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Chorea resulting from paraneoplastic striatal encephalitis.

T Tani1, Y Piao, S Mori

  • 1Department of Neurology, Saiseikai Niigata 2nd Hospital, Niigata, Japan.

Journal of Neurology, Neurosurgery, and Psychiatry
|September 16, 2000
PubMed
Summary

This study reports the first pathologically proven case of paraneoplastic chorea with striatal encephalitis. A 73-year-old man developed dementia and choreic movements linked to small cell lung cancer.

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Area of Science:

  • Neurology
  • Oncology
  • Immunology

Background:

  • Paraneoplastic syndromes can manifest with diverse neurological symptoms.
  • Chorea and dementia are less common presentations of autoimmune encephalitis.
  • Small cell lung cancer is a known paraneoplastic trigger.

Observation:

  • A 73-year-old male presented with progressive choreiform movements and cognitive decline.
  • Serum analysis revealed an antineuronal antibody targeting a 68 kDa protein, which stained neuronal cell bodies.
  • Autopsy confirmed small cell lung cancer and severe striatal neuronal loss with lymphocytic infiltration, predominantly in the caudate nucleus.

Findings:

  • The patient's neurological symptoms were attributed to an autoimmune response against neuronal antigens.

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  • The presence of specific antineuronal antibodies correlated with the observed neuropathology.
  • This case establishes a link between small cell lung cancer and a specific form of autoimmune encephalitis affecting the striatum.

    • Implications:

    • This case highlights the importance of considering paraneoplastic autoimmune encephalitis in patients with unexplained movement disorders and dementia.
    • Early diagnosis and treatment of the underlying malignancy may improve neurological outcomes.
    • Further research into the specific autoantigens involved could lead to targeted therapies for paraneoplastic chorea.