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Paraneoplastic acute diffuse encephalitis revealing Hodgkin's disease
O Epaulard1, S Courby, P Pavese
1Service des Maladies Infectieuses et Tropicales DMAS, Centre Hospitalo-Universitaire de Grenoble BP 217, 38043, Grenoble Cedex 09 France. OEpaulard@chu-grenoble.fr
Leukemia & Lymphoma
|December 29, 2004
Summary
Acute encephalitis can be an early indicator of Hodgkin's disease (HD). This rare paraneoplastic neurological syndrome improved with chemotherapy and plasma exchange, alongside cancer regression.
Area of Science:
- Neurology
- Oncology
- Immunology
Background:
- Paraneoplastic neurological syndromes (PNS) are rare autoimmune disorders triggered by cancer.
- While cerebellar and limbic manifestations are documented with Hodgkin's disease (HD), diffuse encephalitis is exceptionally uncommon.
Observation:
- A patient presented with acute, severe diffuse encephalitis.
- Clinical, cerebrospinal fluid, and MRI findings were consistent with encephalitis.
- The encephalitis preceded the diagnosis of an undiagnosed extra-cerebral Hodgkin's disease.
Findings:
- Treatment involved chemotherapy targeting the Hodgkin's disease and plasma exchange for the neurological symptoms.
- Neurological status showed dramatic improvement within days of initiating treatment.
- Tumor regression was observed concurrently with neurological recovery.
Implications:
- This case highlights that encephalopathy can be the initial presenting sign of occult Hodgkin's disease.
- It underscores the importance of considering paraneoplastic syndromes in unexplained neurological disorders.
- Early diagnosis and combined treatment of the neoplasm and neurological syndrome are crucial for favorable outcomes.