Anti-LGI1 encephalitis (formerly limbic encephalitis with LGI1 antibodies) causes seizures and cognitive impairment without tumors.
Anti-LGI1 encephalitis:
- Usually non-paraneoplastic
- No associated tumor in most
- Good prognosis
- Distinctive seizure types
- LGI1 important for synapses
- CA1 dysfunction
- Memory impairment
- Seizure focus
- Emotional/memory integration
- Temporal lobe seizures
- FBDS (faciobrachial dystonic seizures)
- Autonomic involvement
- Diencephalic seizures
- Leucine-rich glioma inactivated 1
- AMPA receptor auxiliary subunit
- Synaptic transmission
¶ Antibody Effects
- Disrupts LGI1 function
- Alters AMPAR trafficking
- Seizure generation
- Very characteristic
- Brief dystonic events
- Often precede limbic encephalitis
- Typical limbic seizures
- Automatisms
- Secondary generalization
- Memory impairment
- Executive dysfunction
- Often improves with treatment
- Corticosteroids
- IVIG
- Often very effective
- Can occur
- May need maintenance
- Usually responsive
- Anti-LGI1 encephalitis (2022)
- FBDS and LGI1 (2021)