paraneoplastic limbic encephalitis
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Etiology
- mediators:
- intracellular autoantigens:
- cell-surface autoantigens
- CASPR2 (LG1), formerly thought to be VGKC
- NMDA receptor
- LBI1 protein
- associated neoplasms:
Epidemiology
rare
Pathology
- neuronal loss in medial temporal lobe & elsewhere in the limbic system
- perivascular & meningeal lymphocytic infiltration
Clinical manifestations
- evolution over weeks to months*
- patients frequently present with neurologic symptoms prior to diagnosis of malignancy
- acute or subacute mood & behavioral changes
- agitation
- confusional state
- memory loss with progession to dementia
- seizures[2]
- personality change
- psychosis
- oral dyskinesias
- SIADH (lung cancer, thymoma)
* distinguishing from Herpes simplex encephalitis
Laboratory
- anti-NMDA receptor antibody
- anti-AMPA receptor antibody
- anti-CASPR2
- anti-Hu
- anti-CRMP5
- anti-GABA B receptor
- voltage-gated potassium channel Ab in CSF[3]
- LGI1 Ab in CSF[3]
- CSF analysis may show lymphocytic pleocytosis
Diagnostic procedures
- EEG may be abnormal
Radiology
- MRI may be abnormal
Differential diagnosis
- Herpes simplex encephalitis evolves over days[3]
- anti-NMDA receptor encephalitis
- anti-leucine-rich glioma inactivated 1 encephalitis
Management
- treat empirically with intravenous acyclovir for Herpes simplex encephalitis until diagnosis is clarified[3]
- intracellular autoantigen (+)
- generally refractory to therapy
- cell-surface autoantigen (+)
- removal of tumor
- glucocorticoids
- IV gamma-globulin
- plasmapheresis
- evaluation/surveillance for systemic neoplasm may need to continue for years after initial presentation
More general terms
More specific terms
- anti-leucine-rich glioma inactivated 1 (LGI1) encephalitis
- anti-NMDA receptor encephalitis; NMDAR Ab encephalitis
Additional terms
References
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 624
- ↑ 2.0 2.1 Tuzun E & Dalmau J Limbic encephalitis and variants: Classification, diagnosis and treatment. Neurologist 2007, 13:261 PMID: https://www.ncbi.nlm.nih.gov/pubmed/17848866
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 19 American College of Physicians, Philadelphia 2012, 2015, 2021