To clarify the pathophysiological substrates of residual weakness and fatigue
ID
Source
Brief title
Condition
- Peripheral neuropathies
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
Amount of MU reduction and changed MU recruitment.
Secondary outcome
-
Background summary
Guillain-Barré syndrome (GBS) is a severe post-infectious polyneuropathy. Its
cardinal symptom is weakness. Recovery after GBS is usually slow and
incomplete. Despite current treatments, 20% of GBS patients have a considerable
residual handicap and most former patients experience weakness in addition to a
severe and disabling form of fatigue. Somewhat surprisingly, these complaints
are also common in clinically well recovered patients, who on conventional
electrophysiological testing have a normal outcome. Until now, the exact
mechanisms underlying the disabling fatigue and residual weakness have remained
unclear. Preliminary evidence from our group suggests that both may result from
reduced numbers of motor units (MUs). MUs are the functional units of the
peripheral motor system, comprising a single alpha-motoneuron and the 20-2000
muscle fibers that this neuron innervates. Loss of MUs implies that the same
amount of work (force generation) has to be performed with less MUs, with
obvious consequences for weakness and fatigability. An alternative hypothesis
is that reinnervation of denervated muscle fibers, a process that is inherent
in the disease and subsequent recovery, may render previously small MUs large.
Unintentional recruitment of these large MUs may then easily result in an
overshoot of force. It is conceivable that the subsequent and ongoing
compensatory mechanisms make extra demands on the muscle and on motor control
that result in increased fatigue.
Study objective
To clarify the pathophysiological substrates of residual weakness and fatigue
Study design
In this study, 30 severely fatigued and 30 mildly fatigued patients with or
without residual weakness will be recruited from our database of former GBS
patients. All patients will undergo various assessments in a single session of
approximately 3 hours: 1 hour for various scores, questionnaires, and a full
neurological exam, and two hours for neurophysiological tests, particularly
specialized electromyography (EMG) tests (collection of MU samples from
electrically elicited and voluntary contractions). Control values will be
collected in 30 healthy subjects.
Study burden and risks
The investigations are noninvasive. There are no risks, nor are there immediate
benefits for individual patients.
Department of Clinical Neurophysiology, Erasmus MC, University Medical Center, Postbus 2040
3000 CA Rotterdam
NL
Department of Clinical Neurophysiology, Erasmus MC, University Medical Center, Postbus 2040
3000 CA Rotterdam
NL
Listed location countries
Age
Inclusion criteria
*Age 18 years and older
*Residual fatigue after Guillain-Barré syndrome
*Written informed consent
Exclusion criteria
*severe other neurological or psychiatric disease
*Any psychological, familial, sociological and geographical condition potentially hampering compliance with the study protocol and follow-up schedule. Judgment is up to the investigator
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL16937.078.07 |