Anti-LGI1 encephalitis

Disease modifying treatment available:
Time critical diagnosis and management:
Lateralising:

Definition

None



Taxotomy

Aetiology

Antibodies against LGI1 are associated with neuronal hyperexcitability. Mutations in LGI1 have been associated with a genetic epilepsy - autosomal dominant lateral temporal lobe epilepsy.

The disorder usually occurs in older men. Less than 10% of patients have an underlying cancer. When a tumour is identified, thymoma is most common.



Associated Disorders




Clinical features


The disorder can present with an isolated rapidly progressive cognitive deficit in the absence of clear features of encephalitis.

Anti-LGI1 encephalitis may cause the following:




Treatment


As with other autoimmune encephalitides, management is centred around immunosuppression. Agents used include steroids, intravenous immunoglobulins and plasma exchange.




Research


Open questions

None







Supercategory
Autoimmune encephalitis