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Paraneoplastic syndromes and autoimmune encephalitis: Five new things
Myrna R Rosenfeld1, Maarten J Titulaer, Josep Dalmau
1IDIBAPS and Service of Neurology (MRR, MJT, JD), Hospital Clinic, University of Barcelona, Barcelona, Spain; Department of Neurology (MRR, MJT, JD), University of Pennsylvania, Philadelphia; and Institució Catalana de Recerca i Estudis Avançats (ICREA) (JD), Barcelona, Spain.
This review covers new findings in paraneoplastic syndromes, focusing on cell surface antigen antibodies. It highlights diagnostic advancements and interpretation algorithms for these rare neurological disorders.
Area of Science:
- Neurology
- Immunology
- Oncology
Background:
- Paraneoplastic syndromes involve neurological disorders secondary to cancer.
- Antibodies targeting cell surface antigens are key diagnostic markers.
- Lambert-Eaton myasthenic syndrome is one such disorder.
Purpose of the Study:
- To review novel findings in paraneoplastic syndromes.
- To focus on antibody-associated disorders and diagnostic strategies.
- To provide an interpretation algorithm for antibody testing.
Main Methods:
- Literature review of recent studies on paraneoplastic syndromes.
- Analysis of novel antibody targets and associated clinical features.
- Discussion of diagnostic challenges and antibody testing interpretation.
Main Results:
- Anti-NMDAR encephalitis shows a distinct EEG pattern ('extreme delta brush') and metabolic gradient.
- GABA(B) receptor antibodies are linked to small-cell lung cancer (anti-Hu negative).
- mGluR5 antibodies associate with Hodgkin lymphoma (Ophelia syndrome).
Conclusions:
- Novel insights into paraneoplastic syndromes improve diagnostic accuracy.
- Understanding antibody targets is crucial for managing these immune-mediated disorders.
- Standardized interpretation of antibody tests aids clinical decision-making.
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