Cannabidiol as an adjuvant treatment in adults with drug-resistant focal epilepsy.

Cannabidiol oil (CBD) has been approved as an anti-seizure medication for the treatment of uncommon types of epilepsy, occurring in children: Dravet syndrome, Lennox-Gastaut syndrome, and Tuberous Sclerosis Complex. There are few publications in relation to use the CBD in adult patients with focal drug-resistant epilepsy. The objective of this study was to evaluate the efficacy, tolerability, safety, and quality of life, of adjuvant treatment with CBD, in adult patients with drug-resistant focal epilepsy for at least 6 months. An open, observational, prospective cohort study was conducted using a before-after design (time series) in adult patients undergoing outpatient follow-up in a public hospital in Buenos Aires, Argentina. From a total of 44 patients, 5% of patients were seizure-free, 32% of patients reduced more than 80% of their seizures and 87% of patients reduced 50% of their monthly seizures. Eleven percent presented a decrease of less than 50% in seizure frequency. The average final dose was 335 mg/d orally administered. Thirty-four percent of patients reported mild adverse events and no patient reported severe adverse effects. At the end of the study, we found in most patients a significant improvement in the quality of life, in all the items evaluated. Adjuvant treatment with CBD in adult patients with drug-resistant focal epilepsy was effective, safe, well tolerated, and associated with a significant improvement in their quality of life.

[Ectopic craniopharyngioma and Gardner's syndrome: Case report and literature review]. / Craneofaringioma ectópico y síndrome de Gardner: a propósito de un caso y revisión de la literatura.

INTRODUCTION: Craniopharyngioma accounts for around 3% of all primary tumours of the central nervous system. It is usually located in the suprasellar region, although it may also have an ectopic location. CASE REPORT: The case is presented on 29 year-old male who underwent surgery for a jaw osteoma when he was 19 years old and was subsequently diagnosed with Gardner's syndrome. He was admitted in our Hospital with right facial paresis and diplopia of one day onset. The examination showed mild right VII and VI cranial nerves paresis. Magnetic resonance imaging of the brain demonstrated a rounded solid and cystic lesion with well-defined contours of about 2cm in diameter filling the fourth ventricle. The patient underwent a posterior fossa craniotomy using a telovelar approach with complete removal of the tumour implanted at roof level of the fourth ventricle. The final histology of the tumour was reported as adamantinomatous craniopharyngioma. CONCLUSION: Craniopharyngioma may appear in another location other than the suprasellar region. Its atypical location may be related to Gardner syndrome by still unknown pathogenic mechanisms.

Ovarian Metastasis of a Thymoma: Report of a Case and Literature Review.

Thymomas are infrequent, although they are the most common neoplasms in the anterior mediastinum. They are slow-growing tumors that may involve surrounding structures by direct extension. In these aggressive cases, intrathoracic recurrences are frequent. However, distant metastases are uncommon, the most common sites including liver, lymph nodes, and bones. Metastatic thymoma to the ovary is exceedingly rare, with only 6 cases reported in the literature. We report a 46-yr-old female with a metastatic thymoma to her left ovary 6 yr after the initial diagnosis of thymoma, Type B1 with pleural invasion.

Stereo-EEG features of temporal and frontal lobe seizures with loss of consciousness.

The loss of consciousness (LOC) during seizures is one of the most striking features that significantly impact the quality of life, even though the neuronal network involved is not fully comprehended. We analyzed the intracerebral patterns in patients with focal drug-resistant epilepsy, both with and without LOC. We assessed the localization, lateralization, stereo electroencephalography (SEEG) patterns, seizure duration, and the quantification of contacts exhibiting electrical discharge. The degree of LOC was quantified using the Consciousness Seizure Scale. Thirteen patients (40 seizures) with focal drug-resistant epilepsy underwent SEEG. In cases of temporal lobe epilepsy (TLE, 6 patients and 15 seizures), LOC occurred more frequently in seizures with mesial rather than lateral temporal lobe onset. On the other hand, in cases of frontal lobe epilepsy (7 patients; 25 seizures), LOC was associated with pre-frontal onset, a higher number of contacts with epileptic discharge compared to the onset count and longer seizure durations. Our study revealed distinct characteristics during LOC depending on the epileptogenic zone. For temporal lobe seizures, LOC was associated with mesial seizure onset, whereas in frontal lobe epilepsy, seizure with LOC has a significant increase in contact showing epileptiform discharge and a pre-frontal onset. This phenomenon may be correlated with the broad neural network required to maintain consciousness, which can be affected in different ways, resulting in LOC.

Causes and prognosis of adults experiencing a first seizure in adulthood: A pilot cohort study conducted in five countries in Latin America.

There are limited data on first seizure (FS) among adults in low and middle-income countries. We describe findings from a prospective cohort study involving 180 adults presenting with seizures in emergency departments in five Latin American countries. Overall, 102 participants (56.7%) had acute symptomatic seizures (ASyS) while 78 (43.3%) had unprovoked seizures (UPS). Among patients with ASyS, 55 (53.9%) had structural causes, with stroke (n = 24, 23.5%), tumor (n = 10, 9.8%), and trauma (n = 3, 3%) being the most frequent. Nineteen patients (18.6%) had infectious causes, including four (4%) with meningoencephalitis, three (3%) neurocysticercosis, and two (2%) bacterial meningoencephalitis. Twenty patients (19.6%) had metabolic/toxic evidence, including four (4%) with uremic encephalopathy, two (2%) hyponatremia, and three (3%) acute alcohol intoxication. Immune dysfunction was present in seven (7%) patients and neurodegenerative in two (2%). Among participants with UPS, 45 (57.7%) had unknown etiology, 24 (30.7%) had evidence of structural disorders (remote symptomatic), four (5%) were related to infectious etiology (>7 days before the seizure), and five (6.4%) had genetic causes. During the 3- and 6-month follow-up, 29.8% and 14% of patients with UPS, respectively, experienced seizure recurrence, while 23.9% and 24.5% of patients with ASyS had seizure recurrence. Longer follow-up is necessary to assess seizure recurrence for patients with ASyS after the acute cause is resolved and to determine the 10-year risk of recurrence, which is part of the definition of epilepsy. PLAIN LANGUAGE SUMMARY: We monitored 180 adults who presented with their first seizure in emergency departments across five Latin American countries. Among these patients, 57% had acute symptomatic seizures, with structural causes such as stroke (23%), infection (17%), or tumor (10%) being more prevalent. Among the 43% with unprovoked seizures, 58% showed no identifiable acute cause, while 6.4% were due to genetics. Within 3 months after their initial seizure, 26.6% of individuals experienced a second seizure, with 11.9% continuing to have seizures in Months 3-6. Between Months 3 and 6, an additional 20% of patients encountered a second seizure. Research is needed to better understand the cause and prognosis of these patients to improve outcomes.

Brain metastasis treatment guidelines: consensus by the Spanish Society of Neurosurgery Tumor Section.

Brain metastases are tumors that arise from a tumor cell originated in another organ reaching the brain through the blood. In the brain this tumor cell is capable of growing and invading neighboring tissues, such as the meninges and bone. In most patients a known tumor is present when the brain lesion is diagnosed, although it is possible that the first diagnose is the brain tumor before there is evidence of cancer elsewhere in the body. For this reason, the neurosurgeon must know the management that has shown the greatest benefit for brain metastasis patients, so treatments can be streamlined and optimized. Specifically, in this document, the following topics will be developed: selection of the cancer patient candidate for surgical resection and the role of the neurosurgeon in the multidisciplinary team, the importance of immunohistological and molecular diagnosis, surgical techniques, radiotherapy techniques, treatment updates of chemotherapy and immunotherapy and management algorithms in brain metastases. With this consensus manuscript, the tumor group of the Spanish Society of Neurosurgery (GT-SENEC) exposes the most relevant neurosurgical issues and the fundamental aspects to harmonize multidisciplinary treatment, especially with the medical specialties that are treating or will treat these patients.

18F-Fluorocholine PET/CT in the Prediction of Molecular Subtypes and Prognosis for Gliomas.

AIM: To study the association of metabolic features of F-fluorocholine in gliomas with histopathological and molecular parameters, progression-free survival (PFS) and overall survival (OS). METHODS: Prospective multicenter and nonrandomized study (Functional and Metabolic Glioma Analysis). Patients underwent a basal F-fluorocholine PET/CT and were included after histological confirmation of glioma. Histological and molecular profile was assessed: grade, Ki-67, isocitrate dehydrogenase status and 1p/19q codeletion. Patients underwent standard treatment after surgery or biopsy, depending on their clinical situation. Overall survival and PFS were obtained after follow-up. After tumor segmentation of PET images, SUV and volume-based variables, sphericity, surface, coefficient of variation, and multilesionality were obtained. Relations of metabolic variables with histological, molecular profile and prognosis were evaluated using Pearson χ and t test. Receiver operator caracteristic curves were used to obtain the cutoff of PET variables. Survival analysis was performed using Kaplan-Meier and Cox regression analysis. RESULTS: Forty-five patients were assessed; 38 were diagnosed as having high-grade gliomas. Significant differences of SUV-based variables with isocitrate dehydrogenase status, tumor grade, and Ki-67 were found. Tumor grade, Ki-67, SUVmax, and SUVmean were related to progression. Kaplan-Meier analysis revealed significant associations of SUVmax, SUVmean, and multilesionaly with OS and PFS. SUVmean, sphericity, and multilesionality were independent predictors of OS and PFS in Cox regression analysis. CONCLUSIONS: Metabolic information obtained from F-fluorocholine PET of patients with glioma may be useful in the prediction of tumor biology and patient prognosis.

Hiperplasia sebácea ectópica en mucosa oral de neonatos: reporte de tres casos / Ectopic sebaceous gland hyperplasia in neonates oral mucosa: 3 case studies / Hiperplasia sebácea ectópica na mucosa oral de recém-nascidos: relato de 3 casos

La hiperplasia de glándulas sebáceas es un hallazgo benigno y transitorio, común en el período neonatal. Secundariamente al estímulo hormonal androgénico se produce un hipercrecimiento de las glándulas, con mayor frecuencia en nariz y mejillas, donde existen en mayor densidad. La hiperplasia de glándulas en una localización ectópica, llamada gránulos de Fordyce (GF), es excepcional en el período neonatal. Se han reportado en aproximadamente 1% de los recién nacidos, y con frecuencia se localizan en la mucosa oral. Los GF se describen como lesiones papulares de aspecto vesiculoso blanco amarillentas de 1-3 mm2, que podrían confundir al neonatólogo o al pediatra con entidades infecciosas, dando lugar a pruebas invasivas y tratamientos innecesarios. Se describen tres casos clínicos de neonatos con diagnóstico de hiperplasia sebácea ectópica localizada en mucosa oral, con el objetivo de revisar la etiología, las características clínicas, los diagnósticos diferenciales y la evolución de esta entidad benigna. Conclusiones: la hiperplasia sebácea ectópica en mucosa oral de neonatos es un hallazgo benigno autolimitado que se presenta con baja frecuencia. El reconocimiento clínico de esta entidad es importante para evitar diagnósticos incorrectos y tratamientos innecesarios.

Sebaceous gland hyperplasia is a common transient and benign finding in neonates. After androgenic hormonal stimulation, there is a gland overgrowth mainly in the nose and cheeks where there is a greater density of glands. Ectopic sebaceous gland hyperplasia, called Fordyce's Granules (FG), is exceptional in neonates and it is reported in approximately 1% of newborns and frequently located in the oral mucosa. FGs are described as 1-3mm2 yellowish-white papular and vesicular lesions. Neonatologists or pediatricians may confuse these clinical features with infectious diseases, leading to invasive tests and unnecessary treatment. We describe three clinical cases of neonates with diagnosis of ectopic sebaceous gland hyperplasia located in the oral mucosa, with the aim of reviewing the etiology, clinical characteristics, differential diagnoses and evolution of this benign entity. Conclusions: ectopic sebaceous gland hyperplasia of the lips is a self-limited benign finding occurring infrequently in newborns. The clinical recognition of this entity is important to avoid inaccurate diagnoses or unnecessary treatment.

A hiperplasia das glândulas sebáceas é um achado benigno e transitório comum nos neonatos. Secundário ao estímulo hormonal androgênico, há um hipercrescimento das glândulas com mais frequência no nariz e nas bochechas onde há uma maior densidade das glândulas. A hiperplasia das glândulas num local ectópico, chamado Fordyce Granules (FG), é excepcional no período neonatal, e ela é relatada em aproximadamente 1% dos recém-nascidos e muitas vezes está localizada na mucosa oral. Os FGs são descritos como lesões vesiculares brancas amareladas de 1-3mm2, o que poderia confundir o neonatologista ou pediatra com entidades infecciosas, levando a testes invasivos e tratamentos desnecessários. Descrevemos três relatos clínicos de recém-nascidos com diagnóstico de hiperplasia sebácea ectópica localizada na mucosa oral, com o objetivo de rever a etiologia, características clínicas, diagnósticos diferenciais e evolução desta entidade benigna. Conclusões: hiperplasia sebácea ectópica na mucosa oral de recém-nascidos é um achado benigno autolimitante que ocorre com baixa frequência. O reconhecimento clínico desta entidade é importante para evitar diagnósticos incorretos e tratamentos desnecessários.

Consenso sobre el tratamiento neuroquirúrgico de las metástasis intracraneales elaborado por el Grupo de Trabajo de Neurooncología (GTNO) de la Sociedad Española de Neurocirugía / Brain metastasis treatment guidelines: Consensus by the Spanish Society of Neurosurgery Tumor Section

Las metástasis cerebrales (MC) son tumores que se forman a partir de una célula tumoral originada en otro órgano y que a través de la sangre llega al cerebro donde es capaz de crecer e invadir los tejidos vecinos, como meninges y hueso. En la mayor parte de los pacientes existe un tumor conocido cuando se diagnostica la lesión cerebral, aunque es posible que el tumor del cerebro sea el primer hallazgo antes de que se tenga evidencia de la patología oncológica en otro lugar del organismo. Por este motivo, el neurocirujano debe conocer el manejo que ha demostrado mayor beneficio para estos sujetos, de manera que se agilicen y optimicen los tratamientos. Concretamente, en este documento se desarrollarán, entre otros temas: la selección del paciente oncológico candidato a la resección quirúrgica y el papel del neurocirujano en el equipo multidisciplinar, la importancia del diagnóstico inmunohistológico y molecular, técnicas quirúrgicas y de RT, actualización de tratamientos de quimioterapia e inmunoterapia y algoritmos de manejo en MC. Con este manuscrito de consenso, el Grupo de Tumores de la Sociedad Española de Neurocirugía (GT- SENEC) expone las cuestiones neuroquirúrgicas más relevantes y los aspectos fundamentales para armonizar el tratamiento multidisciplinar, sobre todo con las especialidades médicas que estén tratando o vayan a abordar a estos pacientes (AU)

Brain metastases are tumors that arise from a tumor cell originated in another organ reaching the brain through the blood. In the brain this tumor cell is capable of growing and invading neighboring tissues, such as the meninges and bone. In most patients a known tumor is present when the brain lesion is diagnosed, although it is possible that the first diagnose is the brain tumor before there is evidence of cancer elsewhere in the body. For this reason, the neurosurgeon must know the management that has shown the greatest benefit for brain metastasis patients, so treatments can be streamlined and optimized. Specifically, in this document, the following topics will be developed: selection of the cancer patient candidate for surgical resection and the role of the neurosurgeon in the multidisciplinary team, the importance of immunohistological and molecular diagnosis, surgical techniques, radiotherapy techniques, treatment updates of chemotherapy and immunotherapy and management algorithms in brain metastases. With this consensus manuscript, the tumor group of the Spanish Society of Neurosurgery (GT-SENEC) exposes the most relevant neurosurgical issues and the fundamental aspects to harmonize multidisciplinary treatment, especially with the medical specialties that are treating or will treat these patients (AU)

Craneofaringioma ectópico y síndrome de Gardner: a propósito de un caso y revisión de la literatura / Ectopic craniopharyngioma and Gardner's syndrome: Case report and literature review

Introducción: El craneofaringioma representa alrededor del 3% de todos los tumores primarios del sistema nervioso central. Por lo general, se localiza en la región supraselar, sin embargo, puede tener una localización atípica. Caso clínico: Presentamos el caso de un varón de 29 años de edad que fue intervenido de un osteoma de mandíbula a los 19 años de edad, y a quien posteriormente se le diagnosticó síndrome de Gardner. Ingresó en nuestro hospital con una paresia facial derecha de un día de evolución acompañada de diplopía. La exploración neurológica confirmó la existencia de una paresia leve del VI y VII pares craneales derechos. La resonancia magnética cerebral mostró una lesión sólido-quística, redondeada, de contorno bien definido, de unos 2cm de diámetro que ocupaba el cuarto ventrículo. El paciente fue intervenido mediante una craneotomía fosa posterior, abordaje telovelar y extirpación completa del tumor implantado a nivel del techo del cuarto ventrículo. El estudio anatomopatológico definitivo informó de un craneofaringioma adamantinomatoso. Conclusión: El craneofaringioma es un tumor que puede aparecer en otra localización diferente de la región selar-supraselar, y por mecanismos etiopatogénicos hasta ahora desconocidos su localización atípica puede guardar relación con alguna forma de poliposis adenomatosa familiar como el síndrome de Gardner

Introduction: Craniopharyngioma accounts for around 3% of all primary tumours of the central nervous system. It is usually located in the suprasellar region, although it may also have an ectopic location. Case report: The case is presented on 29 year-old male who underwent surgery for a jaw osteoma when he was 19 years old and was subsequently diagnosed with Gardner's syndrome. He was admitted in our Hospital with right facial paresis and diplopia of one day onset. The examination showed mild right VII and VI cranial nerves paresis. Magnetic resonance imaging of the brain demonstrated a rounded solid and cystic lesion with well-defined contours of about 2cm in diameter filling the fourth ventricle. The patient underwent a posterior fossa craniotomy using a telovelar approach with complete removal of the tumour implanted at roof level of the fourth ventricle. The final histology of the tumour was reported as adamantinomatous craniopharyngioma. Conclusion: Craniopharyngioma may appear in another location other than the suprasellar region. Its atypical location may be related to Gardner syndrome by still unknown pathogenic mechanisms