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anti-leucine-rich glioma inactivated 1 (LGI1) encephalitis
Etiology:
- CSF antibody to leucine-rich glioma-inactivated protein 1 (LGI1)
Clinical manifestations:
- confusion, psychosis, agitation, delusions, hallucinations
- hyponatremia
- drug-resistant seizures
- may present as refractory temporal lobe epilepsy complicating convulsive status epilepticus
Laboratory:
- LGI1 Ab in CSF
- oligoclonal bands in CSF (variable)
- serum sodium: hyponatremia
Special laboratory:
- electroencephalography (EEG): temporal lobe seizures
Radiology:
- MRI neuroimaging
- temporal lobe hyperintensity
Complications:
- may be associated with cancer (10%), thymoma most common
Differential diagnosis:
- anti-NMDA receptor encephalitis
- Herpes simplex encephalilitis
Management:
1) empiric IV acyclovir for Herpes simplex encephalitis until diagnosis is clarified [1]
3) immunosuppressive therapy:
- combination of plasmapheresis, intravenous gamma-globulin & glucocorticoids [1]
- rituximab or cyclophosphamide for long-term therapy [1]
General
paraneoplastic limbic encephalitis
temporal lobe (psychomotor) epilepsy
References
- Medical Knowledge Self Assessment Program (MKSAP) 19
Board Basics. An Enhancement to MKSAP19.
American College of Physicians, Philadelphia 2022