A followed by rehabilitation reduces spasticity and improves motor function of fingers.     

Peripheral nerve conduction in patients with a cervical rib and band

In 14 patients with wasting of the hand due to a cervical rib and band, motor and sensory conduction studies on the peripheral parts of the median and ulnar nerves were helpful in establishing the correct diagnosis. The median nerve findings excluded carpal tunnel syndrome even when the clinical pattern of wasting in the hand suggested this diagnosis. Preservation of conduction velocity in the ulnar nerve excluded ulnar entrapment at the elbow; the reduced amplitude of the ulnar sensory action potentials (SAPs) indicated that the lesion was distal to the dorsal root ganglia. In 3 patients with ulnar SAP amplitudes that were low but not clearly abnormal, the level of the lesion was confirmed by a reduced response to intradermal injection of histamine on the inner side of the forearm.     

Flexion and supination deformities of the elbow in tetraplegics

Fixed flexion and supination deformities of the elbow occur occasionally in tetraplegics. The patients in whom this was seen were those who following injury had a neurological level at C5 and who subsequently developed radial wrist extension and brachioradialis function. They were generally patients who spent long hours with their elbows flexed and supinated. A simple effective method of biceps tenotomy and plaster correction is described. Recurrence of deformity was seen if the flexion supination posture was continued. Strength of elbow flexion was not reduced. Patients maintained correction if they refrained from poor elbow posture and wore a simple plastic splint. The procedure of correction is not difficult and because of its simplicity it can be repeated if deformity recurs.     

Clinical trial readiness in non‐ambulatory boys and men with duchenne muscular dystrophy: MDA–DMD network follow‐up

Introduction: Outcomes sensitive to change over time in non‐ambulatory boys/men with Duchenne muscular dystrophy (DMD) are not well‐established. Methods: Subjects (n = 91; 16.8 ± 4.5 years old) were assessed at baseline and 6‐month intervals for 2 years. We analyzed all subjects using an intent‐to‐treat model and a subset of stronger subjects with Brooke Scale score ≤4, using repeated measures. Results: Eight patients (12–33 years old) died during the study. Sixty‐six completed 12‐month follow‐up, and 51 completed 24‐month follow‐up. Those taking corticosteroids performed better at baseline, but rates of decline were similar. Forced vital capacity percent predicted (FVC% predicted) declined significantly only after 2 years. However, Brooke and Egen Klassifikation (EK) Scale scores, elbow flexion, and grip strength declined significantly over both 1 and 2 years. Conclusion: Brooke and EK Scale scores, elbow flexion, and grip strength were outcomes most responsive to change. FVC% predicted was responsive to change over 2 years. Corticosteroids benefited non‐ambulatory DMD subjects but did not affect decline rates of measures tested here. Muscle Nerve 54 : 681–689, 2016     

联合应用尤瑞克林治疗缺血性脑卒中对患者执行功能的影响

目的观察尤瑞克林治疗对缺血性脑卒中患者后期执行功能的影响。方法 98例缺血性脑卒中患者随机分为治疗组与对照组,根据卒中治疗指南,两组均给予常规基础治疗,治疗组在此基础上联合尤瑞克林治疗,分别于治疗21d及3m后对两组患者采用蒙特利尔认知评估量表(MoCA)、简明智能状态量表(MMSE)、威斯康星卡片分类测验(WCST)进行神经心理学测验,评估执行功能。结果在21d测试中,治疗组仅在WCST中持续性错误数少于对照组(P<0.05),完成分类数多于对照组(P<0.05),但在3m后,治疗组MoCA总分高于对照组(P<0.01),治疗组MMSE成绩显著高于对照组(P<0.05),治疗组错误应答数明显减少(P<0.05),持续性错误数也明显减少(P<0.01),完成分类数增多(P<0.01)。与21d测验成绩相比,3m后第2次测验,治疗组成绩所有下降均不明显(P>0.05),而对照组MoCA总分及MMSE成绩显著下降(P<0.05),WCST持续性错误次数明显增多(P<0.01),完成分类数明显减少(P<0.01)。结论联合尤瑞克林治疗缺血性脑卒中可有效阻止患者视空间能力、认知灵活性、工作记忆等多项执行功能减退,预防血管性认知功能障碍的发生。     

Musculoskeletal growth in the upper arm in infants after obstetric brachial plexus lesions and its relation with residual muscle function

Aim Denervation after obstetric brachial plexus lesion (OBPL) is associated with reduced musculoskeletal growth in the upper arm. The aim of this study was to investigate whether reduced growth of upper arm flexor and extensor muscles is related to active elbow function and humeral length. Method In this study, 31 infants age less than 6 months (mean age 4.3mo; range 2.1–5.9mo; 17 males; 14 females;) with unilateral OBPL (Narakas class I, 19; II, 3; III, 2; and IV, 7) treated at the VU medical centre, in whom neurosurgical reconstruction was considered were prospectively studied using magnetic resonance imaging of both arms at a mean age of 4.3 months. Humeral length and the cross‐sectional area (CSA) of elbow flexor and extensor muscles were measured in both upper arms. Paresis of elbow function was estimated when the infants were a mean age of 4.5 months using the Gilbert score. Results Both flexor and extensor CSAs were significantly smaller on the affected side than on the unaffected side (88% [SD 32%], p=0.020, and 88% [SD 24%], p=0.001 respectively), as was humeral length (96% [SD 7%], p=0.005) (unaffected side 100% in all cases). There was no relation between the reduction in flexor and extensor CSA and residual muscle function. In 17 out of 31 patients, hypertrophy of flexor and/or extensor muscles was observed. Humeral length was not related to muscle parameters. Interpretation Denervation has different effects on muscle growth and function as well as bone growth. In young infants with an OBPL, muscle size is not a predictor of muscle function. Flexion contractures of the elbow later in childhood may not be explained by a dominance of flexor muscle mass in infants.     

MUSCLE STRENGTH IN MYASTHENIA GRAVIS: Effects of Exhaustion and Anticholinesterase Related to Muscle Fibre Size

Isometric muscle strength and the effects of anticholinesterase and exhaustion were measured in standardized ways in 14 myasthenia gravis patients and 50 controls. The muscle fibre size in m. biceps brachii sin was studied in 12 patients and 8 healthy controls. Significant (positive) response to anticholinesterase of elbow flexors was found in those patients where neuromuscular blocking at single fibre EMG had been found in an upper extremity muscle. In one or more of the six muscle groups tested positive response was found in 12 of the 14 patients. A low relative value of isometric muscle strength did not imply a positive response to anticholinesterase. Post-tetanic potentiation of elbow flexion strength could only be demonstrated in the group of patients with positive response of elbow flexors to anticholinesterase. A pronounced exhaustion effect was seen in four of the seven patients with positive response. The remaining three patients showed small muscle fibres of type II (see below). The muscle fibres were divided into two groups on the basis of staining reactions for myofibrillar ATPase–type I, weak reaction, and type II, strong reaction. The female patients had significantly reduced type I muscle fibre size. Significantly reduced type II fibre size was found only in the female group with positive response of elbow flexors to anticholinesterase. The 4 male patients had essentially normal fibre sizes.     

马来酸桂哌齐特对脑出血血肿体积和周围水肿带的影响

目的探讨马来酸桂哌齐特对高血压性脑出血血肿、水肿吸收及神经功能恢复的影响。方法90例高血压性脑出血患者,随机分为2组,对照组给予常规治疗,治疗组加用马来酸桂哌齐特(160mg/d)静滴。入组前、治疗7、21d测定神经功能评分、血肿和水肿体积。结果治疗7d,治疗组血肿体积、水肿体积及CSS评分明显小于对照组(P<0.05);治疗21d,治疗组无论血肿体积、水肿体积还是CSS评分均较对照组显著减少(P<0.05)。治疗21d神经功能评分与血肿体积相关性较高(P<0.01),与水肿体积无相关性(P>0.05)。结论马来酸桂哌齐特有利于高血压性脑出血水肿和血肿的吸收,改善脑出血的预后。     

尼莫地平对Alzheimer病患者认知功能和听觉事件相关电位P300的影响

目的 观察尼莫地平对Alzheimer病(AD)患者认知功能和听觉事件相关电位P300的影响.方法 将65例AD患者随机分成两组,尼莫地平治疗组(尼莫地平组,32例):尼莫地平注射液治疗;吡拉西坦对照组(吡拉西坦组,33例):吡拉西坦注射液治疗.应用简易精神状态检查量表(MMSE)评定两组患者治疗前后认知功能状况,并进行治疗前后P300检查.同时记录药物不良反应.结果 两组各30例进入结果分析.治疗前两组MMSE评分、P300的潜伏期及波幅差异无显著性(均P＞0.05).治疗14 d、30 d时,两组MMSE评分显著提高(均P＜0.01),P300潜伏期均有缩短,波幅均有提高(尼莫地平组P＜0.01,吡拉西坦组P＜0.05);治疗90 d时,尼莫地平组MMSE评分显著提高、P300潜伏期显著缩短、波幅显著提高(均P＜0.01).治疗后14 d时认知功能改善尼莫地平组明显优于吡拉西坦组(总有效率分别为76.67%,70%)(P＜0.05).两组治疗期间无严重不良反应.结论 尼莫地平注射液能够改善AD患者的认知功能,比吡拉西坦更有效;这可能与尼莫地平的脑保护作用有关.     

Does reducing spasticity translate into functional benefit? An exploratory meta-analysis

BACKGROUND: Spasticity and loss of function in an affected arm are common after stroke. Although botulinum toxin is used to reduce spasticity, its functional benefits are less easily demonstrated. This paper reports an exploratory meta-analysis to investigate the relationship between reduced arm spasticity and improved arm function. METHOD: Individual data from stroke patients in two randomised controlled trials of intra-muscular botulinum toxin were pooled. The Modified Ashworth Scale (elbow, wrist, fingers) was used to calculate a "Composite Spasticity Index". Data from the arm section of the Barthel Activities of Daily Living Index (dressing, grooming, and feeding) and three subjective measures (putting arm through sleeve, cleaning palm, cutting fingernails) were summed to give a "Composite Functional Index". Change scores and the time of maximum change were also calculated. RESULTS: Maximum changes in both composite measures occurred concurrently in 47 patients. In 26 patients the improvement in spasticity preceded the improvement in function with 18 showing the reverse. There was a definite relationship between the maximum change in spasticity and the maximum change in arm function, independent of treatment (rho = -0.2822, p = 0.0008, n = 137). There was a clear relationship between the changes in spasticity and in arm function in patients treated with botulinum toxin (Dysport) at 500 or 1000 units (rho = -0.5679, p = 0.0090, n = 22; rho = -0.4430, p = 0.0018, n = 47), but not in those treated with placebo or 1500 units. CONCLUSIONS: Using a targeted meta-analytic approach, it is possible to demonstrate that reducing spasticity in the arm is associated with a significant improvement in arm function.     

Botulinum toxin A injection for spasticity in diplegic-type cerebral palsy

Botulinum toxin type A can be both safe and effective in relieving spasticity in pediatric patients with cerebral palsy. In our prospective study, we evaluated the functional effect of botulinum toxin A in spastic diplegic-type cerebral palsy. Patients were examined on enrollment and at 1, 3, and 6 months after injection. Passive dorsiflexion of the ankle joint was measured using a goniometer as an angle of possible maximal dorsiflexion with the knee extended and flexed. Spasticity was graded using the Modified Ashworth Scale. Selective motor control at the ankle was assessed, and observational gait analysis was done. The functional status of the patients was determined by using the gross motor classification system. Botulinum toxin A was injected into the gastrocnemius muscle in all patients, and in four patients with concomitant jump knee gait, a hamstring muscle injection was added. Fourteen patients were included in the study. The mean age was 58.81 +/- 15.34 months. Following injection, spasticity was clinically decreased and statistically significant improvement was noticed in all clinical parameters after 1, 3, and 6 months of injection. The improvement in the clinical parameters decreased after 6 months but not to the baseline. One patient was Level II, four patients were Level III, and six patients were Level IV according to the Gross Motor Function Classification System at baseline. Improvement in the gross motor classification system is continued after 6 months in 12 children. The main goal of spasticity treatment in cerebral palsy is functional improvement. In our study, most of our patients had functional improvement according to the gross motor function classification system and did not change at 6 months.     

Short-segment nerve conduction studies in ulnar neuropathy at the elbow

The aim of the study was to assess the diagnostic value of short-segment nerve conduction studies (NCS) at 2-cm intervals from 4 cm above to 4 cm below the medial epicondyle in a large group of patients with ulnar neuropathy at the elbow (UNE). Furthermore, we wanted to compare electrodiagnostic and clinical findings. We evaluated 73 arms in 70 patients with UNE and observed the following abnormalities on short-segment NCS: focal conduction block (CB) in 1, focal CB with increased latency change in 34, and increased latency change alone in 25. Short-segment NCS had an additional localizing value in 28 arms of the 37 patients (76%) with motor conduction velocity (MCV) slowing across the elbow only or with nonlocalizing electrodiagnostic findings. The lesion was located above the elbow in 32 arms (53%), at the epicondyle in 16 arms (27%), and below the epicondyle in 12 (20%) of the 60 arms with focal CB or increased latency change on short-segment NCS. Patients with CB on routine and short-segment NCS had muscle weakness significantly more often than patients without CB. Thus, short-segment NCS are useful in localizing the lesion in patients with UNE and CB on routine NCS and have additional diagnostic value in patients with MCV slowing across the elbow or with nonlocalizing signs on routine nerve conduction studies. We recommend its use in all patients in whom UNE is suspected.     

Prediction of voluntary activation, strength and endurance of elbow flexors in postpolio patients

To examine the long-term effects of polio, maximal voluntary strength and voluntary activation of elbow flexor muscles of 177 patients from a postpolio clinic were investigated using twitch interpolation. Muscle endurance was studied in 142 patients during 45 min of submaximal exercise, and predictors of impaired muscle performance were investigated. Twenty-nine of 177 patients (16.4%) had impaired voluntary drive to their elbow flexor muscles, but only 16 (9.0%) had markedly reduced elbow flexor strength, despite 74 (41.8%) reporting they were initially affected in their tested limb and 172 (97.2%) patients reporting new generalized symptoms. Seven patients had impaired muscle endurance in the tests of strength and voluntary drive. During the submaximal exercise, 16 patients (11.3%) had impaired peripheral muscle endurance with normal voluntary activation. These results confirm a low incidence of impaired upper-limb muscle performance in postpolio patients, despite many patients having subjective symptoms consistent with postpolio syndrome. There was an increased relative risk for impaired muscle function in those patients with a subjective decrease in strength in the tested limb, a recent decline in activities of daily living in their tested limb, and who used orthotic devices in their tested limb. Monitoring of function in prior-polio patients with impaired muscle performance may be useful, particularly when combined with investigation of other potential contributory factors to the functional impairment.     

Endothelial function markers in parkinsonian patients with hyperhomocysteinemia.

Hyperhomocysteinemia is considered a risk factor for vascular disease causing endothelial damage and consequently atherogenesis. The purpose of this study was to investigate the effect of elevated homocysteine on certain biochemical markers of endothelial function in patients with idiopathic Parkinson's disease (PD). Blood homocysteine levels were assessed in 57 PD patients and 40 matched normal controls. Investigation of the C677T 5,10 methylenetetrahydrofolate reductase (MTHFR) genotype was also performed in 43 PD patients. The following markers of endothelial function were assessed: superoxide dismutase (SOD), nitric oxide (NO), sICAM-1 and sE-selectin. Homocysteine levels were found mildly elevated in PD patients particularly in those treated with L-Dopa. MTHFR genotype did not influence significantly this finding. SOD activity was found reduced but it was not correlated to homocysteine levels. All other parameters measured were normal and were not related to hyperhomocysteinemia. Our findings indicate that mild hyperhomocysteinemia in PD patients was not associated with endothelial dysfunction.