Epileptic encephalopathies secondary to hypothalamic hamartomas treated with radiosurgery: A case series.

OBJECTIVE: Hypothalamic hamartomas are congenital lesions that typically present with gelastic seizures, refractory epilepsy, neurodevelopmental delay, and severe cognitive impairment. Surgical procedures have been reported to be effective in removing the hamartomas, however, they are associated with significant morbidity. Therefore, it is not considered a safe therapeutic modality. Image-guided robotic radiosurgery (CyberKnife® Radiosurgery System) has been shown to provide good outcomes without lasting complications. METHODS: This series of cases describes the clinical, radiological, radiotherapeutic, and postsurgical outcomes of five patients with epileptic encephalopathies secondary to hypothalamic hamartomas who were treated with CyberKnife®. RESULTS: All patients exhibited refractory epilepsy with gelastic seizures and were unsuitable candidates for surgical resection The prescribed dose ranged between 16 and 25 Gy, delivered in a single fraction for four patients and five fractions for one patient while adhering strictly to visual pathway constraints. After radiosurgery, four patients maintained seizure control (one with an Engel class Ia, three with an Engel class 1d), and another presented sporadic, nondisabling gelastic seizures (with an Engel class IIa). After 24-26 months of follow-up, in three patients, their intelligence quotient scores increased. No complications were reported. SIGNIFICANCE: This report suggests that Cyberknife may be a good option for treating hypothalamic hamartoma, particularly in cases where other noninvasive alternatives are unavailable. Nevertheless, additional studies are essential in order to evaluate the effectiveness of the technique in these cases.

Therapeutic options for primary meningeal angiosarcoma: A case report.

BACKGROUND: Primary angiosarcoma (AS) of the central nervous system (PACNS) is an extremely rare malignancy. The meninges represent an uncommon site of origin of PACNS. This report describes a recurrent meningeal PACNS treated with surgery, radiotherapy, stereotactic radiosurgery, and paclitaxel at different stages of the disease. CASE DESCRIPTION: A 36-year-old Asian male presented to our facility with a 4-month history of worsening headaches and complete right homonymous hemianopia. Neuroimaging revealed a left occipital lobe hematome with an underlying left tentorial tumor. After subtotal resection, neuropathological examination revealed features of a malignant endothelial cell AS. He received a course of adjuvant radiation therapy but experienced disease progression. He subsequently received additional stereotactic radiosurgery followed by weekly paclitaxel. Magnetic resonance imaging during the course of treatment revealed stable disease until patient died following another progression of his tumor. CONCLUSION: This case of a meningeal PACNS highlights the importance of considering this entity in the face of a malignant lesion presenting with intracranial hemorrhagic activity. Our observations suggest that the use of paclitaxel provided a modest clinical response in PACNS, highlighting the need to consider a combined approach structured mainly on surgery and radiotherapy. Stereotactic radiosurgery appears to be a promising treatment option.

Características clínicas y patológicas de pacientes con tumores del sistema nervioso central en un centro en Suramérica desde 2010 hasta 2015 / Clinico-pathological characteristics of central nervous system tumors in a South American center from 2010 to 2015

Resumen Objetivo: Caracterizar los pacientes diagnosticados con tumores del sistema nervioso central en el Instituto Neurológico de Colombia durante el periodo 2010-2015. Métodos: Estudio descriptivo retrospectivo. Para los tumores primarios se usó la clasificación de la Organización Mundial de la Salud y para los metastásicos se usó la Clasificación Internacional de Enfermedades en Oncología. Resultados: Se identificaron 288 pacientes, 194 de ellos con tumores primarios y 94 tumores metastásicos. No se lograron clasificar los tumores primarios en el 23,7 % de los casos y para los metastásicos no se obtuvo la clasificación morfológica (histológica) en el 35,1 % de los casos. Los hombres presentaron con mayor frecuencia tumores de comportamiento maligno tipo glioblastoma NEO (no especificado de otra manera) (14,9 %) y en las mujeres predominaron los tumores de comportamiento benigno tipo meningioma (23,2 %). En mayores de 65 años, tanto el glioblastoma NEO como el meningioma fueron más frecuentes en mujeres con 17,4 % y 28,3 %, respectivamente. Entre los pacientes con tumores metastásicos, de acuerdo con la clasificación topográfica, los tumores primarios más frecuentes se localizaron en pulmón (39,4 %) y mama (17 %). No se identificó el sitio primario de metástasis en el 11,7 % de los casos. La histología más comúnmente identificada fue el adenocarcinoma (14,9 %), seguido del carcinoma (8,5 %). Conclusiones: Para una vigilancia efectiva de la enfermedad es necesario realizar un monitoreo epidemiológico y clínico de tumores primarios y metastásicos, mediante el uso de registros institucionales de cáncer, incluyendo datos topográficos, histológicos y moleculares, según disponibilidad.

Abstract Objective: The aim of this study was to characterize patients with a diagno- sis of a central nervous system (CNS) tumors at the Instituto Neurologico de Colombia during the period between 2010 to 2015. Methods: A retrospective descriptive study was conducted. The Classification of the World Health Organization was used for CNS primary tumors whereas the International Classification of Diseases for oncology (ICD-O) was used for CNS metastatic tumors. Results: 288 patients were identified, 194 of them with primary tumors of CNS and 94 with metastatic tu- mors from systemic cancer. It was not possible to classify primary tumors in 23.7% of the cases and regarding the metastatic tumors it was not possible to obtain the classification in 35.1 % of the cases. Men presented more frequently tumors of ma- lignant behavior such as glioblastoma NOS (not otherwise specified) (14.9 %) while in women benign behavior tumors such as meningioma predominated (23.2 %). For population older than 65 years old, both glioblastoma NOS and meningioma were more frequent in women with 17.4 % and 28.3 %, respectively. In patients with CNS metastatic tumors, according to the topographic classification, the most frequent primary tumors were lung (39.4 %), followed by breast (17 %). Its origin was not iden- tified in 11.7 % of the cases. The most identified histology was adenocarcinoma (14.9 %), followed by carcinoma (8.5 %). Conclusion: For disease surveillance, it is necessary to complete epidemiological and clinical monitoring of primary and metastatic tumors of the CNS by using institutional cancer registries including topographic, histological and molecular data according to availability.

Textiloma Mimicking a Recurrent High-Grade Astrocytoma: A Case Report.

Introduction Textiloma (Txm) is a nonmedical term that has been given to foreign body-related inflammatory pseudotumor arising from retained nonabsorbable cotton matrix that is either inadvertently or deliberately left behind during surgery, which may trigger an inflammatory reaction. This report describes a case of Txm mimicking a recurrent high-grade astrocytoma. Case Report We, here, present the case of a 69-year-old female with a 6-month history of progressive left-sided weakness. Neuroimaging studies revealed a large nonenhancing mass in the right frontoparietal lobe. Pathology reported a World Health Organization tumor classification grade II, diffuse astrocytoma. After surgical intervention, external beam radiation was given to the remaining areas of residual tumor. Routine magnetic resonance imaging (MRI) revealed a nodular area of contrast enhancement in the dorsal and inferior margin of the biopsy tract, growing between interval scans, and perfusion-weighted imaging parameters were elevated being clinically asymptomatic. She underwent a complete resection of this area of interest and pathology returned as a Txm with Surgicel fibers. Conclusion After treatment of a neoplasm, if unexpected clinical or imaging evidence of recurrence is present, a foreign body reaction to hemostatic material used during the initial surgery should be included in the differential diagnosis.

Cognitive and brain structural changes in long-term oligodendroglial tumor survivors.

BACKGROUND: We identify cognitive impairment and MRI structural brain changes in long-term oligodendroglial tumor survivors treated with radiation therapy (RT) alone (21%) or with chemotherapy (CT) (79%). METHODS: Oligodendroglial tumor patients (based on the World Health Organization [WHO] 2007 classification) who completed RT ± CT at least 2 years before the study initiation, were classified into 3 groups according to the time treatment was completed: Group 1 = 2-5 years (n = 22), Group 2 = 6-10 years (n = 13), and Group 3 >10 years (n = 13). All patients had a cross-sectional neuropsychological evaluation (n = 48) and a longitudinal volumetric analysis (gray matter [GM; n = 34]) between postsurgical and last follow-up MRI. White matter (WM) changes on MRI were assessed using a qualitative scale. RESULTS: There were no differences regarding tumor or treatment-related characteristics between groups. Six of 22 patients (27.3%) in Group 1; 5/13 (38.5%) in Group 2; and 9/13 (69.2%) in Group 3 had cognitive impairment that was considered severe in 3/22 patients (13.6%) in Group 1; 4/13 (30.8%) in Group 2; and 6/13 (46.2%) in Group 3. Patients in Groups 2 and 3 showed significant GM atrophy and more leukoencephalopathy than Group 1. Cognitive deficits were associated with brain atrophy and WM changes. CONCLUSIONS: Long-term oligodendroglial tumor survivors who underwent standard RT ± CT treatment, mainly >5 years of its completion, present cognitive impairment, especially on memory and executive functions, associated with late GM and WM damage, thus highlighting the need of developing future strategies in patients with oligodendroglial tumor and long expected survival.

Psychogenic non-epileptic and epileptic seizures: clues for a differential diagnosis. Findings from a Colombian study / Crisis psicógenas no epilépticas y crisis epilépticas: pistas para un diagnóstico diferencial. Hallazgos de un estudio colombiano

Introduction. Psychogenic non-epileptic seizures (PNES) are paroxysmal changes in behavior that resemble epileptic seizures, although they have no electrophysiological correlation or clinical evidence of epilepsy. Aim. To compare clinical and sociodemographic characteristics of patients diagnosed with PNES-alone and PNES-andepilepsy. Patients and methods. A cross-sectional study of consecutive patients diagnosed with PNES in a 20-month period was carried out. A video-EEG was performed in all patients. Socio-demographical, clinical and semiological characteristics were compared between those patients with and without concomitant epilepsy. Results. Sixty-five patients were included, 35 (53.9%) had PNES-alone and 30 (46.1%) had PNES-and-epilepsy. The proportion of women in the study was 70.8%. The median age at seizure onset was 16 years. A late start was recorded in PNES-alone group (23 years) compared to PNES-and-epilepsy group (11 years), however, it was not significant. There was a lower frequency of antiepileptic drugs use in the PNES-alone group compared with the PNES-and-epilepsy group. The most frequent semiological features were the gradual onset of events (69.2%) and the duration longer than two minutes (63.1%). Conclusion. The waxing and waning pattern during paroxysmal events suggest a non-epileptic origin. However, it is not uncommon to find patients with concomitant epileptic seizures

Introducción. Las crisis psicógenas no epilépticas (CPNE) son cambios paroxísticos en el comportamiento que se asemejan a las crisis epilépticas, aunque no tienen correlación electrofisiológica ni evidencia clínica de epilepsia. Objetivo. Comparar las características clínicas y sociodemográficas entre pacientes diagnosticados con CPNE, con y sin epilepsia concomitante. Pacientes y métodos. Estudio transversal de pacientes consecutivamente diagnosticados de CPNE durante un período de 20 meses. A todos los participantes se les realizó un videoelectroencefalograma (video-EEG). Se compararon las características sociodemográficas, clínicas y semiológicas entre los que presentaban y los que no presentaban epilepsia concomitante. Resultados. Se incluyó a 65 pacientes, 35 con CPNE (53,9%), y 30 con CPNE y epilepsia (46,1%). La edad mediana en el inicio del video-EEG fue de 33 años, y un 70,8% eran mujeres. La edad mediana de inicio de las crisis fue de 16 años. En el grupo de CPNE hubo un inicio más tardío (23 años) en comparación con el grupo de CNPE y epilepsia (11 años), pero la diferencia no fue significativa. La proporción de pacientes en terapia con fármacos antiepilépticos fue significativamente mayor en el grupo con CPNE y epilepsia comparado con el grupo con CPNE. Las características semiológicas más frecuentemente encontradas fueron el inicio gradual de las crisis (69,2%) y una duración de más de dos minutos (63,1%). Conclusión. La variabilidad en los síntomas sugiere un origen no epiléptico de los eventos paroxísticos, los cuales se presentan frecuentemente en pacientes con epilepsia

Efficacy and tolerability of add-on Lacosamide treatment in adults with Lennox-Gastaut syndrome: An observational study.

PURPOSE: To evaluate the efficacy, safety, and tolerability of lacosamide in adults with LGS in the clinical setting. METHOD: The present report is a retrospective, open-label treatment study carried out from June 2013 to December 2014 at the National Institute of Colombia. Lacosamide was introduced as add-on therapy. All caregivers were instructed to initiate lacosamide at low doses (25-50 mg) and gradually increasing it every 2 weeks. The efficacy was evaluated based on the reduction in the rate of each countable type of seizure. We also evaluated the retention rate for lacosamide as the number of days with lacosamide during follow-up. The tolerability was evaluated base on account the adverse events. RESULTS: We found that lacosamide only improves the seizure rate in three out of 19 patients with LGS, in two of them by more than 50%. The highest seizure reduction rate was observed in the focal and tonic-clonic seizures. The most commonly reported adverse events were worsening of seizures, aggressiveness and irritability. Nine patients (47.4%) showed worsening of their behavior during the treatment with lacosamide. CONCLUSION: Lacosamide can exacerbate both, the tonic and astatic seizures, and the encephalopathy associated with this epileptic syndrome. However, it is interesting to consider the likelihood of suppression of generalized tonic-clonic and focal seizures. That is why; lacosamide could be an option after carefully balancing risks and benefits in each individual case.

Lacosamide in Lennox-Gastaut syndrome: case report.

INTRODUCTION: Lennox Gastaut syndrome (LGS) is an epileptic encephalopathy characterized by tonic, atonic, and atypical absence seizures usually refractory to pharmacological treatment. Patients generally continue with seizures despite treatment with the commercially available antiepileptic drugs (AEDs). Lacosamide (LCM) is a new AED recently approved for treatment of partial onset seizures with or without secondary generalization. Lacosamide has a novel mechanism of action that seems to be different in relation to other conventional AEDs. OBJECTIVE: To report LCM-caused worsening of tonic seizures and electroencephalographic pattern in a patient with Lennox-Gastaut syndrome. CASE REPORT: We report the evolution of a patient with LGS resistant to several AEDs with a cryptogenic hepatopathy in whom LCM caused worsening of tonic seizures and electroencephalographic pattern. Once LCM was discontinued, the patient returned to his clinical and electrical baseline. CONCLUSION: Lennox Gastaut syndrome may exacerbate tonic seizures and electrical pattern of patients with LGS.