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Reversible acute axonal polyneuropathy associated with Wernicke-Korsakoff syndrome: impaired physiological nerve conduction due to thiamine deficiency?

Abstract

Acute axonal polyneuropathy and Wernicke-Korsakoff encephalopathy developed simultaneously in three patients. Nerve conduction studies (NCS) detected markedly decreased compound muscle action potentials (CMAPs) and sensory nerve action potentials (SNAPs) with minimal conduction slowing; sympathetic skin responses (SSRs) were also notably decreased. Sural nerve biopsies showed only mild axonal degeneration with scattered myelin ovoid formation. The symptoms of neuropathy lessened within two weeks after an intravenous thiamine infusion. CMAPs, SNAPs, and SSRs also increased considerably. We suggest that this is a new type of peripheral nerve impairment: physiological conduction failure with minimal conduction delay due to thiamine deficiency.

  • ATPase
  • neural conduction
  • polyneuropathy
  • thiamine
  • Wernicke encephalopathy
  • AMAN, acute motor axonal neuropathy
  • CMAP, compound muscle action potential
  • CSF, cerebrospinal fluid
  • EMG, electromyogram
  • GBS, Guillain-Barré syndrome
  • MRI, magnetic resonance imaging
  • NCS, nerve conduction study
  • NCV, nerve conduction velocity
  • NDS, neurological disability score
  • PDH, pyruvate dehydrogenase
  • PSW, positive sharp wave
  • SNAP, sensory nerve action potential
  • SSR, sympathetic skin response
  • TPN, total parenteral nutrition
  • TPP, thiamine pyrophosphate

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