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Reversible limbic encephalitis caused by ovarian teratoma
K Nokura1, H Yamamoto, Y Okawara
1Department of Neurology, School of Medicine, Fujita Health University, Aichi, Japan.
Acta Neurologica Scandinavica
|June 1, 1997
Summary
A young woman experienced severe neurological symptoms due to an ovarian teratoma. Tumor removal led to recovery, suggesting a rare paraneoplastic syndrome.
Area of Science:
- Neurology
- Oncology
- Immunology
Background:
- Paraneoplastic syndromes can manifest with diverse and severe neurological deficits.
- Ovarian teratomas, though rare, are known oncological entities associated with paraneoplastic neurological disorders.
Observation:
- A 19-year-old woman presented with memory loss, psychosis, coma, and central hypoventilation.
- Cerebral MRI showed minimal changes, while SPECT revealed cortical uptake. Initial treatments with acyclovir and corticosteroids were ineffective.
- Elevated alpha-fetoprotein (AFP) and an ovarian tumor were identified.
Findings:
- Surgical resection of the immature teratoma resulted in significant cognitive recovery.
- Negative anti-neuronal antibody (anti-Hu) testing was noted.
- The suspected diagnosis was a rare combination of limbic and brainstem encephalitis, potentially due to immunologic cross-reactivity from the tumor's neuronal components.
Implications:
- This case highlights the importance of considering paraneoplastic syndromes in young patients with unexplained neurological deterioration.
- Immature teratomas should be investigated in cases of paraneoplastic encephalitis, even with negative specific antibody tests.
- The findings suggest a possible cell-mediated immune response in this paraneoplastic limbic and brainstem encephalitis.