Acute behavioural change in a young woman evolving towards cerebellar syndrome

Clin Neurol Neurosurg. 2010 Jul;112(6):509-11. doi: 10.1016/j.clineuro.2010.03.002. Epub 2010 Mar 29.

Abstract

Symptomatic paraneoplastic neurological syndromes are rare manifestations of cancers. Recently, a new type of encephalitis associated with antibodies against NMDA-glutamate receptors (A-NMDAR) was defined. The patients, usually young women, present with acute onset of psychiatric symptoms and decreased consciousness. We describe the case of a patient who presented with acute onset of delirium alternating with sub-comatose state. Blood analyses were within normal range. Lumbar puncture showed lymphocytic pleiocytosis. Brain gadolinium injected MRI, brain and full body PET scans were normal. Investigations led to suspect a paraneoplastic syndrome and a right ovarian teratoma and A-NMDAR were found and the teratoma removed. The remaining sequellae included a cerebellar syndrome seldom described before. As cerebellar and cortical neurons share the same excitatory pathway through NMDA-glutamate receptors, the cerebellar function impairment observed in our patient could be explained by a disabling action on glutamate NMDAR by the A-NMDAR.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Autoantibodies / immunology*
  • Autoimmune Diseases / immunology*
  • Autoimmune Diseases / psychology*
  • Behavior
  • Brain Chemistry / physiology
  • Coma / etiology
  • Coma / psychology
  • Delirium / etiology
  • Delirium / psychology
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Ovarian Neoplasms / complications
  • Ovarian Neoplasms / surgery
  • Paraneoplastic Cerebellar Degeneration / etiology
  • Paraneoplastic Cerebellar Degeneration / psychology*
  • Positron-Emission Tomography
  • Psychomotor Agitation / psychology
  • Receptors, N-Methyl-D-Aspartate / immunology*
  • Teratoma / complications
  • Teratoma / surgery

Substances

  • Autoantibodies
  • Receptors, N-Methyl-D-Aspartate