Electrical status epilepticus during sleep (ESES) in benign childhood epilepsy with Centrotemporal spikes (BCECTS): insights into predictive factors, and clinical and EEG outcomes.

OBJECTIVE: Benign childhood epilepsy with centro-temporal spikes (BCECTS), otherwise known as benign rolandic epilepsy, is the most common focal epilepsy in childhood. This study aimed to evaluate the development and resolution of ESES in children with BCECTS and evaluate the clinical and electroencephalography (EEG) parameters associated with prognosis. PATIENTS AND METHODS: Resolution of ESES was defined as the reduction of the spike-wave index (SWI) to <50%. The SWI short method, measurements from the first 180 s of non-rapid eye movement; and the conventional method, measurements from total NREM stage 2, SW count during the first 60 and 180 s of NREM, SW localization, and ESES type were determined. RESULTS: Of a total of 126 BCECTS patients, 33, including 13 females, 20 males, who developed ESES during follow-up, were included in the study. ESES remission was observed in 42.4% (n = 14) of the patients. The median time to remission was 10.5 months. The rate of resolution was 87.9 % for the entire population. The mean age at resolution was 9.8 ± 2.05 years and the mean time to resolution was 8.8 months. CONCLUSIONS: The data demonstrated that age at ESES diagnosis, the time between BCECTS diagnosis and the onset of ESES, time to resolution of ESES, ESES remission, and seizure freedom after ESES were significantly associated with prognosis. The early recognition of ESES evolution in children with BECTS, the better understanding of the relationship between age at ESES diagnosis and remission and prognosis, and timely intervention can prevent long-term sequelae.

Comparison of 2017 ILAE and Semiological seizure classifications before and after video-EEG monitoring in childhood epilepsy.

OBJECTIVE: Our aim in this study is to evaluate epilepsy classification in children with epilepsy before monitoring (based on information received from the family) and after monitoring (based on video-EEG) by comparing two separate classification systems, namely the 2017 International League Against Epilepsy (ILAE) and Semiological Seizure Classification (SSC) systems. Classifications and methods were compared in terms of simplicity, intelligibility, and applicability during daily outpatient care. PATIENTS AND METHODS: The study was performed with 230 recorded seizures of 173 patients aged between 1 and 18 years who underwent video-EEG monitoring and clinical seizure recordings. Seizure types and video-EEG data of the patients were recorded. Seizures were first classified based on information obtained from the parents of the patients in interviews, recorded as "before video-EEG monitoring classification," and a second characterization, based on video EEG-monitoring, was subsequently recorded as "after video-EEG monitoring classification". The consistency of both seizure classifications was evaluated. RESULTS: For both classifications, autonomic seizures were the least congruent seizures (κ=0.27, κ=-0.005). The families generally described the seizures very well; the consistency before and after video-EEG monitoring was good. Focal seizures with impaired awareness were most common in the 2017 ILAE classification (κ=0.6), while for the SSC simple motor seizures were most common (κ=0.84). Among subtypes, clonic-tonic seizures were the most common, and the second most common subtype was dialeptic (κ=0.67). Overall, the harmony between the SSC and ILAE systems was good. The rate of good and excellent coefficients of concordance for both the SSC and 2017 ILAE was determined as 77.8% for the expanded SSC, 48% for the 2017 ILAE, 71.4% for the basic SSC, and 60% for the 2017 ILAE. CONCLUSIONS: In practice, it is difficult to determine seizure patterns reliably in cases of childhood epilepsy. Parents, however, can generally describe seizures very well. Although the SSC seems to be superior, both the SSC and 2017 ILAE systems can be applied in daily use. Such classification enables the rise of new concepts and a better understanding of disease groups. The continuing development of classification systems will lead to advancements for patients.

COBALAMIN C DEFICIENCY WITH INFANTILE SPASM AND CUTANEOUS FINDINGS: A UNIQUE CASE.

Cobalamin C (CbIC) deficiency is a rare disorder of vitamin B12 metabolism which results from impaired conversion of both its active forms methylcobalamin and adenosylcobalamin. Early onset cblC typically presents in the first year of life with hypotonia, lethargy, seizures, microcephaly, hydrocephalus, developmental delay and other multisystem involvement including hematologic, ocular, renal, hepatic and cardiac symptoms. We report a case of a female infant with cblC deficiency who presented with seizures, developmental delay and hypopigmented cutaneous lesions. To our knowledge, the patient is the first diagnosed with cblC deficiency who had skin hypopigmentation.

Atypical presentations of subacute sclerosing panencephalitis in two neurologically handicapped cases.

Subacute sclerosing panencephalitis (SSPE) is a neurodegenerative disorder caused by persistent measles infection. Here, we report two neurologically handicapped cases presenting with atypical features of SSPE. Patient 1 who had mild mental retardation manifested acute encephalopathy with partial seizures and hemiplegia, mimicking encephalitis. He showed a fulminant course without myoclonia or a periodic electroencephalogram complex. Although SSPE is usually associated with an increased diffusion pattern, diffusion-weighted imaging of our patient showed decreased diffusion in the right hippocampus. Patient 2 with infantile hemiparesis presented with secondary generalized seizures, followed by asymettrical myoclonias involving the side contralateral to the hemiparesis. A periodic electroencephalogram complex was absent on the previously damaged brain regions. Our findings show that preexisting neurological disorders may modify the clinical or electrophysiological findings of SSPE, leading to atypical presentations. SSPE should be considered in the differential diagnosis of acute encephalopathy with lateralizing signs or unidentified seizures. Decreased diffusion resolution in diffusion-weighted-imaging may correlate with rapid clinical progression in SSPE.