Spectrum of Mri Findings in Pediatric Epilepsy–Medical and Surgical Causes of Epilepsy in Children And its’ Radiological Correlation

@#Introduction: Epilepsy is the common condition encountered in both adults and pediatric population. It occurs as a result of various spectrum of etiology ranging from infections to tumors. EEG and Neurosonogram can characterize the type of epilepsy; however, imaging is the only tool to identify the lesion, its location, and extent and resection possibility. CT was the only modality before the era of MRI. However, CT was only used to identify the lesion with hemorrhage and calcification. It is having the disadvantage of having poor spatial resolution and using radiation. The era of MRI has changed the imaging due to its higher spatial resolution, gray white matter differentiation, status of myelination and non-utilization of radiation. Purpose: The aim of study was to detect and characterize various lesions causing epilepsy in pediatric age group (0-12 years) and also to detect frequency with which they occurred using MRI. Methods: The study was performed on 50 children under the age of 12 years over a period of 1 year who presented with epilepsy. Patients with trauma and febrile seizure disorders were excluded. Conventional and contrast MRI was performed in all cases and lesions were characterized in location, signal intensity, and other features. Results: The mean age group of the study population was 1-5 years. Generalized seizures constituted the major seizure group. Our study shows infection as the most common etiology followed by mesial temporal sclerosis and Focal cortical dysplasia. It was followed by neoplastic etiology, phacomatosis and demyelinating diseases. Conclusion: MRI is the imaging modality of choice in the evaluation of pediatric patients presenting with epilepsy. Proper MRI seizure protocol helps to establish the correct diagnosis, plan the management according to diagnosis as well as helps in prognosis.

Composite pleomorphic xanthoastrocytoma-ganglioglioma; assessing and addressing the dilemma of differential expression of neuronal markers: Case report with diagnostic perspective

We report the case of a 5-year-old male child presenting with seizures for 4 months. Magnetic resonance imaging (MRI) revealed a cortical-based solid cystic lesion in the right parietal lobe. Histopathological examination showed a tumour comprised of spindled glial fibrillary acid protein (GFAP) positive neoplastic cells interspersed with bizarre pleomorphic cells showing nuclear pseudoinclusions and intermingled dysplastic ganglion cells variably immunopositive for synaptophysin, chromogranin, Neu-N and immunonegative for neuron filament protein (NFP). This report highlights the occurrence of the rare composite pleomorphic xanthoastrocytoma-ganglioglioma and the vagaries of immunohistochemical analysis in highlighting neuronal differentiation in such a case setting. In addition, to the best of our knowledge this is the youngest patient till date to present with this entity.

Analysis of clinical characteristics, surgical strategy and prognosis for brainstem gangliogliomas / 中国医师进修杂志

Objective To analyze the clinical and imaging features, surgical strategy and prognosis in patients with brainstem gangliogliomas (GG). Methods The clinical data of 46 patients with brainstem GG from February 2010 to August 2017 were retrospectively analyzed. The clinical and imaging features, surgical methods and prognosis were reviewed. Results The age of 46 brainstem GG patients was (22.5 ± 4.6) years, the duration was (38.1 ± 8.9) months, and the most common symptoms were dizziness and headache, accounting for 47.8％(22/46). The MRI results showed that endogeny type was in 5 cases, exogenesis type in 10 cases, and endogeny-exogenesis type in 31 cases. The exogenesis type was enrolled as non-infiltrate brainstem group (10 cases), and the endogeny type and endogeny-exogenesis type were enrolled as infiltrate brainstem group (36 cases). The axial T1WI showed that the crescent sign was in 20 cases (43.5％, 20/46), and the sagittal T1WI showed that the patching sign was in 31 cases (67.4％, 31/46). Among all patients, total resection was achieved in 13 cases, near total resection in 9 cases, subtotal resection in 16 cases, partial resection in 7 cases and biopsy in 1 case. No operative death occurred. The patients were followed up for 3 to 87 (40.6 ± 12.8) months, and 5 patients died (all in infiltrate brainstem group). The progression- free survival in non- infiltrate brainstem group was significantly longer than that in infiltrate brainstem group:(46.0 ± 13.1) months vs. (19.5 ± 8.9) months, and there was statistical difference (P<0.05). Conclusions Most brainstem GG has some unique MRI characteristics. Microsurgical resection might be preferred. Tumors that locate outside the brainstem can be fully removed. The long-term prognosis of brainstem GG is better.

Arteriovenous malformation masking associated anaplastic ganglioglioma in an adolescent / Malformación arteriovenosa asociada enmascara un ganglioglioma anaplásico multifocal en un adolescente

Abstract Grade III anaplastic ganglioglioma is an aggressive, rare, and radiosensitive central nervous system (CNS) tumour. It is more common in males, with a ratio of 1.3 to 1. Its peak incidence is in the third decade of life. Only 10 cases were recorded in children in Colombia from 2000 to 2014, with a fatal outcome in spite of radiation therapy. This is a case of an adolescent, who began having headaches, with warning signs related to an arteriovenous malformation hindering the diagnosis of this rare tumour. This presented in its aggressive, multi-focus form. Knowledge of clinical manifestations of space-occupying intracranial lesions facilitates the assessment and treatment of affected children.

Resumen El ganglioglioma anaplásico grado III es un tumor del sistema nervioso central (SNC) agresivo, infrecuente y radiosensible. Afecta más a hombres en una relación 1,3 a 1. Su pico de incidencia se encuentra en la tercera década de la vida. Existen solo 10 casos registrados en niños en Colombia desde el 2000 al 2014, con desenlace fatal a pesar de la radioterapia. Se presenta un caso de un adolescente que debutó con cefalea con signos de alarma asociado a una malformación arteriovenosa que dificultó el diagnóstico de este raro tumor, cuya presentación fue la más agresiva: la forma multicéntrica. El conocer las manifestaciones clínicas de lesiones intracraneales ocupantes de espacio facilita la evaluación y tratamiento a los niños afectados.

Ganglioglioma: a clinicopathological study of 19 cases / 临床与实验病理学杂志

Purpose To study the clinicopathologic features of ganglioglioma. Methods The clinicopathologic data of the cases pathologically diagnosed as ganglioglioma that underwent resection of epileptic focus were retrospectively analyzed. Results In the 19 cases studied, the mean onset age was 9.1 years, and the duration of disease was 9.3 years. MRI images showed abnormal signals. The majority of the site was temporal lobe (14/19, 73.7％). The tumors showed heterogeneity and often accompanied by focal cortical dysplasias (13/19, 68.4％). Immunohistochemical staining showed CD34 positive in 18 cases, Nestin positive in 16 cases, and BRAF-V600E positive in 6 case. The positive expression rate of CD34 and Nestin did not have significant differences. Conclusion The diagnosis of ganglioglioma relies on pathological observations combined with clinical features and neuroradiological examinations. Differential diagnosis should be done from other tumors or cortical dysplasia. Immunohistochemical staining of CD34 and Nestin can help diagnosis.

Anaplastic Ganglioglioma of the Pineal Region ­ A Case Report

Introduction Gangliogliomas are tumors commonly found in the temporal lobe and related to seizures; their appearance in the pineal region is rarely described. This report characterizes the first case of anaplastic ganglioglioma of the pineal region. Case Report The authors describe the case of a 32-year-old woman that developed progressive headache. An MRI investigation revealed a pineal tumor. The patient tested negative for biomarkers and underwent surgery through supracerebellar infratentorial approach and achieved gross total resection of the tumor in a challenging location. Pathological analysis revealed a biphasic neoplasm with the following two distinct phenotypes in separate fields: an immature neuronal component with several atypical mitoses and a mature astrocytic component with bipolar cells, microcysts, and eosinophilic bodies. The Ki67/MIB1 proliferation index was 20­30% in localized hotspots. Based on the pathological findings, the tumor was defined as an anaplastic ganglioglioma World Health Organization (WHO) grade III. Discussion/Conclusion Gangliogliomas are classified as glioneural neoplasms based on the histologic findings described as a mixture of neoplastic astrocytes and neurons. Moreover, these tumors represent 0.4­1.3% of tumors of the central nervous system. Authors describe de novo anaplastic ganglioglioma as 1% of the largest series. Gross total resection and adjuvant treatment may play important role in patients' prognostic. In this case, due to the malignant anaplastic component of her tumor, the patient received treatment with temozolamide and radiotherapy after gross total resection of the lesion.

Introdução Gangliogliomas são tumores comumente encontrados no lobo temporal e se relacionam com crises epilépticas; o aparecimento desses tumores na região da pineal é raramente descrito. Este relato caracteriza o primeiro caso de ganglioglioma anaplásico da região da pineal. Relato de Caso Paciente do sexo feminino, 32 anos, apresentou-se com cefaleia de piora progressiva. Investigação com ressonância magnética revelou tumor na região da pineal. Os biomarcadores para tumores da pineal foram negativos e a paciente foi submetida a microcirurgia com o acesso supracerebelar e infratentorial atingindo ressecção total da lesão. A análise patológica revelou neoplasia bifásica com dois fenótipos distintos em campos separados: um componente era composto por células neuronais imaturas com inúmeras mitoses atípicas e o segundo componente era composto por astrócitos maduros, microcistos e corpos eosinofílicos. Foi encontrado um índice proliferativo Ki67/M1B1 de 20­30%. Baseado nos achados anatomopatológicos, o tumor foi definido como ganglioglioma anaplásico grau III da OMS. Discussão/Conclusão Gangliogliomas são classificados como neoplasias glioneurais baseado nos achados histológicos descritos como misto de neoplasia neuronal e glial; esses tumores representam 0,4­1,3% de todos os tumores do sistema nervoso central. Ganglioglioma anaplásico de novo tem sido descrido em 1% nas maiores series de gangliogliomas. Ressecção total da lesão e tratamento adjuvante desempenham um papel importante no prognóstico dos pacientes. Devido ao componente anaplásico do tumor em questão, a paciente foi tratada com temozolamida e radioterapia após ressecção total da lesão.

Unusual ganglioglioma with extensive calcification and ossification.

Ganglioglioma is a slow‑growing relatively low‑grade mixed glioneuronal tumor with most cases corresponding to the WHO Grade I category. It frequently presents with seizures. The temporal lobe is the most common location followed by frontal, parietal, and occipital lobes. These generally behave in a benign fashion and have a favorable prognosis. We describe a case of a 24‑year‑old male presenting with convulsions and a calcified parieto‑occipital mass. This mass removed from the parietal lobe showed neoplastic glial and dysplastic neuronal tissue amidst extensive areas of calcification and foci of ossification. On immunohistochemistry, the glial component expressed glial fibrillary acidic protein whereas the dysplastic neuronal component expressed synaptophysin and CD34. Epithelial membrane antigen was negative and Ki‑67 showed a low proliferative index. After the surgery, the patient is free of neurological symptoms. Widespread calcification and ossification are very unusual in ganglioglioma, which prompted us to report this case

Undiagnosed Anaplastic Ganglioglioma Resulting in the Sudden Unexpected Death of a Young Woman / 대한법의학회지

Intracranial neoplasms usually have definitive symptoms, causing a need for medical intervention, but a few cases result in sudden unexpected death even before diagnosis. In these cases, autopsy or post-mortem imaging may be the only way of identifying the existence of a tumor, so investigators or forensic specialists who participate in the post-mortem inspection should be aware of these possibilities. We report on a case of a 26-year-old woman without any medical history found dead in her home. A 5-cm intraventricular tumor was found during autopsy, which was histologically consistent with anaplastic ganglioglioma a very rare type of neuroglial tumor with the potential for malignant behavior. The tumor showed the characteristic features of anaplastic ganglioglioma, such as increased cellularity, a high Ki-67 proliferative index, and necrosis. There were signs of increased intracranial pressure, including flattened gyri and dilated ventricles, which led to the conclusion that this brain tumor was the cause of death.

Ganglioglioma in Brainstem : Case Report and a Review of Literatures

Ganglioglioma is an infrequent tumor of the central nervous system (CNS); mostly supratentorial region. But, they can occur anywhere in the central nervous system such as brainstem, cerebellopontine angle (CPA), thalamus, optic nerve and spinal cord. Although it occurs rarely, ganglioglioma should be included in the differential diagnosis of a posterior fossa mass because early recognition is important for treatment and patient counseling.

Treatment and Outcomes for Gangliogliomas: A Single-Center Review of 16 Patients

BACKGROUND: Ganglioglioma is a rare and slowly growing benign tumor. We investigated the outcomes of patients who underwent different combination treatments. METHODS: Between 1998 and 2012, 16 patients, including 11 men and 5 women, with a median age of 12.5 years (range, 2.5-65 years) were treated for intracranial gangliogliomas at our institution. The median follow-up period was 5.7 years (range, 48 days-15.6 years). Fifteen cases were included in the outcome assessment because one patient was lost to follow-up. Complete resection was achieved in 8 (53%) patients. Six (40%) patients underwent incomplete resection with or without adjuvant radiotherapy, and one patient with a brainstem tumor underwent only stereotactic biopsy. RESULTS: Gangliogliomas predominantly affected young (87.5%), male patients and most frequently presented with seizures (64%). Of eight patients who underwent complete resection, seven did not show recurrence, whereas only three of six with incomplete resection showed no recurrence. Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries). A tumor control rate of 93% (14/15) was achieved at the last follow-up. No recurrence or malignant changes were observed after a median follow-up of 12 and 4.5 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment. CONCLUSION: Complete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes. In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery, GK radiosurgery should be considered for the treatment of residual or recurrent tumors.

Ganglion cells in circumscribed astrocytic tumors: possible implication in classification and prognosis / Implicação da presença de células ganglionares na classificação e evolução de tumores astrocíticos circunscritos

INTRODUCTION: Glial and neuroglial cell neoplasms comprise pilocytic astrocytoma (PA), pleomorphic xanthoastrocytoma (PXA) and ganglioglioma (GG), which share various similarities, though PA has better prognosis. As ganglion cells (GC) may be scarce in GG and these gangliogliomas may recur or progress to grade III, an accurate diagnosis is essential. OBJECTIVES: The aim was to identify GC and eosinophilic granular bodies (EGB) in PA and PXA, to evaluate its effect on patient’s outcome and compare them with GG. METHODS: A retrospective analysis of radiological, morphological and follow-up aspects (disease free-survival, recurrence and death) of 30 cases (14 PA, 8 PXA, 8 GG). Hematoxylin and eosin (HE) stained sections were reviewed to identify the presence of neoplastic GC and EGB. They were immunostained for synaptophysin (SYN) and neurofilament (NF). Glial fibrillary acidic protein (GFAP) immunostaining was performed in selected cases. RESULTS: Six PA were reclassified as GG due to the presence of GC by HE or immunohistochemistry. Some EGB resembling degenerate GC were also immunostained for SYN/NF and most of them were negative for GFAP. The mean disease-free survival was 62.16 months. Four tumors recurred and one patient died. All PXA had GC, suggesting that they were variants of GG, 4 of which recurred and one patient died. Mean disease-free survival was 69 months. The radiological aspect was predominantly cystic. CONCLUSION: We propose that PA and PXA with GC or with EGB immunopositive for neuronal markers could be variants of GG, and some EGB may represent degenerate GC. However, the presence of GC does not seem to modify the biological behavior of these neoplasms.

INTRODUÇÃO: As neoplasias circunscritas incluem astrocitoma pilocítico (AP), xantoastrocitoma pleomórfico (XP) e ganglioglioma (GG), que compartilham diversas semelhanças, sendo o AP o de melhor prognóstico. Como as células ganglionares (CG) no GG podem ser escassas e os GGs podem recidivar ou evoluir (grau III), é fundamental o diagnóstico preciso. OBJETIVOS: Identificar CG e corpos granulares eosinofílicos (CGE) em AP e XP, avaliar sua implicação na evolução e comparar com o GG. MÉTODOS: Análise retrospectiva dos aspectos radiológicos, morfológicos e evolutivos (tempo livre de doença, recidiva e óbito) de 30 casos (14 AP, oito XP, oito GG). Cortes corados com hematoxilina e eosina (HE) foram revistos para a identificação da presença de CG neoplásicas e CGE. Estes foram imunomarcados para sinaptofisina (SIN) e neurofilamento (NF) e, em casos selecionados, para glial fibrillary acidic protein (GFAP). RESULTADOS: Seis AP foram reclassificados para GG pela presença de CG (HE ou imunomarcação). Alguns CGE, semelhantes às CG degeneradas, também imunomarcaram para SIN/NF, a maioria sendo negativa para GFAP. O tempo médio livre de doença foi de 62,16 meses. Quatro tumores recidivaram; um deles evoluiu para óbito. Todos os XP possuíam CG, sugerindo que são variantes de GG, dos quais quatro recidivaram (um óbito). O tempo médio livre de doença foi de 69 meses. O aspecto radiológico foi predominantemente cístico. CONCLUSÃO: Sugerimos que AP e XP com CG ou CGE imunopositivos para marcadores neuronais possam ser variantes de GG e alguns CGE representem CG degeneradas; entretanto, a presença de CG ganglionares parece não modificar o comportamento biológico dessas neoplasias.

Sudden Death Due to a Ganglioglioma / 대한법의학회지

The incidence of sudden death due to undiagnosed primary intracranial tumor is very low in forensic autopsy practice. The ganglioglioma is a well differentiated, slow growing primary brain tumor that is encountered predominantly in childhood. We report a sudden, unexpected death in a 6-year-old girl from a ganglioglioma. On autopsy, there was an intraventricular tumor with recent hemorrhage, occupying the anterior aspect of the lateral ventricle and resulting in hydrocephalus. Acute intratumoral hemorrhage appears to be the mechanism of sudden death.

Cerebellar Ganglioglioma

The location of ganglioglioma (GG) within the infratentorial compartment is unusual. The authors report a rare case of GG in the cerebellar hemisphere. A 12-year-old boy suffered from headache and gait disturbance. Neuroimaging studies demonstrated a large enhancing cerebellar mass with cystic components compressing the forth ventricle. After complete resection of the tumor, the patient became symptom free. Histological examination on the tumor disclosed glial cells and dysplastic ganglion cells. Although it is a rare tumor, in the appropriate clinical setting, a GG should be considered in the presence of a cerebellar mass with both solid and cystic components on magnetic resonance images in children.

Anaplastic Ganglioglioma in a Middle-aged Woman: A Case Report with a Review of the Literature

We report a case of anaplastic ganglioglioma. A 45-yr-old woman was admitted with a 5-month history of headache and dizziness, both of which progressed slowly. Preoperative magnetic resonance imaging revealed a strong enhancing mass in the left frontal lobe extending to the cingulate gyrus. Adjuvant radiation therapy and chemotherapy were given after gross total resection of the tumor. Histological and immunohistochemical studies showed an anaplastic ganglioglioma. Gangliogliomas of the central nervous system are rather uncommon tumors, and anaplastic ones are extremely rare. The pertinent literature regarding gangliogliomas is reviewed.

Cerebellar Ganglioglioma in an Old Patient

Gangliogliomas could be found anywhere throughout the central nervous system and mainly affect children and young adults during the first three decades of life. Cerebellar gangliogliomas may be rarely found, especially in old ages. Here, we present a case of ganglioglioma of the cerebellum in an old patient. The cystic cerebellar mass was associated with calcifications, intratumoral hemorrhage without ng edema. When a cystic cerebellar mass is associated with calcifications and intratumoral hemorrhage, ganglioglioma should be included in differential diagnosis. Gangliogliomas usually have good prognoses. Radiation therapy should be deferred even in subtotally removed cases.

Ganglioglioma of the Brain Stem: Case Report

Ganglioglioma comprises about 2% of all intracranial neoplasm, however, it is rarely originated from the brain stem. We report a case of ganglioglioma arising from the brain stem. A 15-year-old girl presented with gait disturbance and dysarthria. Magnetic resonance image revealed a high signal intensity mass with cystic cavities in the right cerebellum and the brain stem. The patient underwent subtotal resection and microscopic examination revealed a ganglioglioma. It is suggested that ganglioglioma should be included in the differential diagnosis of posterior fossa tumors, even located in the brain stem.

Gangliogioma of Cerebellar Vermis: Case Report

We present a case of ganglioglioma in the cerebellar vermis. A 2-year-old boy presented with symptoms and signs of raised intracranial pressure and the physical examination revealed large head and developmental delay. Magnetic resonance image demonstrated a hypointense mass in the cerebellar vermis on T1-weighted image with heterogeneous enhancement after gadolinium administration, mimicking a malignant tumor. A gross total resection was performed and pathologic findings demonstrated a ganglioglioma. There was no evidence of recurrence of the tumor during 16 months follow up period.

Immunohistochemical Expression of Synaptophysin in Brain Tumors

BACKGROUND: Perikaryal or perikaryal surface immunostaining for synaptophysin has been posited to distinguish the neoplastic neuronal elements of gangliogliomas from entrapped non-neoplastic neurons in other gliomas of various types. However, recent studies revealed that perikaryal surface immunoreactivity can be seen in the neurons of normal human spinal cords and brains, as well as in the brain tissues around certain non-neuronal lesions. To access the validity of this criterion in the diagnosis of ganglion cell neoplasms, we evaluated patterns of immunostaining of synaptophysin in neuronal, glial and some non-neuroepithelial tumors. METHODS: We selected 104 cases of gangliogliomas, gangliocytomas, central neurocytomas, dysembryoplastic neuroepithelial tumors, astrocytomas, oligodendrogliomas, glioblastomas, a pleomorphic xanthoastrocytoma, meningiomas, arterio-venous malformations, craniopharyngiomas, a foreign body granuloma, temporal lobe epilepsies, and autopsied brains. A representative block including the gray matter was identified for each case, and synaptophysin immunostaining was performed. RESULTS: Perikaryal and perikaryal surface immunoreactivity for synaptophysin was observed in the neurons of various types of lesions. Percentage of perikaryal and perikaryal surface immunoreactivity of the gangliogliomas, glial tumors, and non-neuroepithelial lesions were 100%/93%, 80%/58% and 57%/26%, respectively. CONCLUSIONS: Although synaptophysin positive neurons are found in the ganglioglioma, these patterns are clearly not pathognomonic for glioneuronal tumors.

Cervical Ganglioglioma

Gangliogliomas are benign tumors, composed of neoplastic astrocytes and nerve cells. They are rare, account for 0.4-6.25% of all primary central nervous system neoplasms. Gangliogliomas affect predominantly infants or young adults. Gangliogliomas are preferentially encountered supratentorially, predominantly affecting the temporal lobe. Spinal gangliogliomas affect predominantly the cervical spinal cord. We report a case of cervical ganglioglioma which was successfully removed surgically, with a review of literature.

A Case of Frontal Ganglioglioma Coincidence with Pituitary Adenoma

A case of frontal ganglioglioma coexisting with pituitary adenoma is reported. A 37-year-old male presented with chief complaint of headache for 2 months. A neurological examination of the patient revealed no deficit. Brain MRI showed a well enhanced lobulated mass on left frontal lobe and another homogeneously enhanced mass on intrasellar and suprasellar portion. The frontal mass was grayish in color and rubbery hard in consistency on operative field, having relatively good boundary from the adjacent normal brain tissue. The sellar mass was 3X3cm in size and compressed optic chiasm. Histological diagnosis of frontal mass was ganglioglioma and sella mass showed typical features of pituitary adenoma.