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P492 Electroclinical features and seizure outcome of peadiatric patients with immune mediated encephalopathy

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Purpose Most patients with immune mediated encephalitis (IME) suffer from seizures, but data on the seizure outcomes in the peadiatric population remains limited. This study was conducted to assess the… Click to show full abstract

Purpose Most patients with immune mediated encephalitis (IME) suffer from seizures, but data on the seizure outcomes in the peadiatric population remains limited. This study was conducted to assess the clinical course and electroclinical features of peadiatric populations diagnosed with seizures associated with IME . Methods Total 15 patients with IME were identified from Cerrahpaşa Medical Faculty Peadiatric Neurology Department and Intensive Care Unit from 2014 to 2018. The demographic,clinical and electrographical data of participants were recorded. Symptoms were classified as psychiatric,seizure,flu-like symptoms,movement disorders and altered state of consciousness.Neuron spesific antibodies (LGI1, CASPR2, AMPAR1/2, NMDAR, GABABR) in CSF/serum, antibodies against GAD, thyroid peroxidase and paraneoplastic syndrome panel(anti-Hu, anti-Yo, anti-Ri, anti-Amphipysian, anti-Tr, anti-PCA-2, anti-Ma, anti-CV-2, anti-ANNA-3) were detected in the serum. Results The mean onset age of patients were 8,3 years (2–13,8 years). Female and male ratio was 8:7. In all patients admitted to the hospital the most common symptom was seizure(73%). The other symptoms were psychiatric(20%) and altered state of consciousness(7%). Seven(46%) patients with acute seizures present with status epilepticus. The mean hospitalization time of patients were 38,5 days (14–70 days) and 20 days(3–50) at Intensive Care Unit. Five patients were mechanically ventilated. Significant serum levels of antibodies were detected in 10 out of 15 patients.(GAD n = 8,antiTPO n=5) CASPR2 and NMDA in CSF were detected in different two patients. One patient diagnosed with FIRES.Oligoclonal banding or elevetad IgG index were detectected in 6 of 15 patients. Lymphocytic pleocytosis (40%) were detected in cerebrospinal fluid. All patients had abnormal electroencephalogram findings but 35.7% of patients had abnormal brain magneticresonance imaging findings at the time admission of the hospital. Immunotherapy,firstline therapy (IVIG n=14, pulse steroid n=13, plasma exchange n=7) and second line therapy (rituximab n=1, cyclophosphamide n=1) were contributed to treatment . After the discharge of the hospital no more seizure were seen in 12 patients (80%) during follow-up period. At the last visit all of these patiens had normal EEG findings. But in the other 3 patients epileptic activity was detected in their EEGs. Among them,only one patient was resistant to the antiepileptic treatment. Conclusions In this study, we observed that seizures with IME were generaly resistant to the treatment during the acute phase of the illness in pediatric population. But after proper and early immunotherapies, seizures remission was detected with good prognosis in most of them.

Keywords: anti anti; patients immune; anti; immune mediated; seizure; electroclinical features

Journal Title: Archives of Disease in Childhood
Year Published: 2019

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