Researchers identify septin autoantibodies in three people with motor-predominant ALS-like disease
Original source
Septin multimer autoantibodies in severe motor neuropathy mimicking lower motor neuron disease. (opens in a new tab)Compass summarised this from the study's abstract.
Study details
- Studied in
- Human, Mouse
A study identified septin multimer autoantibodies in three people diagnosed with the lower motor neuron variant of amyotrophic lateral sclerosis (ALS), among 3,543 samples tested. The antibodies targeted structures in peripheral nerves and were linked to inflammation, myelin damage and axonal damage in one patient. One patient’s disease stabilized after extensive immunotherapy, while two others died after rapid progression.
Why this matters
The finding suggests that some severe motor-predominant neuropathies that resemble lower motor neuron disease may have an autoimmune component. The researchers say septin autoantibody testing could be considered in people with this clinical pattern, but this study does not establish that the antibodies cause disease or that immunotherapy is effective.
Limitations and context
This was a retrospective study. Only three patients had the antibodies, and detailed tissue analysis and four-year treatment follow-up were reported for one patient. The study does not establish whether the antibodies cause disease, and larger studies are needed to assess their value as a biomarker and determine responses to immunotherapy.