Auto-immune limbische encefalitis
Open

Belang van tijdige diagnostiek en behandeling
Klinische les
12-04-2012
Judith van Vliet, Jan Meulstee, Erwin G.T.M. Hartong en Wim M. Mulleners

Autoimmune limbic encephalitis: importance of early diagnosis and treatment

Background

Autoimmune limbic encephalitis is a rare disorder, characterised by the subacute onset of seizures, short-term memory loss, and psychiatric and behavioural symptoms. Initially, it was recognised as a paraneoplastic disorder, but recently a subgroup of patients without systemic cancer was identified. This type of limbic encephalitis is associated with voltage-gated potassium channel (VGKC) or N-methyl-D-aspartate receptor (NMDAR) antibodies.

Case description

We describe a 69-year-old man with anti-VGKC limbic encephalitis suffering from generalised tonic-clonic seizures, severe insomnia, increasing memory deficits, visual hallucinations and depression. We also describe a 22-year-old woman, suffering from complex partial seizures and dysphasia, and displaying inappropriate behaviour. She was diagnosed with anti-NMDAR limbic encephalitis. Both showed marked improvement after starting prednisone and intravenous immunoglobulin therapy.

Conclusion

These case descriptions emphasise the importance of timely recognition of autoimmune limbic encephalitis in order to rule out malignancy and to quickly initiate treatment. This potentially life-threatening disease responds well to immunomodulatory therapy.

Conflict of interest: none declared. Financial support: none declared.