Acute bulbar, neck and limb weakness with monospecific anti-GT1a antibody: A rare localized subtype of Guillain-Barré syndrome

Muscle Nerve. 2016 Jan;53(1):143-6. doi: 10.1002/mus.24935. Epub 2015 Nov 23.

Abstract

Introduction: Acute bulbar, neck, and limb weakness carries several potential differential diagnoses. Although a diagnosis can often be established clinically, investigations such as electrodiagnostic and antibody testing can provide support for the clinical diagnosis and may aid in understanding the pathogenesis. A 65-year-old woman presented with acute bulbar, neck, and rapidly progressive bilateral upper limb weakness.

Methods: Clinical evaluation, electrophysiological, and serological studies were undertaken.

Results: Neurophysiology demonstrated proximal conduction block. A clinical diagnosis of pharyngeal-cervical-brachial weakness, a localized variant of Guillain-Barré syndrome, was made. The patient received treatment with intravenous immunoglobulin and made a remarkable recovery over the next month. She was found to have serum monospecific anti-GT1a antibodies.

Conclusions: We report a case of pharyngeal-cervical-brachial weakness with monospecific anti-GT1a antibodies and discuss the differential diagnosis of acute bulbar, neck, and limb weakness.

Keywords: Guillain-Barré; anti-ganglioside antibodies; clinical neurophysiology; immunology; pharyngeal-cervical-brachial weakness; syndrome.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Antibodies / blood*
  • Bulbar Palsy, Progressive / etiology*
  • Female
  • Gangliosides / immunology*
  • Guillain-Barre Syndrome / blood*
  • Guillain-Barre Syndrome / complications*
  • Humans
  • Immunoglobulin G / blood
  • Muscle Weakness / etiology*
  • Neck Muscles / physiopathology

Substances

  • Antibodies
  • Gangliosides
  • Immunoglobulin G
  • GT1aalpha ganglioside