To investigate the potential value of magnetic resonance imaging (MRI) and diffusion tensor imaging (DTI) on a 3 Tesla and 7 Tesla MRI system and ultrasound (US) to visualize the peripheral nerves and muscles in the forearm to diagnose MMN and ALS…
ID
Source
Brief title
Condition
- Peripheral neuropathies
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
The main study parameters will be on the one hand qualitative in terms of
anatomy based on the anatomical MRI images (T1 and T2 sequences and 3D DTI
tractography) and ultrasound images, and on the other hand quantitative in
terms of diffusion parameters including the fractional anisotropy, mean
diffusivity, axial diffusivity and radial diffusivity and ultrasound
measurements including the cross sectional nerve and fascicle area, nerve
length and echogenicity. These results will be compared to the EMG. With EMG it
is possible to obtain information regarding the nerve and muscle conduction.
Potential differences in conduction will be compared to ultrasound and MRI
parameters.
Secondary outcome
NA
Background summary
Multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis (ALS) are
intriguing but rare disorders that can be clinical similar in presentation.
Both diseases are characterized by their progressive, multifocal, asymmetric
distribution of the nerves without any sensory involvement. However, as ALS is
a dramatic and fatal disease within several years, MMN has a more benign
disease course. In a later stadium, due to progressive axonal loss motor nerves
patients can be severely disabled. Motor deficit usually starts and remains
prominent in the distal arms. It is of extreme importance to distinct MMN from
ALS, but this can be very challenging. The hallmark of MMN is conduction block
(CB) on nerve conduction studies (NCS). However, these are not always found.
Earlier diagnosis will be beneficial as it permits the introduction of
therapies in an earlier stage of the disease. In MMN this means that treatment
with immunoglobulins (IVIg) can be started. Around 80% of the patients improve
significantly over longer time with repeated IVIg infusions. For ALS there is
no cure which completely stops the degeneration of the nerves. The disease is
progressive; the mean duration of survival is three to five years. Riluzole can
slightly reduce disease progression by several months. No other therapies are
available.
More diagnostic tools are urgently needed to distinct these diseases and start
treatment minimizing the time delay.
Study objective
To investigate the potential value of magnetic resonance imaging (MRI) and
diffusion tensor imaging (DTI) on a 3 Tesla and 7 Tesla MRI system and
ultrasound (US) to visualize the peripheral nerves and muscles in the forearm
to diagnose MMN and ALS and test its value to determine the disease progression
and to compare this with the gold standard electromyogram (EMG).
Study design
A prospective monocenter pilot study of 10 MMN patients, 10 ALS patients, and
10 healthy volunteers (time frame: 12 months)
Study burden and risks
Subjects will have a MRI scan during 2 scan sessions of each 25 minutes using a
3.0T and 7.0T MRI scanner (Philips Medical Systems, Best). There are no known
risks associated with MRI, beside temporary dizziness and claustrophobia. No
contrast is needed. The burden for the MRI scan is relatively low. The subjects
will obtain an ultrasound investigation of 20 minutes. There are no risks
associated with ultrasound. Furthermore, an EMG will be obtained. EMG can be
associated with an excitatory feeling and sometimes transient pain is
mentioned. EMG will not affect the nerves or muscles in any way. The
investigation takes approximately 45 minutes. There are no risks associated
with EMG. Previous to the EMG study the arm will be warmed up for about 30
minutes.
The results of this study may help to better diagnose both diseases and
distinct MMN from ALS in the future which can be beneficial for further therapy
and treatment plans of these patients.
Lundlaan 6
Utrecht 3508 AB
NL
Lundlaan 6
Utrecht 3508 AB
NL
Listed location countries
Age
Inclusion criteria
Patients with MMN
* Slowly progressive or stepwise progressive limb weakness
* Asymmetrical limb weakness
* Number of affected limb regions < 7. Limb regions are defined as upper arm, lower arm, upper leg, or lower leg on both sides
* Decreased or absent tendon reflexes in affected limbs
* Signs and symptoms are more pronounced in upper limbs than in lower limbs
* Age at onset of disease: 20*65 years;Patients with ALS
Inclusion criteria are based on the guidelines for diagnosis explained by [18]
* Evidence of lower motor neuron degeneration by clinical, electrophysiological or neuropathological examination
* Evidence of upper motor neuron degeneration by clinical examination
* Progressive spread of symptoms or signs within a region or to other regions, as determined by history or examination
* Patient should be between 20-65 years;Healthy controls
* Volunteers are healthy
* Volunteers are 18 year or older
* Volunteers are capable and prepared to sign an informed consent form
Exclusion criteria
Patients with MMN
* The patients should have no objective sensory abnormalities except for vibration sense
* The patients should have no bulbar signs or symptoms
* The patients should have no upper motor neuron features
* The patients should have no other neuropathies (eg, diabetic, lead, porphyric or vasculitic neuropathy; chronic inflammatory demyelinating polyneuropathy; Lyme neuroborreliosis; postradiation neuropathy; hereditary neuropathy with liability to pressure palsies; Charcot-Marie-Tooth neuropathies; meningeal carcinomatosis)
* The patients should have no myopathy (eg, facioscapulohumeral muscular dystrophy, inclusion body myositis);Patients with ALS
* Patients should not have other disease processes that might explain the signs of lower/upper motor neuron degeneration
* The patients should have no other neuropathies (eg, diabetic, lead, porphyric or vasculitic neuropathy; chronic inflammatory demyelinating polyneuropathy; Lyme neuroborreliosis; postradiation neuropathy; hereditary neuropathy with liability to pressure palsies; Charcot-Marie-Tooth neuropathies; meningeal carcinomatosis)
* The patients should have no myopathy (eg, facioscapulohumeral muscular dystrophy, inclusion body myositis);Healthy controls
* Volunteers with contra-indications for MRI (like a pacemaker, claustrophobia).
* Volunteers with known MMN, ALS or other neuropathy related disease
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 | NL49006.041.14 |