Abstract
Objective: To report on dysphagia as initial sign in a case of anti-IgLON5 syndrome and provide an overview of the current literature.
Methods: The diagnostic workup included cerebral MRI, fiber optic endoscopic evaluation of swallowing (FEES) with the FEES tensilon test, a videofluoroscopic swallowing study, evoked potentials and peripheral nerve conduction studies, polysomnography, lumbar puncture, and screening for neural autoantibodies. A systematic review of all published cases of IgLON5 syndrome is provided.
Results: We report a case of anti-IgLON5 syndrome presenting with slowly progressive neurogenic dysphagia. FEES revealed severe neurogenic dysphagia and bilateral palsy of the vocal cords. Autoantibody screening was positive for IgLON5 IgG (+++, 1:1,000) serum levels but no other known neural autoantibody. Polysomnography was highly suggestive of non-REM parasomnia. Symptoms were partially responsive to immunotherapy.
Conclusions: Slowly progressive neurogenic dysphagia may occur as initial sign of anti-IgLON5 syndrome highlighting another clinical presentation of this rare disease.
GLOSSARY
- AASM=
- American Academy of Sleep Medicine;
- FEES=
- fiber optic endoscopic evaluation of swallowing;
- ICU=
- intensive care unit;
- Ig=
- immunoglobulin;
- NREM=
- non-REM;
- PSG=
- polysomnography
Footnotes
Funding information and disclosures are provided at the end of the article. Go to Neurology.org/nn for full disclosure forms. The Article Processing Charge was paid by the authors.
- Received July 11, 2016.
- Accepted in final form October 3, 2016.
- © 2016 American Academy of Neurology
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