Craniofacial Encephalocele: Updates on Management.

Craniofacial encephaloceles are rare, yet highly debilitating neuroanatomical abnormalities that result from herniation of neural tissue through a bony defect and can lead to death, cognitive delay, seizures, and issues integrating socially. The etiology of encephaloceles is still being investigated, with evidence pointing towards the Sonic Hedgehog pathway, Wnt signaling, glioma-associated oncogene (GLI) transcription factors, and G protein-coupled receptors within primary cilia as some of the major genetic regulators that can contribute to improper mesenchymal migration and neural tube closure. Consensus on the proper approach to treating craniofacial encephaloceles is confounded by the abundance of surgical techniques and parameters to consider when determining the optimal timing and course of intervention. Minimally invasive approaches to encephalocele and temporal seizure treatment have increasingly shown evidence of successful intervention. Recent evidence suggests that a single, two-stage operation utilizing neurosurgeons to remove the encephalocele and plastic surgeons to reconstruct the surrounding tissue can be successful in many patients. The HULA procedure (H = hard-tissue sealant, U = undermine and excise encephalocele, L = lower supraorbital bar, A = augment nasal dorsum) and endoscopic endonasal surgery using vascularized nasoseptal flaps have surfaced as less invasive and equally successful approaches to surgical correction, compared to traditional craniotomies. Temporal encephaloceles can be a causative factor in drug-resistant temporal seizures and there has been success in curing patients of these seizures by temporal lobectomy and amygdalohippocampectomy, but magnetic resonance-guided laser interstitial thermal therapy has been introduced as a minimally invasive method that has shown success as well. Some of the major concerns postoperatively include infection, cerebrospinal fluid (CSF) leakage, infringement of craniofacial development, elevated intracranial pressure, wound dehiscence, and developmental delay. Depending on the severity of encephalocele prior to surgery, the surgical approach taken, any postoperative complications, and the age of the patient, rehabilitation approaches may vary.

Long-Term Neuropsychological Outcomes Following Temporal Lobe Epilepsy Surgery: An Update of the Literature.

We present an update of the literature concerning long-term neuropsychological outcomes following surgery for refractory temporal lobe epilepsy (TLE). A thorough search was conducted through the PubMed and Medline electronic databases for studies investigating neuropsychological function in adult patients undergoing resective TLE surgery and followed for a mean/median > five years period. Two independent reviewers screened citations for eligibility and assessed relevant studies for the risk of bias. We found eleven studies fulfilling the above requirements. Cognitive function remained stable through long-term follow up despite immediate post-surgery decline; a negative relation between seizure control and memory impairment has emerged and a possible role of more selective surgery procedures is highlighted.

Emotional asymmetries in refractory medial temporal and frontal lobe epilepsy: Their impact on predicting lateralization and localization of seizures.

BACKGROUND: Emotional disturbances have been reported in patients with epilepsy. Although conflicting results emanate from relevant studies, depressive symptoms are seen more often in temporal lobe epilepsy (TLE) whereas, hypomanic/manic symptoms usually accompany frontal lobe epilepsy (FLE); the above psychiatric symptoms are especially seen in refractory epilepsy. However, neocortical TLE and medial TLE are considered as distinct epileptic syndromes, and there is limited literature on comparison of affective traits in medial TLE (MTLE) and FLE. AIM: In the present study, we sought to investigate affective traits among epilepsy surgery candidates suffering refractory left medial TLE (LMTLE), right medial TLE (RMTLE), left FLE (LFLE), and right FLE (RFLE). RESULTS: Our results revealed that patients with MTLE scored significantly higher than the ones with FLE in depression, anxiety, asthenia, and melancholia as measured by the Symptoms Rating Scale for Depression and Anxiety (SRSDA), while patients with FLE scored significantly higher in mania than those with MTLE. Moreover, patients with MTLE scored significantly higher than their FLE counterparts on the anxiety scale of the State Trait Personality Inventory (STPI)-trait version. When laterality of the seizure focus was taken into account, no differences were found among both patients with MTLE and patients with FLE, with exception for the Trail Making Test part B (TMT-B) in which patients with RMTLE performed significantly worse than patients with LMTLE. Seizure frequency was higher for FLE. CONCLUSIONS: We provide evidence for an anterior-frontal versus a posterior-medial temporal cerebral functional asymmetry with regard to the manifestation of manic and depressive emotional traits in FLE and MTLE, respectively. Our results are mainly discussed within the frame of their contribution in localizing and to a lesser extent in lateralizing seizures foci in epilepsy surgery candidates. We suggest that this is of great importance in the context of preoperative monitoring of epilepsy surgery, especially when neuropsychologists are called upon to provide anatomical information in defining the functional deficit zone.

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.

Comparison of Intracranial Ictal EEG Patterns between Medial and Lateral Temporal Lobe Epilepsy / 대한간질학회지

BACKGROUND AND OBJECTIVES: Temporal lobe epilepsy is the most common uncontrolled epileptic condition and is increasingly treated with surgery. In the absence of definitive results from noninvasive procedures, patients undergo implantation of intracranial electrodes. Intracranial EEG recordings are more accurate than scalp EEG recordings because of minimal artifact and closer approximation to the area of seizure onset. Intracranial EEG patterns between the medial and the lateral temporal lobe epilepsy were thought to have a little differences. METHODS: The authors compared the morphological pattern of seizure onset area, spread pattern, termination pattern and duration of the intracranial EEG manifestation of spontaneous seizures recorded from combined depth and subdural electrodes in 25 intractable temporal lobe epilepsy patients. RESULTS: Sixteen cases were medial temporal onset, six cases were neocortical onset and three cases were multifocal onset. The morphological onset pattern of medial temporal seizures was more likely to have high frequency rhythmic discharge (>13 Hz) and tended to show repetitive spikes prior to the seizure, whereas neocortical seizures were characterized by slow (4-to 10-Hz) and fast frequencies (>35 Hz), without evidence of repetitive spikes. The mean ictal duration at seizure onset of complex partial seizure of medial onset seizure was 121 seconds and was not different from those of neocortical seizures which were 115 seconds. Neocortical seizures take more time to propagate than medial seizures. Propagation to the opposite side of neocortical onset seizure takes 45.53 seconds, whereas medial temporal onset seizure takes 27.92 seconds. In case of second generalization, neocortical seizures continued longer than medial seizures. Of the medial onset temporal lobe seizures, except the simple partial seizures, the 35.2% of seizures initially spread to ipsilateral neocortex, and the 28.2% of seizures initially spread to the contralateral medial temporal lobe and the 25% of seizures simultaneously propagated to the ipsilateral neocortex and contralateral medial temporal lobe. Among the seizures initiated from the neocortex, 79.3% of seizures initially spread to the ipsilateral medial temporal area, but never initially spread to opposite neocortex. The termination pattern of seizures was divided into three types according to their location. In case of medial temporal lobe seizures, the mean of 31% of seizures was diffusely terminated , 38% of seizures terminated at the onset region and 38% of seizures were elsewhere within onset region. On the other hand, 48.6% of neocortical temporal lobe seizure were terminated at seizure onset region. CONCLUSIONS: The pattern of ictal intracranial EEG provides distinguishable differences between the medial temporal seizure and the neocortical temporal seizure.