Epileptic phenotypes, electroclinical features and clinical characteristics in 17 children with anti-NMDAR encephalitis.

BACKGROUND: Anti-N-methyl d-aspartate receptor (NMDAR) encephalitis is a rare disorder characterized by seizures, neuropsychiatric symptoms, dyskinesia and autonomic instability. OBJECTIVE: Aim of the present study was to evaluate the seizure phenotypes and electroencephalogram (EEG) features in children with anti-NMDAR encephalitis. METHODS: Seizure types, electroclinical features and clinical characteristics of 17 children with anti-NMDAR encephalitis were analysed in a retrospective case series from nine centres in Europe. RESULTS: Nearly half (8/17) of the children presented with psychiatric symptoms, whereas in 4/17 patients seizures were the first symptom and in 5/17 both symptoms occurred at the same time. During the following course seizures were reported in 16/17 children. The first EEG detected generalized slowing in 11/17 patients, focal slowing in 3/17 and normal background activity in only 3/17 children. The extreme delta brush (EDB) pattern was detected in 9/17 (53%) patients. CONCLUSION: In addition to psychiatric symptoms, children with anti-NMDAR encephalitis often show generalized slowing in EEG with or without seizures at initial presentation. EDB is present in half of all children and is potentially a helpful tool for early detection of this immune-mediated disease.

Accumulation of nuclear p53 protein and prognosis of astrocytomas in childhood and adolescence.

The aim of the study was to investigate whether nuclear immunopositivity for p53 is a factor of prognostic significance in astrocytomas of childhood and adolescence. Paraffin-embedded tissues of astrocytomas (10 WHO grade II, 11 WHO grade III) from 21 patients under 18 years of age, when operated for the first time, were studied immunohistochemically. We used a set of 6 different antibodies against p53, suitable for paraffin section (PAb1801, PAb240, DO-1, DO-7, BP53-12, CM-1). The intensity of the nuclear staining was scored and the percentage of stained nuclei counted. A tumor was scored positive if at least 10% of nuclei showed at least medium staining intensity with at least 1 primary antibody. No single antibody detected all cases designated immunopositive. This shows the advantage of using a set of antibodies. The time of survival in the immunopositive cases was significantly shorter, but immunopositivity was correlated with grade III (WHO). Comparing the time of survival between p53-immunopositive and -immunonegative grade III (WHO) astrocytomas no significant difference was found. We conclude that p53 immunoreactivity is not an independent unfavorable prognostic factor in astrocytomas of childhood and adolescence.