Serial Fasciculation Measurements in Motor Neurone Disease

Not Applicable

Completed

• Conditions: Benign Fasciculation-Cramp Syndrome; Motor Neuron Disease
• Interventions: Device: High-density surface electromyography

• Registration Number: NCT03809845
• Lead Sponsor: King's College Hospital NHS Trust

Brief Summary

Patients with motor neurone disease (MND) typically experience relentless motor decline and die within three years of symptom onset from respiratory muscle weakness. There are currently no effective therapies and the discovery of novel therapies is hampered by the lack of a sensitive disease biomarker. Consequently, there is a huge drive to discover novel biomarkers, which can reliably track disease progression over time. These can then be incorporated into clinical drug trials to expedite effective drug discovery.

Muscle fasciculations represent the hyperexcitability of diseased motor neurons and are almost universally present from the early stages of MND. The investigators predict that the site, frequency and shape of fasciculations might provide a sensitive measure of disease progression in an individual.

In order to calibrate this technique, the investigators will conduct a 12-month longitudinal study, recruiting 24 patients from the King's College Hospital Motor Nerve Clinic, comprising a mixture of patients with MND and those with benign fasciculation syndrome. Patients in this latter group have fasciculations but do not develop weakness and have normal lifespans. They are therefore an optimal control group. At each visit, the investigators will take resting HDSEMG recordings from all four limbs and perform standard clinical measures of disease progression. The investigators will also monitor the decline in motor unit number using a newly validated neurophysiological technique, called Motor Unit Number Index (MUNIX).

Detailed Description

Not available

Study Timeline

• Study Start: 2017-07-06
• Study First Submitted: 2018-03-13
• Study First Submitted QC: 2019-01-17
• Study First Posted: 2019-01-18
• Primary Completion: 2019-03-01
• Study Completion: 2019-03-01
• Status Verified: 2019-08
• Last Update Submitted: 2019-08-20
• Last Update Posted: 2019-08-21

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 25

Inclusion Criteria

Not provided

Exclusion Criteria

Not provided

Study & Design

• Study Type: INTERVENTIONAL
• Study Design: PARALLEL

Arm && Interventions

Group	Intervention	Description
Motor neuron disease	High-density surface electromyography	-
Benign fasciculation syndrome	High-density surface electromyography	-

Primary Outcome Measures

Name	Time	Method
Change in fasciculation frequency over time	12 months	To characterise the frequency of fasciculations in patients with motor neurone disease and to determine whether these parameters correlate with the trajectory of disease progression over a 12-month period.

Secondary Outcome Measures

Name	Time	Method
Change in Functional Rating Scale (FRS) over time	12 months	Maximum score of 48; lower scores indicate worse disability
Change in motor unit number index measurements over time	12 months
Change in MRC power sum score over time	12 months
Change in fasciculation morphology over time	12 months
Change in slow vital capacity over time	12 months

Trial Locations

Locations (1)

King's College Hospital NHS Foundation Trust; 🇬🇧 London, United Kingdom