Effects of dance therapy on balance, gait and neuro-psychological performances in patients with Parkinson's disease and postural instability

Postural Instability (PI) is a core feature of Parkinson’s Disease (PD) and a major cause of falls and disabilities. Impairment of executive functions has been called as an aggravating factor on motor performances. Dance therapy has been shown effective for improving gait and has been suggested as an alternative rehabilitative method. To evaluate gait performance, spatial-temporal (S-T) gait parameters and cognitive performances in a cohort of patients with PD and PI modifications in balance after a cycle of dance therapy

Functional impairment in patients with myotonic dystrophy type 1 can be assessed by an ataxia rating scale (SARA)

Myotonic dystrophy type 1 (DM1) is not characterised by ataxia per se; however, DM1 and ataxia patients show similar disturbances in movement coordination often experiencing walking and balance difficulties, although caused by different underlying pathologies. This study aims to investigate the use of a scale previously described for the assessment and rating of ataxia (SARA) with the hypothesis that it could have utility in DM1 patients as a measure of disease severity and risk of falling. Data from 54 DM1 patients were pulled from the PHENO-DM1 natural history study for analysis. Mean SARA score in the DM1 population was 5.45 relative to the maximum score of eight. A flooring effect (score 0) was observed in mild cases within the sample. Inter-rater and test–retest reliability was high with intraclass coefficients (ICC) of 0.983 and 1.00, respectively. Internal consistency was acceptable as indicated by a Cronbach’s alpha of 0.761. Component analysis revealed two principle components. SARA correlated with: (1) all measures of muscle function tested, including quantitative muscle testing of ankle dorsiflexion (r = −0.584*), the 6 min walk test (r = −0.739*), 10 m walk test (r = 0.741*), and the nine hole peg test (r = 0.602*) and (2) measures of disease severity/burden, such as MIRS (r = 0.718*), MDHI (r = 0.483*), and DM1-Activ (r = −0.749*) (*p < 0.001). The SARA score was predicted by an interaction between modal CTG repeat length and age at sampling (r = 0.678, p = 0.003). A score of eight or above predicted the use of a walking aid with a sensitivity of 100% and a specificity of 85.7%. We suggest that further research is warranted to ascertain whether SARA or components of SARA are useful outcome measures for clinical trials in DM1. As a tool, it can be used for gathering information about disease severity/burden and helping to identify patients in need of a walking aid, and can potentially be applied in both research and healthcare settings

Motor patterns evaluation of people with neuromuscular disorders for biomechanical risk management and job integration/reintegration

Neurological diseases are now the most common pathological condition and the leading cause of disability, progressively worsening the quality of life of those affected. Because of their high prevalence, they are also a social issue, burdening both the national health service and the working environment. It is therefore crucial to be able to characterize altered motor patterns in order to develop appropriate rehabilitation treatments with the primary goal of restoring patients' daily lives and optimizing their working abilities. In this thesis, I present a collection of published scientific articles I co-authored as well as two in progress in which we looked for appropriate indices for characterizing motor patterns of people with neuromuscular disorders that could be used to plan rehabilitation and job accommodation programs. We used instrumentation for motion analysis and wearable inertial sensors to compute kinematic, kinetic and electromyographic indices. These indices proved to be a useful tool for not only developing and validating a clinical and ergonomic rehabilitation pathway, but also for designing more ergonomic prosthetic and orthotic devices and controlling collaborative robots

Consensus Paper: Neurophysiological Assessments of Ataxias in Daily Practice

The purpose of this consensus paper is to review electrophysiological abnormalities and to provide a guideline of neurophysiological assessments in cerebellar ataxias. All authors agree that standard electrophysiological methods should be systematically applied in all cases of ataxia to reveal accompanying peripheral neuropathy, the involvement of the dorsal columns, pyramidal tracts and the brainstem. Electroencephalography should also be considered, although findings are frequently non-specific. Electrophysiology helps define the neuronal systems affected by the disease in an individual patient and to understand the phenotypes of the different types of ataxia on a more general level. As yet, there is no established electrophysiological measure which is sensitive and specific of cerebellar dysfunction in ataxias. The authors agree that cerebellar brain inhibition (CBI), which is based on a paired-pulse transcranial magnetic stimulation (TMS) paradigm assessing cerebellar-cortical connectivity, is likely a useful measure of cerebellar function. Although its role in the investigation and diagnoses of different types of ataxias is unclear, it will be of interest to study its utility in this type of conditions. The authors agree that detailed clinical examination reveals core features of ataxia (i.e., dysarthria, truncal, gait and limb ataxia, oculomotor dysfunction) and is sufficient for formulating a differential diagnosis. Clinical assessment of oculomotor function, especially saccades and the vestibulo-ocular reflex (VOR) which are most easily examined both at the bedside and with quantitative testing techniques, is of particular help for differential diagnosis in many cases. Pure clinical measures, however, are not sensitive enough to reveal minute fluctuations or early treatment response as most relevant for pre-clinical stages of disease which might be amenable to study in future intervention trials. The authors agree that quantitative measures of ataxia are desirable as biomarkers. Methods are discussed that allow quantification of ataxia in laboratory as well as in clinical and real-life settings, for instance at the patients' home. Future studies are needed to demonstrate their usefulness as biomarkers in pharmaceutical or rehabilitation trials

Consensus Paper: Radiological Biomarkers of Cerebellar Diseases

Hereditary and sporadic cerebellar ataxias represent a vast and still growing group of diseases whose diagnosis and differentiation cannot only rely on clinical evaluation. Brain imaging including magnetic resonance (MR) and nuclear medicine techniques allows for characterization of structural and functional abnormalities underlying symptomatic ataxias. These methods thus constitute a potential source of radiological biomarkers, which could be used to identify these diseases and differentiate subgroups of them, and to assess their severity and their evolution. Such biomarkers mainly comprise qualitative and quantitative data obtained from MR including proton spectroscopy, diffusion imaging, tractography, voxel-based morphometry, functional imaging during task execution or in a resting state, and from SPETC and PET with several radiotracers. In the current article, we aim to illustrate briefly some applications of these neuroimaging tools to evaluation of cerebellar disorders such as inherited cerebellar ataxia, fetal developmental malformations, and immune-mediated cerebellar diseases and of neurodegenerative or early-developing diseases, such as dementia and autism in which cerebellar involvement is an emerging feature. Although these radiological biomarkers appear promising and helpful to better understand ataxia-related anatomical and physiological impairments, to date, very few of them have turned out to be specific for a given ataxia with atrophy of the cerebellar system being the main and the most usual alteration being observed. Consequently, much remains to be done to establish sensitivity, specificity, and reproducibility of available MR and nuclear medicine features as diagnostic, progression and surrogate biomarkers in clinical routine

A review of gait disorders in the elderly and neurological patients for robot-assisted training

Purpose: Ambulation is an important objective for people with pathological gaits. Exoskeleton robots can assist these people to complete their activities of daily living. There are exoskeletons that have been presented in literature to assist the elderly and other pathological gait users. This article presents a review of the degree of support required in the elderly and neurological gait disorders found in the human population. This will help to advance the design of robot-assisted devices based on the needs of the end users. Methods: The articles included in this review are collected from different databases including Science Direct, Springer Link, Web of Science, Medline and PubMed and with the purpose to investigate the gait parameters of elderly and neurological patients. Studies were included after considering the full texts and only those which focus on spatiotemporal, kinematic and kinetic gait parameters were selected as they are most relevant to the scope of this review. A systematic review and meta-analysis were conducted. Results: The meta-analysis report on the spatiotemporal, kinematic and kinetic gait parameters of elderly and neurological patients revealed a significant difference based on the type and level of impairment. Healthy elderly population showed deviations in the gait parameters due to age, however, significant difference is observed in the gait parameters of the neurological patients. Conclusion: A level of agreement was observed in most of the studies however the review also noticed some controversies among different studies in the same group. The review on the spatiotemporal, kinematics and kinetic gait parameters will provide a summary of the fundamental needs of the users for the future design and development of robotic assistive devices. Implications for rehabilitation The support requirements provide the foundation for designing assistive devices. The findings will be crucial in defining the design criteria for robot assistive devices

Die Gangstörung bei Patienten mit orthostatischem Tremor

Der primäre orthostatische Tremor ist eine seltene Erkrankung, die einen hochfrequenten Tremor von 13-18 Hz der unteren Extremität beim Stehen aufweist mit einer Symptomlinderung beim Sitzen, Gehen oder Hinlegen. Der Erkrankung liegt ein pathologisches, ponto-zerebello-thalamo-kortikales Tremornetzwerk zugrunde, das auch im Liegen weiter aktiviert bleibt. Im Zusammenhang mit dieser Erkrankung befasst sich diese Dissertation mit folgenden Fragestellungen: 1) Wie verändert sich der Tremor bei der Transition vom Stehen zum Gehen? 2) Gibt es eine objektivierbare Gangstörung trotz subjektiver Symptomregredienz beim Gehen? 3) Kann der Tremor durch propriozeptive Reize moduliert und gegebenenfalls gelindert werden? In einer klinischen Untersuchung mittels eines drucksensitiven Laufbands und Oberflächen-Elektromyographie konnte gezeigt werden, dass der Tremor während des Gehens persistiert mit einer zwischenzeitlichen Verlagerung der Tremorfrequenz in höhere Frequenzbereiche. Die Tremorintensität zeigte sich zudem abhängig vom Gangzyklus und der muskulären Belastung während des Gehens. Diese Beobachtungen legen nahe, dass es zu einer Interaktion zwischen dem orthostatischen Tremor und oszillatorischen, supraspinalen Lokomotionsarealen kommt und dass die periphere Manifestation des Tremors durch spinale Interneuron-Verschaltungen moduliert wird. Mittels einer multimodalen, klinisch-apparativen Ganguntersuchung wurden spatiotemporale Gangparameter bei Patienten mit orthostatischem Tremor erhoben, die mit einer altersgleichen, gesunden Kohorte verglichen wurde. Patienten mit orthostatischem Tremor zeigten ein breitbasiges Gangmuster mit erhöhter Gangvariabilität. Das Gangmuster verschlechterte sich beim langsamen Gehen und unter Augenschluss passend zu einer ataktischen Gangstörung mit sensorischen- und/oder zerebellären Defiziten. Eine weitere Aggravation in der kognitiven Dual Task-Bedingung offenbarte zudem motorisch-kognitive Defizite der Patienten. Somit scheint beim orthostatischen Tremor eine komplexe Netzwerkerkrankung mit einer spezifischen spino-zerebello-frontokortikalen Gangstörung vorzuliegen. Schließlich wurde in Folge einer kontinuierlichen Muskelsehnenvibrationsstimulation der unteren Extremitäten bei Patienten mit orthostatischem Tremor eine Reduktion der Tremorintensität und Körperschwankungen beobachtet. Bei bislang limitierten medikamentösen und invasiven Behandlungsmöglichkeiten bietet diese Beobachtung Hoffnung auf eine neue, nicht-invasive Therapieoption für Patienten mit orthostatischem Tremor.Primary orthostatic tremor is a rare disorder with high-frequency (13-18 Hz) leg muscle contractions during standing with relief of symptoms while sitting, walking, and lying. Recent studies found a specific ponto-cerebello-thalamo-cortical tremor network with persisting activity during lying. The aim of this dissertation was to answer following questions: 1) How does the tremor change in response to the transition from standing to walking? 2) Is orthostatic tremor associated with a specific gait disorder despite patients' sensation of symptom relief while walking? 3) Can the tremor be modulated through a non-invasive proprioceptive stimulation? In a first study, we examined the tremor of patients with orthostatic tremor during standing and walking conditions on a pressure-sensitive treadmill by means of surface electromyography of different leg muscles. We found that the tremor persisted during walking. Directly after gait initiation, the tremor frequency was shifted towards higher frequencies, but returned to the initial frequency after gait termination. While walking, the tremor was modulated in dependence of the gait cycle and individual exerted muscle forces. These observations point to a non-linear interference between the tremor and the oscillatory activity in supraspinal locomotor areas; furthermore, they indicate that the peripheral manifestation of the tremor is likely modulated by spinal interneuron connections. In a second study, we performed a multi-conditional, instrument-based gait assessment in patients with orthostatic tremor and age-matched healthy controls. Patients showed a broad-based walking pattern with increased gait variability with aggravation during slow walking modes and while walking with eyes closed. These gait alterations resemble an ataxic gait disorder indicative of sensory and/or cerebellar deficits. In addition, patients' walking performance deteriorated during a cognitive dual task paradigm pointing to a motor-cognitive dysfunction. Overall, the gait impairment in orthostatic tremor manifests in a specific spino-cerebello-frontocortical gait disorder. In a third study, we examined the effects of a proprioceptive leg muscle stimulation via muscle tendon vibration in patients with orthostatic tremor. We found that this stimulation yielded a reduction of tremor intensity and postural sway. In the light of currently limited pharmacological and invasive treatment options for orthostatic tremor, the observations of this study might pave the way for a new, non-invasive treatment option for patients

Rehabilitation for Multiple Sclerosis in Adults (I); Impairment and Impact on Functioning and Quality of Life: An Overview

Multiple sclerosis (MS) is a chronic, central nervous system, disabling disease. International Classification of Functioning and relevant generic and specific outcome measures are reported. Problems perceived by people with MS (PwMS) affect mobility, sight, continence, feeding, or cognitive impairment, depending on whether acute, chronic, or long-term disability was involved. The most common body function and structure impairments leading to disability and reported by health care professionals are fatigue, weakness, decreased fitness, sensory disorders, pain, upper motor neuron syndromes, ataxia and tremor, balance and postural control problems, gait pattern disorders, visual problems, and neurogenic lower urinary tract and bowel dysfunction; sexual, autonomic, neuropsychological, and neuropsychiatric impairment; dysarthrophonia, dysphagia, and respiratory and sleep disorders. The most frequently affected activities and relationships include mobility, domestic life, community and social activities, remunerative employment, interpersonal relationships, self-care, learning and applying knowledge, and economic life. Limitations in activities of daily life because of fatigue, pain, visual problems, incontinence, sexual and cognitive impairment, depressive disorders, sleep disorders, economic pressure, employment status, and lack of information have an impact on quality of life (QoL). Increased caregiving tasks, psychological burden, limitation in activities and participation, and reduced QoL have a profound influence on caregivers. This paper summarizes the perception of problems and needs, the disease's impact on functioning and QoL of PwMS, and the impact on their significant others and caregivers, according to health and social research.Martinez-Assucena, A.; Marnetoft, S.; Roig Rovira, T.; Hernandez-San-Miguel, J.; Bernabeu, M.; Martinell-Gispert-Sauch, M. (2010). Rehabilitation for Multiple Sclerosis in Adults (I); Impairment and Impact on Functioning and Quality of Life: An Overview. Critical Reviews in Physical and Rehabilitation Medicine. 22(1-4):103-178. doi:10.1615/CritRevPhysRehabilMed.v22.i1-4.90103178221-