People With ALS Research Articles

Is There a Role for Exercise in the Management of Bulbar Dysfunction in Amyotrophic Lateral Sclerosis?

The role of exercise in the management of people with amyotrophic lateral sclerosis (PALS) is controversial and currently unclear. The purpose of this review article is to review literature examining the impact of limb, respiratory, and oral motor exercise on function, disease progression, and survival in PALS and the transgenic ALS animal model. A literature review was conducted to examine relevant studies published in peer-reviewed journals between 1960 and 2014. All studies were appraised for quality of research and were assigned a level of evidence, and treatment outcomes were classified as either positive, negative, or neutral. A total of 18 exercise-based intervention studies on limb (13), respiratory (3), or speech (2) function were identified. Of the human clinical trials, 6 were experimental and 4 were exploratory. No experimental studies were identified examining the impact of targeted exercise on speech or swallowing function. Mild to moderate intensity limb or respiratory exercise, applied early in the disease, was noted to have a beneficial impact on motor function and survival. Insufficient data exist to support or refute the role of exercise in the management of bulbar dysfunction in PALS. This represents a critical area of future investigation.

Eye tracking communication devices in amyotrophic lateral sclerosis: Impact on disability and quality of life

People with amyotrophic lateral sclerosis (PwALS) show progressive loss of voluntary muscle strength. In advanced disease, motor and phonatory impairments seriously hinder the patient's interpersonal communication. High-tech devices such as eye tracking communication devices (ETCDs) are used to aid communication in the later stages of ALS. We sought to evaluate the effect of ETCDs on patient disability, quality of life (QoL), and user satisfaction, in a group of 35 regular ETCD users in late-stage ALS with tetraplegia and anarthria. The following scales were administered: 1) the Individually Prioritized Problem Assessment (IPPA) scale, in three conditions: without device, with ETCD and, when applicable, with an Eye Transfer (ETRAN) board; 2) the Psychosocial Impact of Assistive Devices Scale (PIADS); and 3) the Quebec User Evaluation of Satisfaction with Assistive Technology (QUEST 2.0). With ETRAN, IPPA showed an increase in communicative abilities with respect to the condition without device, but ETCD produced a further significant increase. PIADS evidenced a large increase of QoL, and QUEST 2.0 showed high user satisfaction with ETCD use. In conclusion, ETCDs should be considered in late-stage ALS with tetraplegia and anarthria, since in these patients they can reduce communication disability and improve QoL.

Measuring function in advanced ALS: validation of ALSFRS‐EX extension items

With the aid of assistive technology, some patients with amyotrophic lateral sclerosis (ALS) are able to live for several years past the lowest measurable level of function on the Amyotrophic Lateral Sclerosis Functional Rating Scale - Revised (ALSFRS-R), a widely used end-point in ALS assessment. There is a research need to monitor patient function at the end of life, particularly in the face of severe impairment or 'locked in syndrome'. We used an online community for people with ALS (PALS) (PatientsLikeMe) to construct and pilot a number of new items to add to the ALSFRS-R scale to improve its sensitivity at lower levels of physical function in patients with advanced ALS. Ten new scale items were generated by a survey of PALS with advanced disease. These were added to the existing ALSFRS-R and data were received from 326 PALS at baseline with 169 PALS (52%) completing a 1-week test-retest and 218 PALS (67%) completing a 3-month retest [corrected]. Three new items were selected which conformed to the existing factor structure of the ALFRS-R. These relate to the ability to use fingers to manipulate devices, ability to show emotional expression in the face, and ability to get around inside the home. Real-world validation is the next step to assess the utility of the ALFRS-EX.