Dancing epilepsy: A stereo encephalography case report.

Ictal semiology is essential to identify the epileptogenic zone (EZ), especially in drug-resistant focal epilepsy (DRE), as its accurate identification determines the surgical prognosis. Dancing is highly unusual ictal semiology, and its underlying neural networks remain somehow unclear since both temporal and frontal lobe (FL) have been implicated in its generation. We present a 21-year-old male with DRE characterized by dancing seizures. Homemade videos were obtained. Through a non-invasive pre-surgical evaluation, the epileptogenic zone was localized within a gross lesion in the left FL. Using stereo electroencephalography (SEEG), we successfully identified the ictal-onset zone in the mesial middle, inferior, and orbito-frontal cortex, with rapid propagation of ictal activity extending backward and laterally to the precentral regions. Subsequently, a left frontal middle and inferior gyrectomy was performed, resulting in seizure freedom for the patient. Pathology results revealed a mild malformation of cortical development with oligodendroglial hyperplasia (MOGHE). Atypical seizure semiology, such as dancing, provides an interesting starting point for the analysis of the areas involved in the EZ. Further intracranial recordings are required to fully comprehend the underlying networks and interactions of cerebral areas during dancing seizures.

DEPDC5 mutation and familial focal epilepsy with variable foci: genotype and phenotype of a family.

Familial focal epilepsy with variable foci is a relatively rare autosomal disease with an unclear incidence, which is characterized by focal seizures arising from different cortical regions in different family members. We describe three members of a two-generation Argentine family with familial focal epilepsy with variable foci syndrome and a DEPDC5 gene mutation. The mean onset age was nine years old. The father experienced episodes with occipital semiology and both siblings exhibited frontal lobe seizures. Their neurological examination and neuroimaging studies were normal. All three patients are currently seizure-free, in spite of initially experiencing frequent seizures. Complete exome sequencing revealed a new DEPDC5 gene mutation (NM_001242896: c.4718T>C; p.L1573P). This study of a family with clinical characteristics that met all the criteria for familial focal epilepsy with variable foci demonstrates the usefulness of exome sequencing as a diagnostic tool. [Published with video sequence on www.epilepticdisorders.com].

Diferencias neurocognitivas entre las epilepsias parciales frontales y temporales en la infancia / Neurocognitive differences between frontal and temporal partial seizures in childhood

Introducción. Establecer las diferencias neurocognitivas entre las epilepsias pediátricas parciales del lóbulo frontal y del temporal. Métodos. En una investigación clínica de tipo transversal, descriptiva y prolectiva, se analizaron pacientes pediátricos con epilepsia parcial entre los 6 y 12 años de edad, de ambos sexos, en el Departamento de Neurología del Hospital Infantil de México Federico Gómez. Posterior a dividir a los pacientes en epilepsias parciales del lóbulo frontal y temporal, se aplicaron pruebas neurocognitivas, evaluación del cociente intelectual (CI), atención, memoria de trabajo, funciones ejecutivas y ejecución visuoespacial. Además, se evaluaron con resultados de electroencefalograma, neuroimagen y examen físico. Resultados. Se evaluaron 37 pacientes de ambos sexos (22 hombres, 15 mujeres) con epilepsia parcial del lóbulo frontal (17) y del lóbulo temporal (20). Las principales diferencias cognitivas entre estos dos tipos de epilepsia fueron: CI (promedio 82 en las epilepsias frontales y 97 en las epilepsias temporales) con mayor impacto en la memoria de trabajo y la ejecución vi-suoespacial en pequeños con epilepsia frontal. Los pacientes con epilepsia del lóbulo temporal presentaron mayores problemas en la atención de ejecución y de los test de memoria. Conclusiones. Los pacientes con epilepsia parcial del lóbulo frontal tienen mayor impacto sobre las habilidades neurocognitivas. Se considera muy importante esta evaluación con el fin de iniciar un soporte temprano con abordajes terapéuticos en este grupo de epilepsias, intentando revertir el impacto de crisis sobre las capacidades sociales y académicas.

Introduction. Objective: The purpose of this clinical trial was to differentiate the neurocognitive performance between frontal and temporal seizures in pediatric epilepsy. This is an important issue related to measure the impact of the different type of seizures in the neurodevelopment of children with epilepsy. Methods. We analyzed patients with partial epilepsy between 6 and 12 years old, both genders, in the Neurology Department of the Hospital Infantil de Mexico. After classifying frontal and temporal epilepsies, neurocognitive, IQ, attention, working memory, executive functions and visuospatial performance tests were applied. Likewise, EEG, neuroimaging, social evaluation and physical examination were performed. Results. Thirty-seven patients both genders (22 males; 15 females) with frontal partial seizures (17) and temporal partial seizures (20) were evaluated. The main neurocognitive differences between these 2 types of epilepsy were IQ (mean 82 in frontal epilepsy and 97 in temporal epilepsy) a higher impact on working memory and visuospatial performance was observed in infants with frontal epilepsy. The patients with temporal epilepsy had more problems in executing attention and long memory tests. Conclusion. Frontal partial seizures had more impact on the neurocognitive abilities than temporal partial seizures in the studied patients. This observation should be taken into account for the early treatment of children with epilepsy.