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Are there immunologically treatable motor neuron diseases?
E Nobile-Orazio1, M Carpo, N Meucci
1Department of Neurological Sciences, University of Milan, IRCCS Ospedale Maggiore Policlinico, Italy. eduardo.nobile@unimi.it
Summary
Investigating immune system involvement in motor neuron disease (MND) and amyotrophic lateral sclerosis (ALS), this review examines if immune therapies are effective and safe for patients with these conditions.
Area of Science:
- Neurology
- Immunology
Background:
- Amyotrophic lateral sclerosis (ALS) and motor neuron disease (MND) pathogenesis may involve immune system dysfunction.
- Associations with cancer, monoclonal gammopathies, and neural antibodies suggest an immunological link.
- Multifocal motor neuropathy (MMN), a lower motor neuron syndrome, shows positive responses to immune therapies.
Purpose of the Study:
- To evaluate the evidence for immune system involvement in MND (dysimmune MND).
- To determine if immune or cytostatic therapies are justified for MND and lower motor neuron syndrome (LMNS) patients.
- To assess the safety and efficacy of these treatments.
Main Methods:
- Systematic review of existing studies on immunological aspects of MND/ALS.
- Analysis of reported responses to immune and cytostatic therapies in MND/LMNS patients.
- Evaluation of data regarding patients with associated conditions like cancer or monoclonal gammopathies.
Main Results:
- Some MND/ALS patients show occasional responses to immune/cytostatic treatments.
- Multifocal motor neuropathy (MMN) consistently responds to immune therapies.
- Evidence for widespread "dysimmune MND" requires further investigation.
Conclusions:
- The concept of "dysimmune MND" is plausible but requires more robust evidence.
- Immune and cytostatic therapies may benefit specific subgroups of MND/LMNS patients, particularly MMN.
- Careful consideration of potential harm from these therapies is essential.