SEPALS-2: Surface EMG as a tool to earmark effective therapies in ALS
Research type
Research Study
Full title
SEPALS-2: Surface Electromyography as a tool to earmark effective therapies in Patients with Amyotrophic Lateral Sclerosis (Disease Monitoring Workstream)
IRAS ID
359295
Contact name
James Bashford
Contact email
Sponsor organisation
Vice President (Research and Innovation)
Duration of Study in the UK
3 years, 8 months, 28 days
Research summary
Amyotrophic Lateral Sclerosis (ALS) is a progressive neurological disorder that causes the loss of motor nerve cells supplying muscles throughout the body. While new potential treatments are emerging, a major barrier to their evaluation is the lack of sensitive, non-invasive biomarkers to track disease progression.
Fasciculations—small, involuntary muscle twitches—are a hallmark of ALS and often appear before muscle weakness. This study will assess whether changes in fasciculation behaviour, recorded non-invasively using high-density surface electromyography (HDSEMG), can be used as a reliable biomarker of treatment response in ALS.We will recruit 30 individuals with a confirmed diagnosis of ALS caused by mutations in the superoxide dismutase 1 (SOD1) gene who are receiving tofersen, a targeted antisense therapy. HDSEMG involves placing a 64-electrode grid on the skin over specific muscles in the hand, arm, and leg to detect and quantify fasciculations. Participants will undergo assessments every three months in hospital, aligned with routine tofersen dosing visits, and complete weekly home-based recordings for one month following each hospital visit using a portable HDSEMG device.
Prior to the main study, a feasibility phase involving 4–6 newly diagnosed ALS patients and their caregivers will assess the usability and acceptability of the home recording setup. Feedback will be used to optimise the remote monitoring protocol.
The primary aim is to evaluate whether HDSEMG can detect meaningful changes in fasciculation patterns over time that may reflect how well the therapy is working. If successful, this technique could be adopted in future clinical trials as a sensitive, repeatable, and non-invasive method to assess disease progression and treatment efficacy in ALS. Its use in the home environment also offers the potential to reduce the burden of hospital visits and enhance patient-centred monitoring.REC name
London - Surrey Borders Research Ethics Committee
REC reference
25/PR/1215
Date of REC Opinion
26 Sep 2025
REC opinion
Further Information Favourable Opinion