Ocular flutter, generalized myoclonus, and trunk ataxia associated with anti-GQ1b antibodies

Arch Neurol. 2008 May;65(5):659-61. doi: 10.1001/archneur.65.5.659.

Abstract

Objective: To describe a movement disorder characterized by ocular flutter, trunk ataxia, and mild generalized myoclonus associated with anti-GQ1b antibodies.

Design: Case report.

Setting: University hospital.

Patient: A 37-year-old woman presented with rapid, conjugated, and periodic oscillations of the eyes with a strict preponderance for the horizontal plane (ocular flutter); trunk ataxia; and occasional arrhythmic muscle jerks (myoclonus) most pronounced at the neck.

Results: Brain magnetic resonance imaging results were normal. Cerebrospinal fluid examination revealed mild lymphocytic pleocytosis. Results of extensive serological tests on viral, bacterial, and fungal infections from blood and cerebrospinal fluid samples were unremarkable. Results of screening examinations for neoplasms and paraneoplastic antibodies, including whole-body fludeoxyglucose F18 positron emission tomography, were normal. Positive titers of IgG and IgM anti-GQ1b antibodies were found.

Conclusions: This is the first description of an association between the clinical syndrome of ocular flutter, mild stimulus sensitive myoclonus, and trunk ataxia and anti-GQ1b antibodies. The association with ganglioside antibodies lends further support to the notion of an autoimmune-associated pathology of the syndrome.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Ataxia / blood
  • Ataxia / immunology*
  • Ataxia / physiopathology
  • Autoantibodies / blood*
  • Autoimmune Diseases of the Nervous System / diagnosis
  • Autoimmune Diseases of the Nervous System / immunology*
  • Autoimmune Diseases of the Nervous System / physiopathology
  • Brain / immunology
  • Brain / pathology
  • Brain / physiopathology
  • Female
  • Gangliosides / immunology*
  • Humans
  • Magnetic Resonance Imaging
  • Myoclonus / blood
  • Myoclonus / immunology*
  • Myoclonus / physiopathology
  • Ocular Motility Disorders / blood
  • Ocular Motility Disorders / immunology*
  • Ocular Motility Disorders / physiopathology

Substances

  • Autoantibodies
  • Gangliosides
  • GQ1b ganglioside