Rehabilitation in primary lateral sclerosis mimicking parkinsonism: A case report.

BACKGROUND: Primary lateral sclerosis (PLS) is an upper motor neurons disease that on rare occasions may determine bradykinesia and motor fatigue. To date, no rehabilitative treatment has been described as useful for these patients. CASE PRESENTATION: A 68-year-old male developed dysarthria, spastic laugh, impairments of handwriting and fine motor, gait and dysphagia disorders for both solids and liquids over the period from 2015 to December 2018, with normal DaT scans and no clinical benefits from therapy with levodopa, pramipexole and baclofen. The patient underwent exercises for gait training and balance control with sensory treadmill and stabilometric platform and kinesiotherapy to improve fine motor skills of both hands and postural changes, five days a week for two weeks. Based on our data, the patient showed an improvement in balance and gait parameters in T2 compared to T1. CONCLUSION: Thanks to the synergistic action of a combined treatment of physical and instrumental therapy, despite the rare pathology and complex disability, the patient had important benefits in terms of performance and independence in daily activity.

Therapeutic Interventions to Improve Mobility with Spinal Cord Injury Related Upper Motor Neuron Syndromes.

Mobility is essential for quality of life and social participation. Some individuals with spinal cord injury have sufficient residual lower extremity motor control to walk. Improving walking function incorporates practice and training, and assistive devices or stimulation to augment function and balance. Overground robotic exoskeletons may have the potential to transform upright mobility in the future. Most individuals with spinal cord injury use a wheelchair for at least some of their mobility needs. Wheelchair skills training can open up new possibilities for participation. Regardless of the means of mobility, developing habits that protect joint health are essential for optimal lifelong mobility.

Recovery after traumatic thoracic- and lumbar spinal cord injury: the neurological level of injury matters.

STUDY DESIGN: Multicenter prospective cohort. OBJECTIVE: To discern neurological- and functional recovery in patients with a traumatic thoracic spinal cord injury (TSCI), conus medullaris syndrome (CMS), and cauda equina syndrome (CES). SETTING: Specialized spinal cord injury centers in Europe. METHOD: Lower extremity motor score (LEMS) and spinal cord independent measure (SCIM) scores from patients with traumatic TSCI, CMS, and CES were extracted from the EMSCI database. Scores from admittance and during rehabilitation at 1, 3, 6, and 12 months were compared. Linear mixed models were used to statistically analyse differences in outcome, which were corrected for the ASIA Impairment Scale (AIS) in the acute phase. RESULTS: Data from 1573 individuals were analysed. Except for the LEMS in patients with a CES AIS A, LEMS, and SCIM significantly improved over time for patients with a TSCI, CMS, and CES. Irrespectively of the AIS score, recovery in 12 months after trauma as measured by the LEMS showed a statistically significant difference between patients with a TSCI, CMS, and CES. Analysis of SCIM score showed no difference between patients with TSCI, CMS, or CES. CONCLUSION: Difference in recovery between patients with a traumatic paraplegia is based on neurological (motor) recovery. Regardless the ceiling effect in CES patients, patients with a mixed upper and lower motor neuron syndrome (CMS) showed a better recovery compared with patients with a upper motor neuron syndrome (TSCI). These findings enable stratifications of patients with paraplegia according to the level and severity of SCI.

[The Digital Gym in the neuromotor rehabilitation field]. / La Palestra Digitale in ambito neuromotorio.

SUMMARY: The digitization of processes has become a necessity to make health management remain sustainable. Current organizational models do not enable the increasing efficiency in process governance required by the increasing demand for rehabilitation services. Vice versa, new technologies have an innovative potential for compensation of disability and for operations management. This is context in which the concept of a "comprehensive digital rehabilitation" also said "digital gym" is developed: as a governance system for the whole inpatient rehabilitation path. The author describes the fundamentals of the Maugeri clinical model for a "Digital Gym" and examines some methodological, clinical and organizational issue concerning: information technology, machine operation protocols, clinical evaluation, machine operator interaction, patient doctor interaction, personalization of care, operational changes linked to the electronic workschedule, and the important training needs.

Design of Virtual Guiding Tasks With Haptic Feedback for Assessing the Wrist Motor Function of Patients With Upper Motor Neuron Lesions.

Impaired motor function is a common consequence of upper motor neuron lesions (UMNLs). Fine motor skills involved in small movements occurring in the fingers, hand, and wrist are usually regained by patient self-training at home. Most studies focus on the rehabilitation of the fingers but ignore the recovery of wrist motor function. In this paper, three virtual guiding tasks were designed to assess wrist motor functions, including the basic motor flexibility, motion stability, and a range of active motion. A haptic device was used to provide haptic feedback to users who performed virtual tasks in a virtual reality (VR) environment. In total, 46 healthy subjects and 10 UMNL patients were included to test the effectiveness of the designed tasks on improving wrist motor assessments. Quantitative performances, including the completion time, contact force, and motion trajectory, were automatically acquired during the tasks. Measurements for 95% of control subjects were used to establish normative references. Patient deficiencies in the wrist motor function were identified when their quantitative performances were outside the normative control ranges. The results suggest that the designed virtual tasks are sensitive for patients in the later period of rehabilitation, making the assessment suitable for using at home.

Adaptive equipment use by people with motor neuron disease in Australia: a prospective, observational consecutive cohort study.

PURPOSE: People with motor neuron sisease require adaptive equipment to enhance life quality. This study aimed to examine total and concurrent equipment items prescribed with phenotype consideration. METHODS: A prospective, observational consecutive cohort study was undertaken. Data regarding Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised, phenotype, symptom onset and Functional Independence Measure (inpatients only) was recorded. Equipment utilized was coded by therapist as: speech devices; transfer devices; mobility devices (including power wheelchairs); orthoses; activities of daily living equipment; assisted technology and home modification equipment. RESULTS: Two hundred and seventy-three people with motor neuron disease participated, mean age 67 years, mean amyotrophic Lateral Sclerosis Functional Rating Scale-Revised score 32, a moderate level of disability. Equipment items per participant ranged from 0 to 20, median 5. The electric lift recliner chair was the most commonly used equipment item, used by 51.2% of the cohort. There was a statistically significant difference in equipment use between flail leg and bulbar (Md 11, 3 items, respectively; p = .005), and flail leg and cervical phenotypes (Md 11, 3.5 items respectively; p = .009). CONCLUSIONS: People with motor neuron disease have high equipment needs to optimize quality of life. Information regarding phenotype relative to equipment requirement, and most frequently prescribed equipment items can assist health-care providers anticipate equipment needs, burden and intensity for those with motor neuron disease. Implications for rehabilitation People with motor neuron disease have high concurrent equipment needs. Electric recliner lift chairs are the most frequently prescribed equipment item by those with motor neuron disease. There is variation in concurrent equipment needs relative to motor neuron disease phenotype.

The evidence for prolonged muscle stretching in ankle joint management in upper motor neuron lesions: considerations for rehabilitation - a systematic review.

BACKGROUND: As clinicians, muscles stretching approaches are one of the most commonly used interventions in rehabilitation. However, there is a need for an in-depth evaluation of research on prolonged stretching in terms of the features of the stretching approaches, such as duration and frequency, as well as the compatible measures of a successful stretching approach. OBJECTIVE: This review is an effort to synthesize findings from studies on "prolonged" stretching approaches in patients with UMNs including stroke, spinal cord injuries, and traumatic brain injuries. We investigated the compatible features of successful stretching regimens in terms of reducing spasticity, improving the Active Range of Motion (AROM), Passive Range of Motion (PROM), and gait training of spastic patient with upper motor neuron lesions. METHODS: Studies evaluating the effectiveness of "prolonged" stretching on spastic ankle planterflexor muscles and its complications were critically reviewed, and the level of evidence was analyzed. RESULTS: There is a sufficient level of evidence to support the use of stretching as and effective techniques in rehabilitation. However, more research is yet to be done to objectively examine the ideal parameters of a successful stretching approach using functional assessments, such as walking, speed, walking capacity, and balance. CONCLUSION: The review adds stronger understanding with regard to stretching considerations in rehabilitation following UMNs. The ideal approach, as well as the functional implications on motor performance are yet to be further studied.

Patients' perspectives of multidisciplinary home-based e-Health service delivery for motor neurone disease.

Purpose: To explore the views of people with motor neurone disease (MND) on the barriers, facilitators and potential benefits of using home-based e-Health service delivery (telehealth) to access MND multidisciplinary clinic care. Methods: Twelve patients from three MND multidisciplinary clinics and an MND support association group completed a survey of information technology (IT) use and participated in interviews, to gather participants' experiences and perceptions of home-based telehealth for MND clinic care. Survey data were analyzed descriptively, with interview data analyzed using a stepwise inductive approach. Results: Surveys revealed that participants used IT to communicate with family and friends, but were less likely to use the phone, email or videoconferencing with health professionals. Two themes of participants' use of IT in MND care reflected their experiences of MND care; and personal preferences for modes of healthcare delivery. Participants were willing to use telehealth for MND care, with family members acting as patients' main support for telehealth participation. Nevertheless, participants preferred face-to-face contact with the MND clinic team in the initial and early stages of the disease. Conclusions: People living with MND may wish to participate in individual care planning to facilitate their access to a variety of e-Health service modalities. Additionally, individual care planning may allow healthcare professionals to deliver e-Health-based care, such as telehealth, to increase the scope of care provided. Research to ascertain the views of health professionals and family members as co-participants in service delivery via telehealth is needed to fully assess the potential contribution of e-Health. Implications for Rehabilitation People living with MND face a range of barriers to attending specialized multidisciplinary care, including fatigue, caregiver availability and logistical challenges to travel. Patients have indicated willingness to use e-Health applications to improve their access to care. Use of telehealth could expand service delivery to people with MND living long distances from multidisciplinary clinics, and increase the patient-centred focus of care by tailoring care planning. By offering telehealth services routinely, MND multidisciplinary clinics could also improve the quality and timelines of services offered.

Manual Feeding Device Experiences of People With a Neurodisability.

OBJECTIVE: Neurological bilateral upper limb weakness can result in self-feeding difficulties and reliance on care providers. Mealtimes become time consuming and frustrating. In this exploratory inquiry, we examined the experiences of users of a feeding device. METHOD: Semistructured interviews were either conducted by telephone or administered via email to explore quality of life, changes to independence, benefits and limitations, and psychological impact of the equipment. RESULTS: Thematic analysis gave rise to five themes: independence and positivity, emotions, impact on family and social life, equipment functionality, and motivation. CONCLUSION: This exploratory inquiry has contributed new qualitative evidence to the knowledge and understanding of users' experiences of a manual feeding device. Users reported that the need for assistance was reduced and that their quality of life, independence, and freedom improved. Time and resources savings for the family, care providers, and staff appeared to result in a more equal relationship between user and care provider.

Meaningful Assessment in Patients with Acquired Brain Injuries.

There are several key components to the meaningful and comprehensive assessment of patients with acquired brain injuries with respect to management of the upper motor neuron syndrome. Type of brain injury, trajectory of recovery, relevant concomitant complications, development of appropriate goals, and an understanding of resources available for patients are all factors to assess when developing a treatment plan. Using appropriate outcome measures will help monitor the efficacy of interventions and guide ongoing management of spasticity.

Muscle Overactivity in the Upper Motor Neuron Syndrome: Assessment and Problem Solving for Complex Cases: the Role of Physical and Occupational Therapy.

The role of the physical or occupational therapist in addressing muscle hyperexcitability is to carefully assess the implications that the abnormal tone has on function, especially active movement patterns. A thorough evaluation that includes neurologic and nonneurologic attributes allows the clinician to determine the most efficacious treatment interventions, especially when considering severity and chronicity of deficits. A holistic assessment that includes patient factors and resources guides the clinician's plan of care to allow for optimal functional outcomes.

Cuantificación de la espasticidad autopercibida. Revisión de escalas y cuestionarios / Quantification of self-perceived spasticty. Review of scales and questionnaires

A pesar de la gran cantidad de herramientas disponibles para evaluar la espasticidad, su fluctuación durante el día, la interferencia en la vida diaria y la falta de correlación entre los diferentes síntomas, fundamentan la cuantificación de la percepción del paciente de su propia espasticidad. Esta revisión pretende analizar los principales métodos de valoración de la espasticidad autopercibida por el paciente con enfermedad neurológica, descritos en la literatura científica, y realizar una descripción y análisis crítico de sus ventajas y limitaciones. Tras analizar las principales escalas de valoración de la espasticidad percibida, se concluye que existen pocas herramientas de cuantificación de la espasticidad que contemplen la percepción del paciente y, comparadas con las escalas de evaluación clínica tradicionales, han sido poco utilizadas en la literatura científica. Sin embargo, para su correcta valoración, es fundamental incluir al menos una medición que valore la autopercepción de espasticidad (AU)

Several tools are available to evaluate spasticity. However, because of factors such as fluctuation within the day, interference with daily activities and the absence of correlation among spasticity symptoms, there is a need for tools that measure self-perceived spasticity. This review aims to analyse the main methods for the evaluation of self-perceived spasticity by individuals with neurologic disorders and to discuss their advantages and disadvantages. Analysis of the main scales of self-perceived spasticity revealed that there are few spasticity measurement tools that include the patient's subjective point of view and that very few are used in the scientific literature compared with traditional clinical spasticity scales. However, for a comprehensive evaluation of spasticity, it is crucial to include at least one scale that assesses self-perceived spasticity (AU)

Active music therapy approach in amyotrophic lateral sclerosis: a randomized-controlled trial.

This randomized controlled study assessed the efficacy of active music therapy (AMT) on anxiety, depression, and quality of life in amyotrophic lateral sclerosis (ALS). Communication and relationship during AMT treatment were also evaluated. Thirty patients were assigned randomly to experimental [AMT plus standard of care (SC)] or control (SC) groups. AMT consisted of 12 sessions (three times a week), whereas the SC treatment was based on physical and speech rehabilitation sessions, occupational therapy, and psychological support. ALS Functional Rating Scale-Revised, Hospital Anxiety and Depression Scale, McGill Quality of Life Questionnaire, and Music Therapy Rating Scale were administered to assess functional, psychological, and music therapy outcomes. The AMT group improved significantly in McGill Quality of Life Questionnaire global scores (P=0.035) and showed a positive trend in nonverbal and sonorous-music relationship during the treatment. Further studies involving larger samples in a longer AMT intervention are needed to confirm the effectiveness of this approach in ALS.

Motoneuron diseases: impact on health professionals.

The approach to patients affected by motor neuron disease (MND) and their caregivers requires specific training for the care-team. In fact, the progression of the disease, with the decline of physical--and sometimes cognitive--function, the increasing difficulties in speaking, breathing, and swallowing and the need of invasive choices, as the artificial nutrition and tracheostomy, constitute a challenge for the health professionals, often generating distress. For this reason, their cohesion and sharing abilities are fundamental. Psychologist assumes a strategic role in supporting and facilitating the analysis of clinical cases and of the team's intra/interpersonal dynamics. For this aim, he/she needs specific training and instruments. We here present a semi-structured interview--the Motor Neuron Disease-Psychological Interview (MoNeDi-PI)--which may guide in the psychological assessment of patients affected by MNDs and their caregivers. It can also be a handy reference tool for other members of the healthcare team providing necessary information about the patient and caregiver in order to optimize clinical decision making about which health interventions to apply.

"It Gives Me My Freedom": Technology and Responding to Bodily Limitations in Motor Neuron Disease.

People living with motor neuron disease (MND) experience profound and rapidly progressing impairment. In order to maintain their physical and social functioning, people so affected employ a range of technologies and technological aids (body auxiliaries) to enhance their life and maintain well-being. Using a phenomenological study design, we explored the experiences of 42 men and women who had been diagnosed with MND. Although many participants initially resisted the adoption of aids (often-electronic devices that enabled continued participation in daily life) or tools (the instruments that allowed achievement of specific tasks), such technologies offered a way for people with MND to overcome, to some extent, the limitations posed by their physical degeneration. Through generating a sense of 'normality,' these kinds of 'enabling' technologies promoted social engagement and the maintenance of valued relationships or activities. Technologies can provide people with MND with some positive experiences within a way of being-in-the-world that has become so difficult and challenging.

Home Modifications and Ways of Living Well.

People living with a disability or illness and health care professionals often have different perspectives on what needs to be done, and why, in order to create a life they can recognize as good. Focusing on home modifications, I explore the enactment of diverging perspectives on the desired good. I show how one couple living with the effects of motor neuron disease in Wales tried to create a way of living. Drawing from a narrative-based study, I explore what happens when there is an interaction of different perspectives of what is considered to be a desirable outcome. I argue that the construction of some expectations as needs, and others as desires, serves to subjugate people to certain technologies. These technologies are those deemed necessary, following a neo-liberal language of cost-effectiveness where desires can be seen as liabilities.