Treadmill training in patients affected by Charcot-Marie-Tooth neuropathy: results of a multicenter, prospective, randomized, single-blind, controlled study.

BACKGROUND AND PURPOSE: Muscle-strengthening, stretching or proprioceptive treatments may slow symptom progression in Charcot-Marie-Tooth (CMT) neuropathy. The aim of the study was to evaluate safety and efficacy of treadmill training in CMT1A. METHODS: We planned a multicenter, prospective, randomized, single-blind, controlled study. We recruited 53 outpatients affected by CMT1A and randomized them into two treatment groups: one underwent stretching and proprioceptive exercise, whereas the other was additionally treated with treadmill training (TreSPE). Primary outcome measures (OMs) were the walking evaluations and secondary OM was the balance assessment. All participants were assessed at baseline and after 3 and 6 months of treatment. RESULTS: Most patients showed an improvement in at least one OM after 3 months [42/47 (89.4%)] and 6 months [38/40 (95%)] of treatment. No adverse events were reported in either group. CONCLUSIONS: The most important finding was that both stretching and proprioceptive exercise and treadmill training had an objective benefit on patients affected by CMT disease, without causing overwork weakness. We had a low rate of drop out and did not find deterioration in motor performance. Our results also confirm that applying evidence-based medicine methods to rehabilitative research is the correct way to test the efficacy of a treatment.

Development of a patient-centered questionnaire for post-stroke spasticity assessment: a reliability study.

In the emerging scenario of patient-centered medicine, it is becoming increasingly important to involve patients in the management of chronic diseases. The rehabilitation field currently has no assessment tool for evaluating the functional impact of post-stroke spasticity on activities of daily living. The aim of this study was to identify a tool to fill this gap. The "Spasticity Questionnaire in Real Life" (SPQR) was administered, twice, to 39 patients with poststroke spasticity. Statistical analysis showed internal consistency and reliability of the questionnaire, with values greater than 0.96 and 0.76, respectively. These results show that the SPQR is a promising tool for evaluating the functional impact of post-stroke spasticity.

Management of spasticity with onabotulinumtoxinA: practical guidance based on the italian real-life post-stroke spasticity survey.

The present paper provides practical guidance on the management of adult spasticity with OnabotulinumtoxinA. Advisory Board members reviewed the available evidence and discussed their personal experiences in order to address the unmet needs in the management of spasticity with botulinum toxin type A identified by the recent Italian Real-Life Post-Stroke Spasticity Survey. Stroke patients should be referred to spasticity services that have adequate facilities and multidisciplinary teams with the necessary training, competence and expertise. The current literature shows a strong correlation between the development of post-stroke spasticity and the degree of central sensorimotor system destruction/disorganization. Use of tools such as the Poststroke Checklist may help clinicians in the long-term follow-up of stroke patients. The maximum dose of onabotulinumtoxinA - according to the current literature this ranges from 300U to 400U for upper limb and from 500U to 600U for lower limb aggregate postures - should be re-considered. In addition, there is a need for future consensus (also based on pharmacoeconomic considerations) on consistent clinical care models for the management of patients with post-stroke spasticity.

Post Soft Care: Italian implementation of a post-stroke checklist software for primary care and identification of unmet needs in community-dwelling patients.

The needs of community-dwelling stroke patients are often not fully addressed. The Global Stroke Community Advisory Panel had developed the Post Stroke Checklist (PSC) with the aim of standardizing long-term stroke assessment and consequently care of patients. PSC has been validated for UK and Singapore, and an Italian version has been proposed. The aim of this study is to assess the needs of community-dwelling Italian stroke patients using an online version of PSC. Secondary aim is to assess utility of PSC for patients and clinicians. The PSC was administered to a sample of 64 patients with stroke in Italy by general practioners. PSC is a questionnaire formed by 11 questions, each one covering a domain identified as important for patients' health. After administration of PSC, patient and clinician satisfaction with the PSC was assessed by a specific questionnaire. The PSC identified specific problems for patients related to a worsening from the last examination not evaluated by any specialist. The online PSC highlighted problems related to mood, (in 50% of sample), mobility (53.1%), spasticity (42.2%), and pain (37.5%). Both patients and clinicians provided good results about the utility of online PSC. The PSC was confirmed to be a useful tool for identifying the needs of community-dwelling patients with stroke. Particular attention should be deserved to problems related to mood, mobility, and hence participation to social life, pain, and spasticity. For taking into account these aspects, the online PSC can be a useful portable tool for clinicians.

Electrodiagnostic and nerve ultrasonographic features in upper limb spasticity: an observational study.

To better understand the effects of spasticity on peripheral nerves, we evaluated the electrodiagnostic and nerve ultrasonographic features of the median and ulnar nerves in adults with upper limb spasticity. Twenty chronic stroke patients with spastic hemiparesis underwent nerve conduction study and nerve ultrasonography of the median and ulnar nerves at both upper limbs. Affected versus unaffected upper limb comparisons showed significant differences in the median and ulnar nerve distal motor latencies, compound muscle action potentials and F-wave minimal latencies. Furthermore, we observed a significantly greater median nerve crosssectional area at the elbow of the affected upper limb compared with the unaffected one. Our findings confirmed electrodiagnostic asymmetries and nerve ultrasonographic abnormalities in the affected versus the unaffected upper limb after stroke. Slight changes in lower motor neuron activity and spasticity might contribute to these alterations.

European core curriculum in neurorehabilitation.

To date, medical education lacks Europe-wide standards on neurorehabilitation. To address this, the European Federation of NeuroRehabilitation Societies (EFNR) here proposes a postgraduate neurorehabilitation training scheme. In particular, the European medical core curriculum in neurorehabilitation should include a two-year residency in a neurorehabilitation setting where trainees can gain practical experience. Furthermore, it should comprise six modules of classroom training organized as weekend seminars or summer/winter schools. In conclusion, after defining the European medical core curriculum in neurorehabilitation, the next activities of the EFNR will be to try and reach the largest possible consensus on its content among all national societies across Europe in order to further validate it and try to extend it to the other, non-medical, professionals on the neurorehabilitation team in line with their core curricula defined by each professional association.

The Italian real-life post-stroke spasticity survey: unmet needs in the management of spasticity with botulinum toxin type A.

The present national survey seeking to identify unmet needs in the management of spasticity with botulinum toxin type A focused on the use of OnabotulinumoxinA, since this is the brand with the widest range of licensed indications in Italy. Physicians from twenty-four Italian neurorehabilitation units compiled a questionnaire about "real-life" post-stroke spasticity management. OnabotulinumtoxinA was reported to be used in the following average doses: upper limb 316.7 ± 79.1 units; lower limb 327.8 ± 152.3; upper and lower limb 543.7 ± 123.7 units. Of the physicians surveyed, 37.5% felt that increasing the frequency of OnabotulinumtoxinA injection would improve its efficacy; 70.8% use electrical stimulation/electromyography guidance (one fourth of injections with no instrumental guidance). Instrumental evaluation was used by 41.7% of the physicians. The participants expressed the view that early identification of post-stroke spasticity would be facilitated by the availability of a post-stroke checklist, and that this should be used by physiotherapists (91.7%), physiatrists (58.3%), family doctors (50%), stroke unit physicians (25%), patients and caregivers (79.2%). According to our findings, the management of poststroke spasticity has several unmet needs that, were they addressed, might improve these patients' clinical outcomes and quality of life. These needs concern patient follow-up, where a clearly defined pathway is lacking; furthermore, there is a need to use maximum doses per treatment and to ensure early intervention on post-stroke spasticity.

Topical distribution of initial paresis of the limbs to predict clinically relevant spasticity after ischemic stroke: a retrospective cohort study.

BACKGROUND: The degree of initial paresis relates to spasticity development in stroke patients. However, the importance of proximal and distal paresis in predicting spasticity after stroke is unclear. AIM: To investigate the role of topical distribution of initial limb paresis to predict clinically relevant spasticity in adults with stroke. DESIGN: Retrospective cohort study METHODS: Seventy-two first-ever ischemic stroke patients were examined. At the acute phase of illness, demographics and the European Stroke Scale motor items (maintenance of outstretched arm position, arm raising, wrist extension, grip strength, maintenance of outstretched leg position, leg flexion, foot dorsiflexion) were evaluated. At six months after the stroke onset, spasticity was assessed at the upper and lower limb with the modified Ashworth Scale. Clinically relevant spasticity was defined as modified Ashworth Scale ≥3 (0-5). RESULTS: The degree of initial paresis of the proximal muscles of the upper limb and the distal muscles of the lower limb showed the strongest association and the best profile of sensitivity-specificity in predicting clinically relevant spasticity at the upper and lower limb, respectively. Younger age showed higher risk for developing clinically relevant spasticity in the upper limb. CONCLUSIONS: Our findings support the hypothesis that the initial degree of proximal paresis of the upper limb and distal paresis of the lower limb as well as age may be considered early predictors of clinically relevant spasticity in adults with ischemic stroke. CLINICAL REHABILITATION IMPACT: Our findings further improve the role of initial paresis as predictor of spasticity after stroke.

Rehabilitation of brachial plexus injuries in adults and children.

Management of brachial plexus injury sequelae is a challenging issue in neurorehabilitation. In the last decades great strides have been made in the areas of early diagnosis and surgical techniques. Conversely, rehabilitation of brachial plexus injury is a relatively unexplored field. Some critical aspects regarding brachial plexus injury rehabilitation have to be acknowledged. First, brachial plexus injury may result in severe and chronic impairments in both adults and children, thus requiring an early and long-lasting treatment. Second, nerve damage causes a multifaceted clinical picture consisting of sensorimotor disturbances (pain, muscle atrophy, muscle weakness, secondary deformities) as well as reorganization of the Central Nervous System that may be associated with upper limb underuse, even in case of peripheral injured nerves repair. Finally, psychological problems and a lack of cooperation by the patient may limit rehabilitation effects and increase disability. In the present paper the literature concerning brachial plexus injury deficits and rehabilitation in both adults and children was reviewed and discussed. Although further research in this field is recommended, current evidence supports the potential role of rehabilitation in reducing both early and long-lasting disability. Furthermore, the complexity of the functional impairment necessitates an interdisciplinary approach incorporating various health professionals in order to optimizing outcomes.

Applicability of a new robotic walking aid in a patient with cerebral palsy. Case report.

BACKGROUND: Gait training with the help of assistive technological devices is an innovative field of research in neurological rehabilitation. Most of the available gait training devices do not allow free movement in the environment, which would be the most suitable natural and motivating condition for training children with neurological gait impairment. AIM: To evaluate the potential applicability of a new robotic walking aid as a tool for gait training in non-ambulatory children with Cerebral Palsy. DESIGN: Single case study SETTING: Outpatient regimen POPULATION: A 11-years-old child unable to stand and walk independently as a result of spastic tetraplegic cerebral palsy (CP). METHODS: The experimental device was a newly actuated version of a dynamic combined walking and standing aid (NF-Walker®) available in the market which was modified by means of two pneumatic artificial muscles driven by a foot-switch inserted in the shoes. The child was tested at baseline (while maintaining the standing position aided by the non-actuated NF-Walker®) and in the experimental condition (while using the actuated robotic aid). The outcome measures were: 2-minute walking test, 10-metre walking test, respiratory and heart parameters, energy cost of locomotion. RESULTS: At baseline, the child was unable to perform any autonomous form of locomotion. When assisted by the actuated aid (i.e. during the experimental condition), the child was successful in moving around in his environment. His performance was 19.63 m in the 2-minute walking test and 64 s in the 10-metre walking test. Respiratory and heart parameters were higher than healthy age-matched children both at baseline and in the experimental condition. The energy cost of gait, which was not valuable in the baseline condition, was significantly higher than normality during the experimental condition. CONCLUSION: The new robotic walking aid may help children suffering from CP with severe impairment of gait to move around in their environment. CLINICAL REHABILITATION IMPACT: This new robotic walking device may have a potential impact in stimulating the development and in training of gait in children with neurological gait impairment. Future studies are warranted in order to test this hypothesis.

Stimulating visual exploration of the neglected space in the early stage of stroke by hemifield eye-patching: a randomized controlled trial in patients with right brain damage.

BACKGROUND: It has been well established that the presence of neglect is a predictor of poor functional outcome after stroke. Most rehabilitation studies on neglect have been performed with at least two months post-stroke. However, a recent series of stroke management indications highlight the importance of early rehabilitation treatment and evidence regarding neglect rehabilitation in the early phase after stroke is needed. AIM: To evaluate the effectiveness of right half-field patches in treating neglect in patients during the early phase of stroke. DESIGN: Randomized controlled trial. SETTING: Acute care setting in an urban general hospital. POPULATION: Eighteen patients with left unilateral neglect recruited among 56 patients consecutively admitted with right hemispheric stroke. METHODS: The patients were evaluated at pre-treatment, post-treatment, and at a 7-day follow-up. The experimental group received right half-field patch treatment (n=10) for approximately 8 hours a day for 15 consecutive days. The control group received visual scanning training (n=8) for 40 minutes every weekday in a 15 day period. RESULTS: Both groups significantly improved their performance in all outcome measures. No difference in the amount of improvement between the two groups was found. CONCLUSION: Right half-field eye patching could be a promising technique for treating visual spatial neglect during the early stages of stroke. CLINICAL REHABILITATION IMPACT: The eye-patching technique may represent an easily applicable and inexpensive method for neglect rehabilitation in the early stage after stroke.

Effects of myofascial technique in patients with subacute whiplash associated disorders: a pilot study.

BACKGROUND: Whiplash associated disorders commonly affect people after a motor vehicle accident, causing a variety of disabling manifestations. Some manual and physical approaches have been proposed to improve myofascial function after traumatic injuries, in order to effectively reduce pain and functional limitation. AIM: To evaluate whether the application of the Fascial Manipulation© technique could be more effective than a conventional approach to improve cervical range of motion in patients with subacute whiplash associated disorders. DESIGN: Pilot randomized clinical trial. METHODS: Eighteen patients with subacute whiplash associated disorders were randomized into two groups. Group A (N.=9) received three, 30-minute sessions, (every five days during a two week period) of neck Fascial Manipulation©. Group B (N.=9) received ten, 30-minute sessions (five days a week for two consecutive weeks) of neck exercises plus mobilization. Patients were evaluated before, immediately after and two weeks post-treatment. PRIMARY OUTCOME MEASURES: cervical active range of motion (flexion, extension, right lateral-flexion, left lateral-flexion, right rotation, and left rotation). RESULTS: A statistically significant improvement in neck flexion was found after treatment in favour of Group A (60.2±10.8°) compared with Group B (46.3±15.1°). No differences were found between groups for the other primary outcomes at post-treatment or follow-up. CONCLUSION: The Fascial Manipulation© technique may be a promising method to improve cervical range of motion in patients with subacute whiplash associated disorders. CLINICAL REHABILITATION IMPACT: Myofascial techniques may be useful for improving treatment of subacute whiplash associated disorders also reducing their economic burden.

Active ankle dorsiflexion and the Mingazzini manoeuvre: two clinical bedside tests related to prognosis of postural transferring, standing and walking ability in patients with stroke.

BACKGROUND: Lower limb paresis is one of the main determinants of postural transferring, standing and walking disability in patients with stroke. Early prognosis of recovery of lower limb function and of related functional disability is an important issue in neurorehabilitation clinical practice. AIM: Aim of this study was to assess the relationship between active ankle dorsiflexion and the Mingazzini manoeuvre with the prognosis of lower limb function and of postural transferring, standing and walking ability in patients with stroke. DESIGN: This was a longitudinal study with prospectively collected data. SETTING: University hospital. POPULATION: The study included 53 patients with first unilateral brain ischemic stroke. METHODS: Patients were evaluated initially (mean 4.02 days) and approximately at six months (mean 178.6 days) after stroke. Initial assessment included active ankle dorsiflexion and the Mingazzini manoeuvre. The assessment after six months included three outcome measures evaluating the rate of improvement of lower limb function and of postural transferring, standing and walking ability (Postural Assessment Scale for Stroke patients, Functional Ambulation Category, Motricity Index leg subtest). RESULTS: The active ankle dorsiflexion showed to be related with the prognosis of lower limb function and of walking ability, while the Mingazzini manoeuvre was related with the improvement of postural transferring and standing ability. CONCLUSION: Active ankle dorsiflexion and the Mingazzini manoeuvre are related with the prognosis of lower limb function and of postural transferring, standing and walking ability in patients with stroke. CLINICAL REHABILITATION IMPACT: These simple bedside tests give a picture of improvement potential of motor activities connected to lower limb function in patients with acute stroke.

Rehabilitation procedures in the management of spasticity.

Spasticity is a major disabling symptom in many patients with spinal and/or cerebral lesions. During functional movements, spasticity manifests itself within the complex condition of the "spastic movement disorder". The pathophysiology of the spastic movement disorder relies on multiple factors including abnormal supraspinal drive, abnormal control of reflex activities, and changes in muscle mechanical properties. The most widely used procedures for management of spasticity are represented by pharmacological treatment aimed at inhibiting reflex hyperexcitability. In the last decades, several non pharmacological procedures for treating spasticity have been put forward, including muscle stretching, muscle reinforcement, physical agents and pain management. These procedures may have both neurophysiological and biomechanical effects on the spastic movement disorder. In the present paper, the literature concerning non-pharmacological procedures in the treatment of spasticity was reviewed and discussed, taking into account the multifaceted pathophysiology of the spastic movement disorder. Although further research in this field is recommended, existing evidence supports the potential role of rehabilitation interventions as a therapeutic tool, which could be integrated with traditional pharmacological procedures in the management of the spastic movement disorder.

Augmentative and alternative communication in adolescents with severe intellectual disability: a clinical experience.

AIM: Augmentative and alternative communication devices proved to be effective in patients with severe intellectual disability to overcome their communication impairments. In order to give a contribution for design of augmentative and alternative communication systems that better meet the needs of beginning communicators we decided to report our clinical experience about using augmentative and alternative communication in adolescents with severe intellectual disability. METHODS: Five patients who underwent a long time traditional speech rehabilitation program (at least 5 years) with scant improvements in linguistic function were recruited and evaluated by means of the Vineland Adaptive Behaviour Scale before and after a three years augmentative and alternative communication intervention carried out by a multidisciplinary team. RESULTS: After the rehabilitative intervention patients showed an improvement in communication, daily living skills and socialization as measured by the Vineland Adaptive Behaviour Scale. CONCLUSION: Augmentative and alternative communication is an effective rehabilitation approach to people with severe intellectual disability and impairments in linguistic expression. Moreover augmentative and alternative communication is a useful tool allowing these patients to increase their social participation also enhancing their self-esteem. Our clinical experience confirmed these topics also in adolescents who underwent a long time traditional speech rehabilitation program with scant improvements, providing practical information to clinicians.

Are indexes of arm recovery related to daily life autonomy in patients with stroke?

AIM: The level of daily life autonomy in patients with stroke may be related to recovery of affected arm function. The aim of the study was to assess whether four simple bedside indexes of arm recovery can predict levels of autonomy in daily life activities. METHODS: A consecutive sample of 48 patients presenting with upper limb paresis/plegia in the acute stage after stroke was selected. Patients underwent five evaluation sessions at 7, 14, 30, 90 and 180 days after stroke. Forward stepwise multiple regression analysis was used to clarify the role of four potential predictors of upper limb recovery (active finger extension, shoulder abduction, shoulder shrug and hand movement scales). Dependent variables in these models were the Barthel Index score and sub-items of the Burke-Fahn-Marsden Scale. RESULTS: The active finger extension scale showed a highly significant statistical correlation with patient performance in nearly all outcome measures. The shoulder shrug correlated with the BI score, and with the dressing and hygiene Burke-Fahn-Marsden Scale sub-items. Shoulder abduction and hand movement scale played only a minor role. CONCLUSIONS: The active finger extension scale proved to be a strong early predictor of recovery of daily life autonomy in patients with stroke. This finding could be important in order to planning a specific rehabilitation treatment after the onset.

Left ventricular myocardial function in myotonic dystrophy.

Myotonic dystrophy is an autosomal dominant disease affecting many organ systems, including the heart. Abnormalities of the cardiac conduction system are a frequent and well-documented finding in this neuromuscular disease, whereas overt signs of heart failure are rarely reported. However, controversy exists about the prevalence of preclinical left ventricular (LV) dysfunction in patients with myotonic dystrophy who have no symptoms of heart failure. To address this issue, load-independent LV function indexes were compared in patients with myotonic dystrophy and in normal subjects. LV measurements were obtained with M-mode echocardiography in 43 consecutive patients with myotonic dystrophy (mean age 35 +/- 14 years) who had no clinical evidence of heart failure, and in 35 sex- and age-matched controls. A cuff sphygmomanometer was used to determine blood pressure. No difference was found between patients and control subjects in the percentage of LV ejection fraction predicted for end-systolic stress (103 +/- 7 vs 103 +/- 7%; p = 0.97) or in the end-systolic stress/volume index ratio (2.9 +/- 0.5 vs 2.7 +/- 0.4 dyne7/cm3; p = 0.09). Similarly, no significant difference in LV myocardial function was detected between the 19 patients with a mild degree of the neuromuscular disease and in the 24 with a moderate or severe degree of the disease. Finally, LV myocardial function was compared between patients with myotonic dystrophy and atrioventricular or intraventricular conduction disturbances (n = 8) and those without these disturbances (n = 35), but no significant difference in the percentage of LV ejection fraction predicted for end-systolic stress or in the end-systolic stress/volume index ratio could be detected.(ABSTRACT TRUNCATED AT 250 WORDS)

The natural course of cardiac conduction disturbances in myotonic dystrophy.

In this study we noninvasively followed for a mean period of 46 months 21 patients with different grades of myotonic dystrophy to evaluate the course of the involvement of the cardiac conducting system. Six patients (28.6%), 1 affected by a mild and 5 by a severe form of the neuromuscular disorder, showed appearance or deterioration of conduction disturbances suggesting a serious derangement of the specialized tissue; in 3 of them a permanent demand pacemaker was implanted. Conduction defects are the most frequent cardiac manifestations in myotonic dystrophy and become worse with time, mainly in patients with higher degrees of the disease. Furthermore, first-degree atrioventricular block may represent an early sign of developing of more advanced conduction defects.