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Research paper
Non-demyelinating, reversible conduction failure in Fisher syndrome and related disorders
  1. Thirugnanam Umapathi1,
  2. Eyok Yian Tan1,
  3. Norito Kokubun2,
  4. Kamal Verma1,
  5. Nobuhiro Yuki3
  1. 1Department of Neurology, National Neuroscience Institute, Singapore, Singapore
  2. 2Department of Neurology, Dokkyo Medical University, Tochigi, Japan
  3. 3Department of Medicine, National University of Singapore, Singapore, Singapore
  1. Correspondence to Professor Nobuhiro Yuki, Department of Medicine, National University of Singapore, Unit 09-01 Center for Translational Medicine, 14 Medical Drive, Singapore 117599, Singapore; yuki.research{at}gmail.com

Abstract

Background IgG anti-GQ1b antibodies are associated with Fisher syndrome (FS), Bickerstaff brainstem encephalitis (BBE), acute ophthalmoparesis and overlap of FS or BBE with Guillain–Barré syndrome (GBS) (FS/GBS or BBE/GBS). It has not been clearly established if the primary pathology of these disorders is demyelinating or axonal in nature. Rapid resolution of conduction slowing or block without signs of demyelination–remyelination has been reported in axonal subtypes of GBS that are associated with IgG anti-GM1 or -GD1a antibodies. We hypothesised that such reversible conduction failure would be also observed in FS and related disorders.

Methods Serial nerve conduction studies were prospectively performed in 15 patients with FS and related conditions.

Results Neither conduction block nor abnormal temporal dispersion was observed in any of the nerves at any point in all the patients. Conduction velocities for none of the nerves were in the demyelinating range. The amplitude of sensory nerve action potential was decreased in three FS, one FS/GBS and two BBE/GBS patients. Compound muscle action potential amplitudes were decreased in the two BBE/GBS patients. These decreases in amplitudes of sensory nerve action potential and compound muscle action potential promptly resolved without significant change in duration on serial studies.

Conclusions Reversible conduction failure was seen in six of the 15 patients with FS and related disorders on serial nerve conduction studies. There were no signs of demyelination or remyelination in the 15 patients. The pathology appears to be primarily non-demyelinating. We believe these conditions form a continuous spectrum with axonal GBS.

  • Guillain–Barré syndrome
  • Fisher syndrome
  • anti-GQ1b antibody
  • neurophysiology
  • ganglioside
  • CIDP
  • Miller Fisher syndrome

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Footnotes

  • Funding This study was supported by the National Medical Research Council (IRG 10nov086 to N.Y.) and the Ministry of Health and Yong Loo School of Medicine Start-up Grant (N.Y.) in Singapore.

  • Competing interests None.

  • Patient consent Obtained.

  • Ethics approval Ethics approval was provided by the Institutional Review Board of National Neuroscience Institute, Singapore.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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