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. 2016 Nov 22;4(1):e302.
doi: 10.1212/NXI.0000000000000302. eCollection 2017 Jan.

Isolated dysphagia as initial sign of anti-IgLON5 syndrome

Jens Burchard Schröder  1 , Nico Melzer  1 , Tobias Ruck  1 , Anna Heidbreder  1 , Ilka Kleffner  1 , Ralf Dittrich  1 , Paul Muhle  1 , Tobias Warnecke  1 , Rainer Dziewas  1
Affiliations

Isolated dysphagia as initial sign of anti-IgLON5 syndrome

Jens Burchard Schröder et al. Neurol Neuroimmunol Neuroinflamm. .

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.

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Figures

Figure
Figure. Investigations
(A) T1 gadolinium-enhanced axial MRI shows no evidence of underlying brainstem or cranial nerve pathology. (B) No intrathecal immunoglobulin synthesis was observed in CSF analysis using the Reiber scheme. (C) Fiber optic endoscopic evaluation of swallowing reveals postdeglutitive residue in the valleculae epiglotticae (3), on top of the epiglottis (2), and in the piriform sinuses (1).
See this image and copyright information in PMC

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