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Acute behavioural change in a young woman evolving towards cerebellar syndrome

G Naeije1, Q de Hemptinne, C Depondt

  • 1Department of Neurology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium.

Clinical Neurology and Neurosurgery
|March 30, 2010
PubMed

Insights

Antibodies against NMDA-glutamate receptors (A-NMDAR) can cause rare paraneoplastic neurological syndromes. This case highlights A-NMDAR encephalitis presenting with a unique cerebellar syndrome in a young woman.

Area of Science:

  • Neurology
  • Immunology
  • Oncology

Background:

  • Paraneoplastic neurological syndromes are rare cancer manifestations.
  • Anti-NMDA receptor (A-NMDAR) encephalitis, often affecting young women, presents with psychiatric symptoms and altered consciousness.
  • This case explores a rare presentation and sequelae of A-NMDAR encephalitis.

Observation:

  • A young patient presented with acute delirium and a sub-comatose state.
  • Initial blood tests were normal, but cerebrospinal fluid showed lymphocytic pleocytosis.
  • Brain imaging (MRI, PET) was unremarkable, prompting investigation for paraneoplastic causes.

Findings:

  • A right ovarian teratoma and A-NMDAR antibodies were identified.
  • Surgical removal of the teratoma was performed.
  • The patient developed a cerebellar syndrome, a less common sequela.

Implications:

  • The cerebellar syndrome may result from A-NMDAR's disabling effect on NMDA-glutamate receptors shared by cortical and cerebellar neurons.
  • This case expands the understanding of A-NMDAR encephalitis presentations and its neurological manifestations.
  • Early diagnosis and treatment, including tumor removal, are crucial for managing A-NMDAR encephalitis.

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