Abstracts

Rationale: Rasmussen encephalitis (RE) is rare inflammatory disease usually affecting one brain hemisphere characterized with typical trias: farmacoresistant epilepsy, hemiparesis and cognitive decline.The disease typically starts at the age 6-8 years, but there are known cases with delayed onset. 10% of RE start at the age 12-37 years (average 18,9years), the brain atrophy develops later, the clinical course is slowlier, occipital lobe dysfunction is most often the first manifestation of the disease, hemiparesis is usually less pronounced. Hemispherectomy due to intractable epilepsy in this group of patients might be problematic, because the hemiparesis is in many cases for a long time very subtle.Methods: We present three cases of late onset RE. The time from the first partial seizure to first epileptic state ( SE) lasted from 1 to 14 years. Two patients have got left sided and one patient right sided cerebral atrophy, all of them suffered from farmacoresistant seizures, CSF analysis excluded other type of cerebral infection. MRI and 18 FDG PET of the brain was compatible with the diagnosis of RE in all three cases.Results: All patients were treated with immunotherapy- all three patients started with high dose IVIG ( 0,4g/kg/d for 5 days) followed with maintenance phase 0,4g/kg/d every month. This therapy led to 3-year phase of seizure control and stopped progression of neurological deficit in all three pts. After the response to sole IVIG declined, all patients continued with IVIG plus corticosteroids every month. This therapy stabilized the disease course in two patients for 7and 5 years, one patient warsened rapidly in one year to last stage of the disease (severe hemiparesis, severe dysphasia and rare epileptic seizures). One of the responders continued after 5 years to the failure of this treatment (suffered repeated SE) we decided to introduce rituximab. Rituximab (CLL regimen) led to stabilisation patient has very rare epileptic seizures - for 3 month. The efficacy of this treatment for longer time has to be established, patient is followed up.Conclusions: Late onset RE is subset of RE patients with slowlier progression and maybe better response to immunotherapy than childhood onset RE. Long term stabilisation with immunomodulating therapies (IVIG, combined IVIG and corticosteroid treatment, monoclonal antibodies) might delay the need for hemispherectomy.