Wearable Haptics and Immersive Virtual Reality Rehabilitation Training in Children With Neuromotor Impairments.

The past decade has seen the emergence of rehabilitation treatments using virtual reality (VR) environments although translation into clinical practice has been limited so far. In this paper, an immersive VR rehabilitation training system endowed with wearable haptics is proposed for children with neuromotor impairments: it aims to enhance involvement and engagement of patients, to provide congruent multi-sensory afferent feedback during motor exercises and to benefit from the flexibility of VR in adapting exercises to the patient's need. An experimental rehabilitation session conducted with children with cerebral palsy (CP) and developmental dyspraxia (DD) has been performed to evaluate the usability of the system and proof of concept trial of the proposed approach. We compared CP/DD performance with both typically developing children and adult control group. Results show the system was compliant with different levels of motor skills and allowed patients to complete the experimental rehabilitation session, with performance varying according to the expected motor abilities of different groups. Moreover, a kinematic assessmentbased on the presented system has been designed. Obtained results reflected different motor abilities of patients and participants, suggesting suitability of the proposed kinematic assessment as a motor function outcome.

Paciente con notalgia parestésica y siringomielia: ¿relación o coincidencia? / A patient with notalgia paresthetica and syringomyelia: association or coincidence?

La notalgia parestésica (NP) es una neuropatía sensitiva comúnmente manifestada con prurito y aparición de una mácula hiperpigmentada a nivel del raquis dorsal en la mayoría de los casos. La fisiopatología de la NP es aún desconocida, aunque se considera su origen, por distintas causas, en una lesión producida en los nervios espinales. No existe un tratamiento definitivo para este desorden aunque son muchas las alternativas terapéuticas descritas. Presentamos el caso clínico de una paciente diagnosticada de NP y tratada satisfactoriamente con capsaicina, en quien encontramos una posible asociación etiológica con una siringomielia subclínica evidenciada en el estudio por resonancia magnética. Aunque teóricamente posible, no hemos encontrado otros artículos que asocien dichos cuadros (AU)

Notalgia paresthetica (NP) is a sensory neuropathy commonly manifested by pruritus and the appearance of a hyperpigmented macula, usually in the thoracic spine. The physiopathology of NP is unknown, although, for different reasons, its origin is considered to be an injury to the spinal nerves. There is no definitive treatment for this disorder, although many therapeutic alternatives have been used. We report the case of a patient diagnosed with notalgia paresthetica and successfully treated with capsaicin. In this patient, we found a possible etiological association with subclinical syringomyelia revealed by magnetic resonance imaging. Although this association is theoretically possible, we have found no other reports of an association between these two disorders (AU)

Employment status of patients with neuromuscular diseases in relation to personal factors, fatigue and health status: a secondary analysis.

OBJECTIVE: To determine the number of employed people in a group of patients with neuromuscular diseases and in 3 separate subgroups (facioscapulo-humeral dystrophy, hereditary motor and sensory neuropathy, and myotonic dystrophy) to investigate any differences in employment status between the patient groups, and to identify factors related to employment status. DESIGN: Cross-sectional study. PATIENTS: A total of 591 patients with neuromuscular diseases participated in the study, 138 with facioscapulo-humeral dystrophy, 135 with hereditary motor and sensory neuropathy, and 318 with myotonic dystrophy. METHODS: Self-report questionnaires, the Checklist Individual Strength (CIS) and the Short Form-36 (SF-36). RESULTS: Of the patients with neuromuscular diseases in the study, 56.7% were employed. Younger age, being male, and higher education contributed significantly to employment status of the neuromuscular diseases group and the hereditary motor and sensory neuropathy and myotonic dystrophy subgroups. Significant between-group differences for employed vs not employed subjects were present in the total neuromuscular diseases group on all subscales of the CIS and SF-36. Factors related to employment status differed for the 3 neuromuscular diseases subgroups. CONCLUSION: More than half of the patients with neuromuscular diseases were employed. Patients with facioscapulo-humeral dystrophy and patients with hereditary motor and sensory neuropathy were more often employed than patients with myotonic dystrophy. Between-group analyses for differences in baseline factors revealed 11 significant factors related to employment. Multivariate logistic analyses revealed 6 factors contributing to employment for the group of patients with neuromuscular diseases.

Classification of employment factors according to the International Classification of Functioning, Disability and Health in patients with neuromuscular diseases: a systematic review.

PURPOSE: A systematic evaluation of the literature to identify health and contextual factors associated with employment in patients with neuromuscular diseases (NMD) and to perform a best evidence synthesis, taking into account the design of studies, methodological quality and the statistical significance of findings. METHOD: Publications were retrieved by a computerised search in medical and psychological databases. Two reviewers assessed titles and abstracts first and assessed the quality of the remaining full text publications independently as well. Of the residual publications, health and contextual factors associated with employment in patients with NMD were extracted. The factors found were included in a recently developed expanded International Classification of Functioning, Disability and Health scheme. RESULTS: Six hundred and sixty-two titles and abstracts were screened. The main reason to exclude a title and/or abstract was the absence of the study population selected: Facioscapulohumeral Muscular Dystrophy (FSHD), Hereditary Motor and Sensory Neuropathy (HMSN) & Myotonic Dystrophy (MD). Of the remaining 20 full-text publications, eight publications fulfilled the inclusion criteria: two repeated survey designs and six cross-sectional studies. Factor extraction resulted in 94 factors related to employment. Ten factors in five publications were indicative for an association with employment status: Disease related factors HMSN, MD & NMD in general), factors related to functions (physical functions, muscle power functions), general personal factors (age, gender and education), work related personal factors (type of occupation, and expressed interest in employment by patients with NMD). CONCLUSION: In the best evidence synthesis ten factors were indicative for an association with employment status in patients with NMD in five publications with good to excellent methodological quality.

Impacto de programa fisioterapêutico no desempenho funcional da criança com doença de Charcot-Marie-Tooth tipo 2: estudo de caso / Impact of a physical therapy program on the functional performance of a child with Charcot-Marie-Tooth disease type 2: a case report

O estudo visou verificar a influência de um programa fisioterapêutico no desempenho funcional de uma criança com diagnóstico de Doenca de Charcot-Marie-Tooth tipo 2. O participante (sexo masculino, 6 anos) apresentava comprometimento motor e sensitivo em membros superiores e inferiores...

This case study aimed at verifying then influence of a physical therapy program on the functional performance of a child with diagnosis of Charcot-Marie-Tooth disease type 2. The 6 year-old male child presented low and upper limbs affected at both motor and sensitive levels...

Ambulatory disabilities and the use of walking aids in patients with hereditary motor and sensory neuropathy type I (HMSN I).

PURPOSE: To determine the level of ambulatory disability and the use of walking aids in well-ambulant Hereditary Motor and Sensory Neuropathy type I (HMSN I) patients, and to identify the related demographic, physical and psychological variables. METHODS: Seventy-five well-ambulant HMSN I patients, aged 20-58 years, were measured in a cross-sectional assessment, addressing disability of ambulation and mobility (Sickness Impact Profile), demographics, muscle strength (Medical Research Council), use of walking aids, physical activity (actometer), fatigue (Checklist Individual Strength), and quality of life (EuroQoL). RESULTS: Seventy-two percent of the patients perceived a significant amount of ambulatory disability. These patients were less active, and more fatigued compared to patients without ambulatory disability, and healthy reference groups. The total patient sample showed marked distal paresis (mean MRC = 3.3), a high level of pain-discomfort (76%), but normal levels of employment (62.7%) and anxiety-depression (20%). Walking aids were used by 49% of the patients. These patients were older, less active, more fatigued, had less muscle strength, and perceived more disabilities of ambulation and mobility than non-users. Of the patients without walking aids, 41% perceived a significant amount of ambulatory disabilities. CONCLUSION: Ambulatory disability frequently occurred in well-ambulant HMSN I patients. The use of walking aids was not completely in accordance with the perceived ambulatory disability. Therefore prescription requires specific attention as well as complaints about pain and fatigue.

[Treatment of patients with neuromuscular disease in a warm climate]. / Behandling i varmt klima for pasienter med nevromuskulaere sykdommer.

BACKGROUND: Several patient groups request treatment in a warm climate, in spite of the fact that the effects of such treatment are undocumented. MATERIAL AND METHODS: 47 children and 40 adults with neuromuscular diseases were recruited, stratified according to sex, use or non-use of electric wheelchair, primary myopathy or hereditary neuropathy, and randomised into two adult and two children groups. The patients were treated in a rehabilitation centre, either on Lanzarote or in Norway. All patients were monitored with physical tests and questionnaires at the start of the study, at the end of the treatment period, after three months (all groups) and after six months (adults only). RESULTS: No significant differences in effect between the groups were found. In the warm climate, the adult patient group showed a statistically significant improvement regarding pain, quality of life, depression, and results of physical tests at the end of treatment. After three months, the improvement in physical tests was still present. Among adult patients treated in Norway, improvement in physical tests was statistically significant after three months, but not at the end of the treatment period. INTERPRETATION: This study did not show a statistically significant difference between patients with various neuromuscular diseases treated in a warm climate compared to similar patients treated in Norway.

Simulated work performance tasks in persons with neuropathic and myopathic weakness.

OBJECTIVES: To determine the test-retest reliability of selected simulated work performance tasks in persons with neuropathic and myopathic weakness and control subjects, and the association between muscular performance during these work tasks and conventional isolated muscle group testing. DESIGN: Measurement of three tasks performed on a work simulation device on two different days separated by 1 week. Associations between work task performance and previously reported strength measures in six muscle groups by hand-held dynamometry (HHD) were examined. SETTING: Human performance laboratory of a university. PARTICIPANTS: Convenience sample of ambulatory outpatients with hereditary motor and sensory neuropathy, type I (n = 9), myotonic muscular dystrophy (n = 10), and able-bodied controls (n = 11). MAIN OUTCOME MEASURES: For work simulation, isokinetic peak torque and total work; and for HHD, maximal isometric torque. RESULTS: Mean between-session differences for work simulation tasks ranged from -11% to +4% for peak torque and from -12% to +12% for total work; test sessions did not differ significantly for either patient or control groups. All groups had between-session intraclass correlation coefficients usually >.80, indicating good consistency. In general, correlations between peak torque during work simulation and HHD were strongest in the control group. CONCLUSION: Persons with neuromuscular weakness reliably performed the simulated work tasks examined in this investigation. Work simulation tasks may be a useful tool to assess muscular performance in persons with neuromuscular weakness.

Strength training in patients with myotonic dystrophy and hereditary motor and sensory neuropathy: a randomized clinical trial.

A randomized clinical trial on the effects of strength training was performed in myotonic dystrophy (MyD) patients and patients with hereditary motor and sensory neuropathy (HMSN). Training and most measurement tools involved the proximal lower extremity muscles. The participants trained 3 times a week for 24 weeks with weights adapted to their force. Strength was evaluated by isokinetically measured knee torque. Fatiguability was assessed by the time an isometric contraction could be sustained. Functional performance was measured by timed motor performance and by questionnaires on functional performance. Serum myoglobin (Mb) levels were determined to detect changes in muscle fiber membrane permeability. The MyD group included 33 participants, and the HMSN group included 29 participants. Within each diagnostic group, patients were individually matched and subsequently randomized for treatment allocation. In the MyD patients, none of the measurement techniques showed any training effect. Neither were there signs of deterioration caused by the training. In the HMSN group, knee torques increased. Timed motor performance did not change, although the questionnaires showed an improvement on items related to upper-leg function. Mb levels did not change significantly as a result of the training. In conclusion, the MyD group showed neither positive nor negative effects of the training protocol, whereas the training produced a moderate increase in strength and leg-related functional performance in the HMSN group.