This is a 48-week single arm study that incorporates digital tools for assessing motor
      function as part of an ALS telemonitoring program. During the study, neck- and wrist-worn
      "activity sensors" (PAMSys, BioSensics, Newton, MA) that will be worn by subjects while
      performing tasks of daily living. Subjects will also complete a motor, speech, and
      handwriting assessment during site visits. Subjects will complete a digital home assessments
      of speech, handwriting, and pattern tracing tasks throughout the study, and report any falls
      which occur on the study tablet. The investigators will explore whether functional changes
      are sensitive to self-reported changes on the ALS Functional Rating Scale - Revised (ALFRS-R)
      over the length of the study.

Digital Tools for Assessment of Motor Functions and Falls in ALS

Many of the recognized intrinsic factors for falls are commonly seen in ALS, including
      advanced age, muscle weakness, gait and balance problems, and previous falls. The American
      Academy of Neurology ALS Quality Standards Committee recommends querying patients for falls
      occurring in the past 12 months, indicating that prevention of falls is an important part of
      disease management. Despite this, the determinants and prevention of falls in this population
      is critically understudied.

      Among frail older adults, fallers (those who reported at least 1 fall in the last 6-months)
      spent more than twice as much time walking as non-fallers (those who reported no falls in the
      last 6-months). For these individuals, the best predictors of falling are measures related to
      activity exposure, such as time spent walking, average walking bout duration, or steps per
      day. Activity metrics of cadence variability, peak vertical acceleration variability, average
      duration of episodes of walking, frontal acceleration variability, average peak vertical
      acceleration, and average cadence have been shown to be associated with fall risk. During
      walking, individuals with ALS have increased and highly variable gait cycle time (time to
      complete a full walking cycle), along with reduced stride length with increased variability
      in stride length compared to healthy controls.

      The ability to observe changes in gait and posture has rapidly advanced around a revolution
      in mobile health technology. Inertial measurement units (IMUs), a standard inclusion of
      nearly all new smartphone/smartwatch devices, are small electronic chips that detect
      different aspects of inertial change, notably linear acceleration, angular velocity, and
      position relative to the magnetic field of the earth. Unlike their expansive use in movement
      disorders like Parkinson's disease, IMU-based gait assessment has been largely absent in ALS,
      despite the rapid changes to gait that may occur. The standard model for assessing and acting
      upon functional motor changes, including those impacting gait and falls, occurs roughly once
      every three months when patients are seen in the outpatient setting. Furthermore,
      self-reporting of falls has been shown to suffer from recall bias, resulting in low
      sensitivity and underreporting of fall events. In summary, patients with a rapidly
      progressing neurodegenerative disease, who in the standard care setting receive physical
      assessment motor function every three months, might stand to benefit from a home-based,
      objective biomarker for functional motor changes.

PAMSys Activity Monitoring System

Motor, Speech, and Handwriting Assessment

Changes in physical activity from baseline to 10 months will be assessed. Physical activity will be quantified by measuring number of taken steps per day and will be measured using a validated wearable sensor named PAMSys. Monitoring of physical activity will be done during 72 hours at baseline and 72 hours at 10 months.

Assistant Research Professor, Dept of Neurosurgery

Strength testing is performed by the attending neurologist at clinical appointments. Bilateral hip flexion, knee extension and flexion, and foot dorsiflexion strength will be documented according to Medical Research Council (MRC) grade 0 (no power) to 5 (normal power). Total score for bilateral muscle groups ranges from 0 to 40. Change in total lower limb strength score over study period will be calculated.

Strength testing is performed by the attending neurologist at clinical appointments. Bilateral deltoids, biceps, triceps, wrist extension, and interossei strength will be documented according to Medical Research Council (MRC) grade 0 (no power) to 5 (normal power). Total score for bilateral muscle groups ranges from 0 to 50. Change in total upper limb strength score over study period will be calculated.

Reflex testing is performed by the attending neurologist at clinical appointments. Bilateral biceps, triceps, and brachioradialis reflexes will be documented according to Modified Ashworth Scale grade 0 (no tone increase) to 4 (limb rigid). Total score for bilateral muscle groups ranges from 0 to 24. Change in total upper limb reflexes over study period will be calculated.

Reflex testing is performed by the attending neurologist at clinical appointments. Bilateral patellar and achilles reflexes will be documented according to Modified Ashworth Scale grade 0 (no tone increase) to 4 (limb rigid). Total score for bilateral muscle groups ranges from 0 to 16. Change in total lower limb reflexes over study period will be calculated.

Forced vital capacity (FVC) is collected at clinical appointments. FVC is expressed as a percentage (%) of predicted volume against an age, height, and ethnicity matched standard. Change in FVC over this time period will be calculated.

ALS Functional Rating Scale-Revised (ALSFRS-R) measures function in 12 domains. The total score ranges from 48 (normal function) to 0 (no function). Change in total score over this time period will be calculated.

ALS Functional Rating Scale-Revised (ALSFRS-R) gross motor subscore measures function in 3 domains. The total subscore ranges from 12 (normal function) to 0 (no function). Change in total subscore over this time period will be calculated.

ALS Functional Rating Scale-Revised (ALSFRS-R) fine motor subscore measures function in 3 domains. The total subscore ranges from 12 (normal function) to 0 (no function). Change in total subscore over this time period will be calculated.

The Falls Efficacy Scale - International (FES-I) is collected at study visits. The total score ranges from 64 (high concern about falling) to 0 (no concern about falling). Change in total score over this time period will be calculated.

The Fatigue Severity Scale is collected at study visits. The total score ranges from 63 (high fatigue) to 9 (low fatigue). Change in total score over this time period will be calculated.

The Neurological Fatigue Index - Motor Neuron Disease is collected at study visits. The weakness subscale total score ranges from 21 (high fatigue) to 7 (low fatigue). Change in total score over this time period will be calculated.

The Neurological Fatigue Index - Motor Neuron Disease is collected at study visits. The energy subscale total score ranges from 18 (high fatigue) to 6 (low fatigue). Change in total score over this time period will be calculated.

Subjects will complete the audio recording of a standard reading passage every two weeks. The speaking rate will be calculated from this audio, and the change in speaking rate over the study period will be calculated.

3-meter timed-up-and-go task is recorded at study visits. The best time of three trials is recorded. Change in best time over the study period will be calculated.

A 10-meter walk task is recorded at study visits. The best time of three trials is recorded. Change in best time over the study period will be calculated.

As part of the Handwriting Assessment Battery completed at study visits, subjects will copy a standard sentence from a prompt in the booklet. The change in writing speed for this copying task over the study period will be calculated.

As part of the Handwriting Assessment Battery (HAB) completed at study visits, subjects will print the alphabet in upper and lowercase, as well as the numbers 1-12. Legibility will be determined as the percent of individual characters rated as legible according to the HAB handbook. The change in character legibility over the study period will be calculated.

As part of the Digital Handwriting Assessment performed every two weeks on the study tablet, subjects will copy a standard sentence from a prompt on the tablet. The change in writing speed for this copying task over the study period will be calculated.

As part of the Digital Handwriting Assessment performed every two weeks on the study tablet, subjects will print the alphabet in upper and lowercase, as well as the numbers 1-12. Legibility will be determined as the percent of individual characters rated as legible according to the HAB handbook. The change in character legibility over the study period will be calculated.

As part of the Digital Handwriting Assessment performed every two weeks on the study tablet, subjects will be timed as they trace the outline of a spiral. The change in time to complete the tracing over the study period will be calculated.

Fall events (falls and near-falls) detected by the sensor are registered on the study tablet. The total number of fall events registered over the course of the study is a secondary outcome.

Subjects enter fall event reports in the study tablet. The total number of reports reported over the course of the study is a secondary outcome

Total number of assessments (handwriting, speech, and pattern tracing) completed on the tablet over the study period.

Total number of hours registered with wearing the fall sensor over the study period.

Total number of hours registered with wearing the activity sensors over the study period.

Digital Tools for Assessment of Motor Functions and Falls in ALS

About this trial

Study ID

Status

Type

Placebo

Accepting

Not accepting

Trial Timing

What is this trial about?

What are the participation requirements?

Locations

Location

Status