[Paraneoplastic neurological syndromes : A current summary].
1Klinisches Institut für Neurologie, Medizinische Universität Wien, Währinger Gürtel 18-20, 1090, Wien, Österreich. desiree.desimoni@meduniwien.ac.at.
Der Internist
|January 13, 2018
Summary
Paraneoplastic neurological syndromes (PNNS) involve immune responses to tumors, with new antibody-associated types showing better immunotherapy response. Early diagnosis of PNNS aids tumor detection and improves patient outcomes.
Area of Science:
- Neurology
- Immunology
- Oncology
Background:
- Paraneoplastic neurological syndromes (PNNS) arise from altered immune responses to tumors.
- Recent advances reveal PNNS associated with antibodies targeting surface or synaptic antigens.
- These novel PNNS often respond well to immunotherapy, unlike classical forms.
Purpose of the Study:
- To provide an updated overview of significant PNNS.
- Focus on syndromes linked to antibodies against onconeuronal, surface, and synaptic antigens.
- Highlight diagnostic and therapeutic implications.
Main Methods:
- Literature review of paraneoplastic neurological syndromes.
- Analysis of antibody targets (intracellular vs. surface/synaptic).
- Comparison of clinical presentation, tumor association, and treatment response.
Main Results:
- Classical PNNS involve onconeuronal antibodies against intracellular antigens, often preceding tumor detection with poorer prognosis.
- Autoimmunity against surface receptors can mimic classical PNNS but may not require a tumor trigger and shows good treatment response.
- Specific antibodies like anti-NMDAR define distinct syndromes (e.g., anti-NMDAR encephalitis), while others like GABA(B)R and AMPAR antibodies present overlapping clinical features.
Conclusions:
- PNNS diagnosis is crucial for early tumor detection and timely treatment initiation.
- Distinguishing between classical PNNS and surface receptor autoimmunity impacts prognosis and therapeutic strategies.
- Advancements in understanding antibody targets refine PNNS classification and management.
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