Dados do Trabalho

Título

Anti-leucine-rich glioma-inactivated 1 encephalitis: a case serie

RESUMO

Case report: Three eldery patients, mean age of 67 years old, two women and one man, presented to our hospital clinic due to subacute faciobrachial dystonic seizures (FBDS) as main symptoms. FBDS were bilateral and high frequency (mean sixty episodes per day). One patient also had dystonic seizures in legs. Mild chorea was noticed in one woman concurrently. Executive dysfunction and short-term memory loss were noted in all of them, leading to rapidly progressive dementia phenotype in two. The man also showed visual hallucinations, behavior changes, depressed humor and hyponatremia (120 mmol/L), which prohibited the initial management of FBDS with carbamazepine (CBZ).
Electroencephalogram showed disorganized background activity with bifrontal slowing in both women. The Magnetic resonance images evinced white matter hyperintensities presumed of vascular origin in totality, besides hippocampal volume reduction in the woman with worse cognitive status. Search to occult neoplasm was negative to all. There were no cerebrospinal fluid abnormalities besides anti-LGI1 antibody positive, excepting the last patient whose testing is still in progress.
Metilprednisolone was the first line therapy to all, added to plasmapheresis (PLEX) in two. CBZ and levetiracetam associated with immunotherapy were essential to achieve FBDS full control in all patients in the mean period of 1 month. Micofenolato and prednisone were the choices for long term immunosuppression. Cognitive functions were improved in all cases.
Discussion: Anti-LGI1 encephalitis mainly affects eldery (median age 60 years) who present with seizures, cognitive and behavioral disorders. FBDS are very specific for the disease otherwise presenting in a minority of cases. Usually there is no EEG correlation with FBDS. Moreover, about 60-65% of patients develop hyponatremia, which is a clue to the diagnosis. Treatment consists in immunotherapy with corticosteroids, IV immunoglobulins or plasmapheresis.
Final comments: This case series adds knowledge of Brazilian and local epidemiology of this rare cause of rapidly progressive dementia, especially since patients presented with the classical clinical features of the disease had a great response to treatment. This reinforces the importance to recognize the clinical syndrome allowing for promptly request antibody testing and initiate the immunotherapy even before antibody results are available.

Palavras Chave

Anti-LGI1, encephalitis, autoimmune, dementia, seizure

Área

Neuroimunologia