[Bickerstaff's syndrome presenting with coma, tetraplegia and blindness]

Rev Neurol (Paris). 2007 Feb;163(2):231-4. doi: 10.1016/s0035-3787(07)90394-x.
[Article in French]

Abstract

Introduction: Bickerstaff brainstem encephalitis is characterized by the occurrence of ataxia, ophthalmoplegia, motor weakness with areflexia and central nervous system symptoms, with drowsiness, pyramidal syndrome and sensorial symptoms. Diagnosis is based on MR findings and GQ1b antibodies. Treatment is not well known.

Observation: We report a patient aged 39 years native of Laos who presented weakness, loss of reflexes, and drowsiness. Brain MR showed hyperintense signals in the brain stem. GQ1b antibodies were positive. The course was characterized by decrease of the weakness, normalization of MR and negativity of GQ1b antibodies.

Discussion: This observation underlines common features of Bickerstaff brainstem encephalitis, Miller Fisher syndrome and Guillain Barre syndrome. A favorable course and GQ1b antibodies are shared by these syndromes.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Autoantibodies / blood*
  • Autoantibodies / immunology
  • Autoantigens / immunology*
  • Blindness / etiology*
  • Brain Stem / pathology
  • Coma / etiology*
  • Combined Modality Therapy
  • Demyelinating Autoimmune Diseases, CNS / complications
  • Demyelinating Autoimmune Diseases, CNS / diagnosis*
  • Demyelinating Autoimmune Diseases, CNS / immunology
  • Demyelinating Autoimmune Diseases, CNS / therapy
  • Electroencephalography
  • Encephalitis / complications
  • Encephalitis / diagnosis*
  • Encephalitis / immunology
  • Encephalitis / therapy
  • Female
  • Gangliosides / immunology*
  • Humans
  • Magnetic Resonance Imaging
  • Middle Aged
  • Optic Atrophy / etiology
  • Plasmapheresis
  • Quadriplegia / etiology*
  • Reflex, Abnormal
  • Syndrome

Substances

  • Adrenal Cortex Hormones
  • Autoantibodies
  • Autoantigens
  • Gangliosides
  • GQ1b ganglioside