Childhood Absence Epilepsy with Coexisting Centrotemporal Spikes: Frequency, Electroclinical Course and Prognosis.

Components of generalized onset seizures with focal evolution progressing to bilateral tonic-clonic seizures using quantitative electroencephalography in children: Focal or generalized?

Absence seizures evolving to bilateral tonic-clonic seizures may have focal or asymmetric clinical features. Using quantitative electroencephalography (EEG) may evaluate this phenomenon and the involved neuronal network. We conducted a retrospective chart review of pediatric patients with generalized epilepsy and normal brain magnetic resonance imaging (MRI) admitted to an epilepsy monitoring unit. Our results show that seven of 66 patients with generalized epilepsy, all females, had generalized onset seizures with focal evolution (GOFE) to bilateral tonic-clonic seizures. Age at seizure onset was 7.5 years, at epilepsy syndrome diagnosis 8.14 years, and at GOFE diagnosis 13.04 years. Three had juvenile absence epilepsy, two had juvenile myoclonic epilepsy, and two had generalized genetic epilepsy. Eight seizures were recorded. Six patients had normal EEG background and one mild slowing. Quantitative EEG (QEEG) spectrogram with spike detection analysis showed 2.5-3.5-Hz generalized spike-wave activity followed by focal buildup over the lateral frontal, occipital, and frontal-central regions (right more than left) before bilateral tonic-clonic progression. In conclusion, in our cohort of patients with GOFE, QEEG analysis accurately showed that after absence seizure there is focal activation of the frontal (mainly right) and occipital cortices with evolution to bilateral tonic-clonic seizure. We hypothesize that GOFE could involve the activation of the thalamus and the cortex via the thalamocortical network, based on the QEEG analysis and the literature functional MRI evidence in absence seizures. Additional study of more patients to confirm findings is required.

Paroxysmal tonic upgaze mimicking epileptic seizures in infancy: a diagnostic challenge.

Clinical and neurophysiology study of 17 children with epileptic negative myoclonus

Epileptic negative myoclonus (ENM) is a clinically underrecognized seizure type, and systematic data on its clinical manifestations and optimal management strategies in pediatric populations remain scarce. This case series aimed to describe the clinical manifestations, neurophysiological patterns, and therapeutic outcomes in pediatric ENM. A retrospective descriptive analysis was conducted on 17 ENM patients diagnosed at Wuxi Children’s Hospital between October 2017 and June 2024. Clinical data, electroencephalogram (EEG) findings, and treatment responses were systematically evaluated. The cohort showed a marked female predominance (female-to-male ratio of 12:5). All cases exhibited upper-limb ENM, with contralateral central-parietal epileptiform discharges observed in ictal EEG. Two patients presented additional lower-limb ENM accompanied by midline discharges. Clinically, ENM manifested as the initial symptom in five cases (29.4%), including two with isolated ENM. Therapeutic outcomes varied: among four non-electrical status epilepticus during sleep (ESES) patients, antiseizure medications (ASMs) achieved symptom control, including one case of oxcarbazepine (OXC)-associated ENM that resolved after transition to valproate (VPA). Of 13 ESES-positive patients, three responded to ASMs alone, while nine received methylprednisolone pulse therapy (MPT), and one surgical candidate with focal cortical dysplasia (FCD) type IIa achieved post-resection remission. Among the nine children treated with MPT: four showed a good response; four relapsed upon steroid withdrawal; and one responded well to ketogenic diet (KD) therapy. ENM can manifest as the initial or sole epilepsy symptom in children. The marked female predominance in this cohort suggests that sex-related factors may play an important role in the expression or underlying mechanisms of ENM. Additionally, a correlation was found between the EEG discharge patterns and the specific limb regions involved. Regarding treatment, OXC may exacerbate ENM, necessitating cautious use. Steroid therapy should be considered for ESES cases unresponsive to ASMs. Refractory ENM with ESES warrants evaluation for structural abnormalities, particularly cortical dysplasia. Early EEG and neuroimaging improve diagnostic accuracy, while combined therapies-ASMs, immunomodulation, KD, or surgery-optimize outcomes. These findings highlight ENM’s diagnostic intricacy and the need for individualized management. Not applicable.

An autopsy study of a patient with refractory status epilepticus: MRI findings and histopathological correlation

The present case demonstrates that RSE with cortical involvement on MRI could be anatomically associated with CLN, which is accompanied by massive infiltration of plasma cells and T lymphocytes, which was seen in the overlying subarachnoid membrane and in the adjacent intact appearing parenchyma.

Behavioral and autonomic manifestations of suspected focal impaired awareness seizures supported by interictal electroencephalographic findings in three dogs: a case series

Focal epileptic seizures with sensory impairment can be difficult to recognize and are often mistaken for behavioral disorders. Electroencephalography (EEG) can provide important supportive evidence for their epileptic origin when interpreted in conjunction with clinical presentation. This article describes three cases of canine patients: a 2-year-old Border Terrier, a 1-year-old Poodle mix, and a 4-year-old mixed-breed dog. All three were presented with a history of recurrent episodes characterized by fear, restlessness, hyperactivity, hiding, staring into space or at the sky, lip licking, and hypersalivation. In two dogs, there were also signs of impaired consciousness. The frequency of the episodes varied, ranging from multiple episodes per day to one episode every 1–2 weeks. No pre-ictal or post-ictal signs were reported. Clinical and neurological examinations were unremarkable in all three cases. The diagnostic workup included EEG using a Natus Xltek system and magnetic resonance imaging (MRI) of the brain with a Siemens 1.5 T scanner. EEG monitoring lasting 1.5–4 h revealed epileptiform discharges predominantly in the parietal and temporal regions. This case series highlights the occurrence of behavioral and autonomic symptoms without typical motor manifestations as a presentation of presumed idiopathic epilepsy in dogs, supported by EEG findings in conjunction with the clinical presentation. All three dogs were treated with antiseizure medication, which resulted in a decrease in both the frequency and intensity of episodes, with complete seizure freedom achieved in two dogs.

Electroclinical and neuroradiological evolution in pediatric and adult patients with Rasmussen encephalitis.

PURPOSE Rasmussen encephalitis (RE) is a rare progressive inflammatory disorder characterized by drug-resistant focal epilepsy and unilateral cerebral atrophy. This study aimed to evaluate the longitudinal electroclinical, neuroradiological, treatment, and surgical characteristics of pediatric and adult patients with RE followed at a tertiary referral center. METHODS Fourteen patients (10 females) with a median follow-up of 75.5 months were retrospectively reviewed. Clinical characteristics, serial electroencephalography (EEG), brain magnetic resonance imaging (MRI), immunotherapy regimens, surgical interventions, and seizure outcomes were analyzed. RESULTS Median age at symptom onset was 102 months. Focal motor seizures were the initial presenting symptom in 86% of patients, and epilepsia partialis continua developed in 57%. Serial EEG demonstrated progression from focal epileptiform abnormalities to regional and hemispheric slowing confined to the affected hemisphere. Left-hemispheric involvement was observed in 71% of patients in this relatively older cohort. Initial MRI frequently demonstrated cortico-subcortical T2-weighted and fluid-attenuated inversion recovery (FLAIR) hyperintensities preceding progressive cortical atrophy. Serial MRI revealed heterogeneous radiological evolution, including diffuse hemispheric and limited regional cortical atrophy patterns. Despite immunotherapy, most patients showed progressive disease and refractory seizures. Hemispherectomy resulted in seizure freedom in two of three operated patients. CONCLUSION RE demonstrated heterogeneous electroclinical and neuroradiological evolution across pediatric and adult patients. The coexistence of an older age profile and a predominance of left hemisphere involvement may suggest age-related phenotypic variability. Early MRI abnormalities may precede overt hemispheric atrophy, highlighting the importance of longitudinal evaluation for timely diagnosis. Although immunotherapy may provide temporary stabilization, surgical treatment appears to provide the most favorable seizure outcomes in selected patients.