Semiautomated electric source imaging determines epileptogenicity of encephaloceles in temporal lobe epilepsy.

OBJECTIVE: We aimed to assess the ability of semiautomated electric source imaging (ESI) from long-term video-electroencephalographic (EEG) monitoring (LTM) to determine the epileptogenicity of temporopolar encephaloceles (TEs) in patients with temporal lobe epilepsy. METHODS: We conducted a retrospective study involving 32 temporal lobe epilepsy patients with TEs as potentially epileptogenic lesions in structural magnetic resonance imaging scans. Findings were validated through invasive intracerebral stereo-EEG in six of 32 patients and postsurgical outcome after tailored resection of the TE in 17 of 32 patients. LTM (mean duration = 6 days) was performed using the 10/20 system with additional T1/T2 for all patients and sphenoidal electrodes in 23 of 32 patients. Semiautomated detection and clustering of interictal epileptiform discharges (IEDs) were carried out to create IED types. ESI was performed on the averages of the two most frequent IED types per patient, utilizing individual head models, and two independent inverse methods (sLORETA [standardized low-resolution brain electromagnetic tomography], MUSIC [multiple signal classification]). ESI maxima concordance and propagation in spatial relation to TEs were quantified for sources with good signal quality (signal-to-noise ratio > 2, explained signal > 60%). RESULTS: ESI maxima correctly colocalized with a TE in 20 of 32 patients (62.5%) either at the onset or half-rising flank of at least one IED type per patient. ESI maxima showed propagation from the temporal pole to other temporal or extratemporal regions in 14 of 32 patients (44%), confirming propagation originating in the area of the TE. The findings from both inverse methods validated each other in 14 of 20 patients (70%), and sphenoidal electrodes exhibited the highest signal amplitudes in 17 of 23 patients (74%). The concordance of ESI with the TE predicted a seizure-free postsurgical outcome (Engel I vs. >I) with a diagnostic odds ratio of 2.1. SIGNIFICANCE: Semiautomated ESI from LTM often successfully identifies the epileptogenicity of TEs and the IED onset zone within the area of the TEs. Additionally, it shows potential predictive power for postsurgical outcomes in these patients.

Neurosurgical intervention for the Meckel-Gruber Syndrome: A systematic review.

INTRODUCTION: Meckel-Gruber Syndrome (MKS) is an autosomal recessive genetic disorder, notable for its triad of occipital encephalocele, polycystic renal dysplasia, and postaxial polydactyly. Identified by Johann Friederich Meckel in 1822, MKS is categorized as a ciliopathy due to gene mutations. Diagnosis is confirmed by the presence of at least two key features. The condition is incompatible with life, leading to death in the womb or shortly after birth. Recent studies have largely focused on the genetic aspects of MKS, with limited information regarding the impact of neurosurgical approaches, particularly in treating encephaloceles. METHODS: A systematic review was performed according to the PRISMA statement. The PubMed, Embase, and Web of Science databases were consulted for data screening and extraction, which was conducted by two independent reviewers. The search strategy aimed to encompass studies documenting cases of MKS with published reports of encephalocele excisions, and the search strings for all databases were: Meckel-Gruber syndrome OR Meckel Gruber syndrome OR Meckel-gruber OR Meckel Gruber. RESULTS: The study included 10 newborns with MKS associated with occipital encephalocele or meningocele, all of whom underwent surgical repair of the occipital sac. The mean gestational age at birth was 36 (± 2) weeks. The mean of birth weight was 3.14 (± 0.85) kilograms. The average head circumference at birth was 33.82 cm (± 2.17). The mean diameter of the encephalocele/meningocele was 5.91 (± 1.02) cm. Other common central nervous system abnormalities included hydrocephalus, Dandy-Walker malformation, and agenesis of the corpus callosum. 40% required shunting for hydrocephalus. Surgery to remove the occipital sac occurred at a median age of 2.5 days (1.5-6.5). The most common post-surgical complication was the need for mechanical ventilation. The most common cause of death was pneumonia and the median age at death was 6.66 (0.03-18) months. CONCLUSION: Our findings suggest that neurosurgical intervention, especially for managing encephaloceles, may offer some improvement in survival, albeit within a context of generally poor prognosis. However, these results should be interpreted with caution.

[Surgical treatment of focal drug-resistant epilepsy associated with temporal lobe encephalocele]. / Khirurgicheskoe lechenie patsientov so strukturnoi fokal'noi farmakorezistentnoi epilepsiei, assotsiirovannoi s visochnymi entsefalotsele.

BACKGROUND: In recent years, temporal lobe encephalocele has become more common in patients with focal drug-resistant epilepsy. Despite available experience, there are still no clear recommendations for choosing the extent of surgery in these patients. OBJECTIVE: To evaluate the effectiveness of diagnosis and surgical treatment of focal drug-resistant epilepsy associated with temporal lobe encephalocele. MATERIAL AND METHODS: The study included 21 patients with focal temporal lobe epilepsy and temporal lobe encephalocele. All patients underwent continuous video-EEG monitoring and MRI of the brain. There were 12 (57.4%) selective encephalocele resections and 9 (42.6%) anterior temporal lobectomies. The median follow-up period was 31 months. RESULTS: The overall effectiveness of surgical treatment with postoperative Engel class I was 76% (16 cases). Selective encephalocele resection was followed by postoperative Engel class I in 10 patients (83%). There were 6 (67%) patients with similar outcomes after temporal lobectomy. Mean volume of resected tissue adjacent to encephalocele was 8.3 cm3. CONCLUSION: Surgery is a highly effective treatment for patients with epileptic seizures following temporal lobe encephalocele. In our sample, favorable postoperative outcomes were achieved in 76% of patients (Engel class I). There were no significant differences in effectiveness between selective resection and temporal lobectomy. Further research is necessary for a clear protocol of surgical treatment of focal drug-resistant epilepsy associated with encephalocele.

Carolyn's window approach for spontaneous frontal sinus meningoencephalocele.

Spontaneous meningoencephaloceles (MECs) are sparsely documented in the literature. Those occurring in the frontal sinus are an exceedingly rare entity. MECs are commonly associated with cerebrospinal fluid (CSF) rhinorrhoea. CSF rhinorrhoea is frequently misdiagnosed, causing delays in diagnosis and management. The subsequently increased risk of bacterial meningitis can be life-threatening to patients. We report the case of a woman in her late 70s with a spontaneous frontal sinus MEC, presenting with a 6-month history of CSF rhinorrhoea. The patient was successfully treated using the novel Carolyn's window approach endoscopically; 9-month follow-up revealed no skull-base breach. Our case emphasises the importance of considering MEC as a differential diagnosis for clear rhinorrhoea and demonstrates successful repair through a novel surgical approach.

Prevalence of neural tube defect and its identification during antenatal period: a cross-sectional study in eastern Indian state.

OBJECTIVE: To estimate the prevalence of neural tube defects among all birth outcomes in Odisha during 2016-2022. Additionally, to estimate the identification rate of neural tube defects during Pradhan Mantri Surakshit Matritva Abhiyan sessions. DESIGN: A population-based cross-sectional study with a household survey for neural tube defects using pictorial card as well as a hospital-based study for antenatal ultrasonography data. SETTING: The sample population was selected through multistage random sampling. In the first stage, one district from each zone was selected randomly. In the second stage, using simple random sampling, one community health centre and one urban primary health centre were selected from each district. In the third stage, the population from a block and ward were picked from the selected rural and urban settings, respectively. PARTICIPANTS: All married women in the reproductive age group (18-49 years) residing in these cluster villages in the selected districts were enrolled. RESULTS: The study surveyed 49 215 women and recorded 50 196 birth outcomes, including 49 174 live births, 890 stillbirths and 132 medical terminations of pregnancy. A total of 30 neural tube defect cases were detected. The overall prevalence rate of neural tube defect was 0.59 per 1000 birth outcomes. Spina bifida was the most prevalent neural tube defect with the prevalence of, followed by anencephaly and encephalocele. Despite 26 860 mothers receiving antenatal ultrasonography Pradhan Mantri Surakshit Matritva Abhiyan session, data on neural tube defects and other birth defects detected through these scans is unavailable. CONCLUSION: This study found a low prevalence of neural tube defect in Odisha, which is far lower compared with the older studies from India. There is an urgent need to strengthen the quality of antenatal care services provided under Pradhan Mantri Surakshit Matritva Abhiyan through better training regarding anomaly scans and better data keeping at public healthcare facilities. TRIAL REGISTRATION NUMBER: CTRI/2021/06/034487.

Caso Clínico: Meningohidroencefalocele Gigante / Case Report: Giant Meningohydroencephalocele

INTRODUCCIÓN: Los encefaloceles son una anomalía congénita rara, que afecta a 1 de cada 5 000 nacidos vivos. Un meningohidroencefalocele es la herniación de meninges, ventrículos y parte del encéfalo a través de un defecto congénito en el cráneo. El pronóstico de los recién nacidos con encefalocele depende de varios factores. La corta edad y las anomalías congénitas asociadas en estos pacientes plantean desafíos en el diagnóstico y manejo. CASO CLÍNICO: Recién nacido masculino, nacido a las 37 semanas de gestación, por cesárea de emergencia por parto en fase latente, presentado una masa de gran tamaño, sobre la región parieto-occipital, de bordes lobulados, irregulares, cubierta en parte por cuero cabelludo, y en otras zonas de un tono rojo violáceo. El neonato fue poco reactivo, con tono disminuido, llanto ausente. EVOLUCIÓN: Tras valoración del caso por especialistas, debido al importante defecto de la calota craneal y las anomalías estructurales cerebrales, el paciente no fue candidato para tratamiento quirúrgico. Neonato fue dado de alta con expectativa de vida reducida, para recibir cuidados en el hogar. CONCLUSIÓN: El meningohidroencefalocele gigante es un defecto del tubo neural poco frecuente. El manejo de las encefaloceles puede ser complicado y debe ser individualizado y dependerá de las estructuras anatómicas comprometidas. Si bien en este caso el pronóstico fue malo, siempre es necesario el trabajo multidisciplinario para un adecuado manejo.

BACKGROUND: Encephaloceles are a rare congenital anomaly, affecting 1 in 5 000 live births.A meningohydroencephalocele is the herniation of the meninges, ventricles, and part of the brain through a congenital defect in the skull. The prognosis of newborns depends on several factors. The young age and the associated congenital anomalies in these patients contribute to the diagnostic and treatment challenge. CASE REPORT: Male newborn, born at 37 weeks of gestation, by emergency C-section due to latent labor, presenting a large mass over the parieto-occipital region, with lobed, irregular edges, partially covered by the scalp, and in other areas a purplish red tone. The newborn was no very reactive, had decreased tone, absent crying. EVOLUTION: After specialist's evaluation, due to the significant defect in the cranial shell and the structural brain abnormalities, the patient was not a candidate for surgical treatment. The newborn was discharged with a reduced life expectancy, to receive care at home. CONCLUSION: giant meningohydroencephalocele is a rare neural tube defect. The management of encephaloceles can be complicated and must be individualized, and will depend on the anatomical structures involved. Although in this case the prognosis was bad, multidisciplinary work is always necessary for proper management.

Adult Nasoethmoidal Encephalocele Corrected by Supraorbitary Approach: Case Report and Literature Review

Encephalocele is a protrusion of the central nervous system elements through a defect in the dura mater and in the cranium. The prevalence of encephalocele ranges from 0.08 to 0.5 per 1,000 births. The posterior encephaloceles are more common in North America and Europe, while frontal defect is frequently found in Asia. The present paper describes a 26-year-old male patient presenting with cerebrospinal fluid leak and meningitis symptoms. He was diagnosed with congenital nasoethmoidal encephalocele and treated surgically using a supraorbital approach without complications.

Tomografía computada y resonancia magnética de variantes normales/congénitas de apariencia quística y presentación frecuente en el encéfalo / CT and MRI of Normal/Congenital Variables with Cystic Appearance and Frequent Presence in the Brain

La presentación de una imagen de apariencia quística durante el estudio del encéfalo constituye un hallazgo incidental cada vez más frecuente, pudiendo encontrarse en el espacio extra o intraaxial. Las mismas pueden ser de naturaleza congénita o adquirida, benigna o maligna, ocupantes de espacio con desplazamiento de la línea media o simplemente presentarse sin efecto compresivo alguno. De localización supra o infratentorial, esas imágenes constituyen un desafío diagnóstico, siendo imprescindible su reconocimiento para no solicitar estudios o tratamientos innecesarios. Valoraremos las imágenes de apariencia quística más frecuentes empleando tomografía computada o imágenes de resonancia magnética.

Presence of a cystic formation in brain examination is frequently an incidental finding. They can be intra or extra-axial in location, congenital or acquired, benign or malignant with or without mass effect. Intracranial cysts can be a diagnostic challenge and we should know them to avoid unnecessary exams or treatment. We will analyze the most common cystic formations seen in computed tomography and magnetic resonance.

Large Vertex Epidural Hematoma: Case Report and Review of Surgical Approaches

Vertex epidural hematomas (VEHs) are a special clinical entity due to their clinical presentation, vascular etiology and options of surgical approach. The clinical suspicion involves recognizing the mechanism of the injury and the correct visualization of the hematoma in computed tomography (CT) coronal sequences. In the present article, we describe a case of a very large (146 mL) VEH with central brain herniation, and provide a technical note on the surgical planning and treatment. A 34-year-old male patient was admitted to the hospital after an injury on the left superior parietal region. The Glasgow coma scale score was 6, and the left pupil of the patient was dilated. The CT scan showed a large epidural hematoma on the vertex between the coronal e lambdoid sutures, and a fracture over the sagittal suture. During the surgery, multiple burr holes were made laterally to the sagittal suture, and after inspection and no visualization of bleeding in the superior sagittal sinus (SSS), we performed a standard biparietal craniotomy. The patient was discharged three days after the surgery without any deficits. Currently, with the improvement in imaging modalities,more cases of VEH are being identified. Identifying the etiology prior to the craniotomy is challenging in severe cases. Tears in the SSS can bleed profusely, and they demand strategies during the craniotomy.With multiple burr holes parallel to the sagittal suture, we can visualize whether there is bleeding in the SSS and design a craniotomy with or without a central osseous bridge to anchor the dura. Neurosurgeons must be prepared to plan a surgical strategy in cases of large VEHs. Due to its rare frequency and bleeding risks, VEHs are considered challenging.

Dermoid cyst with cerebellar meningoencephalocele at different locations accompanied by posterior fossa abnormalities: case report

ABSTRACT CONTEXT: Dermoid cysts are well-defined cysts containing sebaceous glands and dermal structures. In the literature, dermoid cysts and associated closure defects have been described in the same locations. CASE REPORT: In this case, a dermoid cyst was found at the base of the mouth with a coexisting closure defect in the occipital calvarium. Additional abnormalities were also observed, including posterior myeloschisis, right cerebellar dysgenesis, vermian hypogenesis and posterior fusion of the second and third vertebrae. The finding of a dermoid cyst located at the base of the mouth is discussed here, with additional imaging findings. CONCLUSION: Dermoid cysts in the head and neck region may be accompanied by posterior fossa abnormalities.

Encefalocele frontonasal: reporte de un caso con diagnóstico prenatal / Frontonasal encephalocele: report of a case with prenatal diagnosis

El encefalocele es un defecto congénito mayor que se caracteriza por la herniación del tejido nervioso y las meninges a través de un defecto de la bóveda craneal. Esto se debe a alteraciones del cierre del tubo neural ocurridas en la cuarta semana de gestación. El diagnóstico prenatal es posible realizarlo a través de ecografía 2D y 3D. Se presenta el caso de un neonato con encefalocele anterior, entidad poco frecuente dentro de los defectos del cierre del tubo neural. Se realizó el diagnóstico presuntivo prenatal por ecografía 2D y 3D, y se confirmó al nacimiento por los hallazgos clínicos y escanografía(AU)

The encephalocele is a major birth defect characterized by herniation of neural tissue and meninges through a defect in the skull, due to alterations of neural tube closure occurring in fourth week of pregnancy. Prenatal diagnosis is possible through 2D and 3D ultrasound. We report a case of anterior encephalocele, a rare disease among the defects of neural tube closure, with diagnosis by 3D and 2D ultrasonography, which was confirmed at birth by clinical findings and scan(AU)