Reinnervation and Neuromuscular Transmission in ALS
For patients and families
In plain language
An automatic summary of structured registry data. It is an orientation aid, not a substitute for the official protocol or a physician assessment.
- What is being studied
- The protocol lists: Observational study.
- Who it may be relevant to
- Registry conditions: Amyotrophic Lateral Sclerosis. Basic parameters: from 18 years · All.
- What needs checking
- Age, condition and sex are only basic indicators. Prior treatment, laboratory values and other mandatory requirements appear in the eligibility criteria below.
- Where it takes place
- Denmark
- Next step
- Save the trial, show it to the treating physician, and confirm current recruitment with the study center. Costs, documents and travel →
Unsure about the terms? Read our patient guide →
Official title
Reinnervation and Neuromuscular Transmission in Patients With Amyotrophic Lateral Sclerosis
Overview
The aim of this study is to describe the changes in the neuromuscular connection in patients with amyotrophic lateral sclerosis (ALS). The study consist of three substudies that have the following main hypothesis: 1. that ALS patients do not demonstrate equal capacity for muscle reinnervation and that reinnervation preserves muscle function and thereby slows down progression. 2. that blood concentrations of c-terminal agrin fragment (bCAF) reflect neuromuscular transmission deficiency and that blood concentration of neural cell adhesion molecule reflects degree of muscle denervation in patients. 3. that ALS patients with decrement when examined with repetitive nerve stimulation have more physical fatigue, slower progression, higher degree of reinnervation and higher bCAF compared to ALS patients without decrement. There will be 3 inclusion groups. 1. patients referred for neurophysiological examination on suspicion of motor neuron disease. 2. healthy controls 3. disease control: patients with another motor neuron disease with slow progression. All participants will be invited for at least 1 visit (baseline). If participants in group 1 eventually receive the diagnosis of ALS they will be invited for 2 additional visits 4 og 8 months after baseline visit, respectively. Examinations will consist of: * nerve conduction study * repetitive nerve stimulation (except for healthy controls) to examine impairment of the neuromuscular connection. * motor unit number estimation with MScanFit to estimate number and size of motor units. * ultrasound examination of muscles to measure size and condition of muscles. * questionnaires on fatigue and functional status. * blood sample for measurement of specialized analysis (c-terminal agrin fragment and neural cell adhesion molecule) and routine analysis (liver and kidney function as well as neurofilament light chain) * muscle strength assessment manually and by dynamometer to follow progression of muscle weakness * bioelectrical impedance measurement to follow the overall body composition.
Interventions
- Other Observational study
Observational study.
Primary outcome measures
- Reinnervation [Time frame: From baseline and 8 months]
- Blood biomarkers [Time frame: Baseline]
- Fatigue and decrement [Time frame: Baseline]
Eligibility criteria
Inclusion criteria
- Referred to clinical neurophysiological examination on suspicion of motor neuron disease or diagnosed with ALS according to Gold Coast criteria within the last 3 months.
- Age ≥18 years old
- Able and willing to provide informed consent
Exclusion criteria
- Former central or peripheral nervous system disease
- Diabetes
- Electrophysiological signs of polyneuropathy at baseline visit
- Pacemaker
- Pregnancy
For disease controls the exclusion criteria are the same, but the inclusion criteria:
- Diagnosed with disease with slow, progressive loss of motor neurons
- Age ≥18 years old
- Able and willing to provide informed consent
Criteria are shown verbatim from the registry (in English). Final eligibility is always assessed by the study center.
Healthy volunteers: Yes
Study design
- Observational model
- Cohort
Study locations
Denmark · 1 center
- Department of Neurology, Aarhus University Hospital — Aarhus
Identifiers
NCT: NCT06219759 · 8340 · 1-10-72-153-23