Search
limbic encephalitis
Etiology:
1) autimmune mediators:
a) intracellular autoantigens:
- anti-Hu, CV2/CRMP5, Ma2, & amphilysin
b) cell-surface autoantigens
- VGKC, NMDA receptor
2) paraneoplastic limbic encephalitis
3) Herpes simplex encephalitis
Epidemiology: rare
Pathology:
1) neuronal loss in medial temporal lobe & elsewhere in the limbic system
2) perivascular & meningeal lymphocytic infiltration
Clinical manifestations:
1) symptoms evolve over weeks
2) memory impairment, amnesia, confusion [1]
3) personality change
4) psychosis
5) encephalopathy
6) temporal lobe seizures
Laboratory:
- serum sodium may show hyponatremia [1]
- CSF analysis may show lymphocytic pleocytosis
Special laboratory:
- EEG may be abnormal
- case presentation with nonconvulsive status epilepticus [1]
Radiology:
- neuroimaging
- MRI may be abnormal in temporal lobe
- gadolinium enhancement of temporal lobe & hippocampus
Management:
1) treat empirically with intravenous acyclovir for Herpes simplex encephalitis until diagnosis is clarified [1]
2) intracellular autoantigen (+)
- generally refractory to therapy
3) cell-surface autoantigen (+)
a) removal of tumor
b) glucocorticoids
c) IV gamma-globulin
d) plasmapheresis
Interactions
disease interactions
Related
anti-Hu antibody (type-1 ANNA)
hippocampal 38K autoantigen
Specific
Herpes simplex encephalitis
paraneoplastic limbic encephalitis
General
encephalitis
References
- Medical Knowledge Self Assessment Program (MKSAP) 16, 18.
American College of Physicians, Philadelphia 2012, 2018
- Tuzun E & Dalmau J
Limbic encephalitis and variants: Classification, diagnosis
and treatment.
Neurologist 2007, 13:261
PMID: 17848866
- Shin YW, Lee ST, Shin JW et al
VGKC-complex/LGI1-antibody encephalitis: clinical manifestations
and response to immunotherapy.
J Neuroimmunol. 2013 Dec 15;265(1-2):75-81. Epub 2013 Oct 17.
PMID: 24176648