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INTRODUCTION: Present study attempted to analyze seizure freedom and detailed functional outcomes after functional hemispherotomy and utility of hemispherotomy outcome prediction scale (HOPS) scores in predicting outcomes. METHODS: Patients who underwent functional hemispherotomy were analyzed for clinical presentation, neuroimaging, seizure outcomes, and functional outcomes. RESULTS: A total of 76 procedures were performed on 69 patients. Mean age at the surgery was 8 ± 6.1 years. Fourteen patients were < 2 years. Age of onset epilepsy of the cohort was 2.0 ± 3.3 years. All had severe catastrophic epilepsy with multiple daily seizures. All patients had motor deficits with 36 (52%) patients had contralateral dysfunctional hand. Perinatal stroke (49%) was most common substrate followed by cortical malformations (21.7%). Eight patients had contralateral imaging abnormalities. Fifty-nine (86.76%) patients remained seizure free (Engle 1a) at 41 + -20.9 months. HOPS scores were available for 53 patients and lowest seizure outcome was 71% for HOPS score of 4. Lower HOPS scores predicted better seizure outcomes. Cortical malformations operated earlier than 2 years predicted poor seizure outcomes (66.6%). Positive functional outcomes are recorded in 80% of patients with 78% reporting improvement from the pre-surgical level. Five (7.2%) patients underwent shunt surgery. One mortality recorded. CONCLUSIONS: Hemispherotomy has excellent seizure outcomes. Early surgery in cortical malformations appears to be predictor of poorer seizure outcomes. HOPS score is a good tool to predict the seizure outcomes. Hemispherotomy is perceived to improve the Cognitive and functional performance.
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Epilepsia , Hemisferectomia , Humanos , Lactente , Pré-Escolar , Criança , Adolescente , Resultado do Tratamento , Hemisferectomia/métodos , Estudos Retrospectivos , Convulsões/cirurgia , Epilepsia/cirurgiaRESUMO
PURPOSE: To determine the impact of intraoperative magnetic resonance imaging (iMRI) in epilepsy surgeries on the extent of surgical resection and seizure outcome along with its feasibility and limitations. METHODS: Patients with pharmacoresistant epilepsy (PRE), who underwent surgeries in operating theater equipped with high-field 1.5-Tesla MRI, were evaluated for extent of resection, operative time, scanning time, pathologies, resultant extra resection, and seizure outcomes. RESULTS: Thirty-nine patients with mean age of 18 (range: 3-65) years with PRE underwent surgical intervention. Mean duration of epilepsy was 10.2 years. Surgical interventions included tumor resection (31%), resection of focal cortical dysplasia (28%), mesial temporal lobe surgeries (18%), and disconnection surgeries (23%). iMRI alone, apart from navigation and electrophysiology, improved resection rates in 13% (5 out of 39) of these patients. In lesional group, iMRI modified operative strategy resulting in increased resections in 21% (5/23) patients. Complete resection was observed in 87% of patients. iMRI scanning time constituted 25% (mean: 72 ± 21 min) of time spent under anesthesia by the patient. Major and minor complications were observed in 2.5% and 7.5% of patients, respectively. The mean follow-up was 14 months. Favorable postoperative seizure control (Engel Classes I and II) was achieved in 85% and complete seizure freedom was achieved in 77% of patients (Engel Class IA) at 1-year follow-up. CONCLUSIONS: iMRI increases the extent of resection mainly in lesional epilepsy surgeries translating into good seizure outcomes but not found to be much beneficial in prototype mesial temporal sclerosis surgeries and disconnection surgeries.
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Lobectomia Temporal Anterior , Epilepsia/cirurgia , Imageamento por Ressonância Magnética , Lobo Temporal/cirurgia , Adolescente , Adulto , Idoso , Lobectomia Temporal Anterior/métodos , Criança , Pré-Escolar , Eletroencefalografia/métodos , Feminino , Humanos , Lactente , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Adulto JovemRESUMO
OBJECTIVE: Rasmussen encephalitis (RE) is a focal encephalitis, characterized by epilepsia partialis continua (EPC) with or without seizures and progressive unilateral deficits. Imaging characteristics of RE have been rarely described in detail in relation to EPC. So, the study aimed to explore if any relationship exists between the imaging characteristics and the presence or evolution of EPC in patients with RE. METHODS: This retrospective study included 11 patients with RE fulfilling the European consensus statement on RE followed between 2015 and 2020. RESULTS: The mean age for onset of seizures was 12 years (range 2.5-24 years). Seven patients had limb EPCs, two had face EPCs, face, and limb EPC in one, and lingual EPC in one patient. The first MRI was done within 1 day to 1 month of the onset of seizures. It was normal in two patients and showed only cortical atrophy, focal or hemispheric in four patients, caudate atrophy in two, and cortical or subcortical hyperintensity (HI) in six patients. Follow-up MRI, within 3 weeks to 6 months of the onset of EPC (mean 1.6 months) showed paramedian frontal HI with limb EPC in six patients. Insular HI in four patients; two had facial EPCs while lingual EPC and limb EPC with facial EPC was observed in one patient each. SIGNIFICANCE: Fluid-attenuated inversion recovery (FLAIR) HI and focal cortical atrophy on MRI is the most common finding in the early course of RE. T2 and FLAIR hyperintensity in the paramedian frontal or insular cortex may antedate the onset of EPC or may occur simultaneously with EPC.
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Encefalite , Epilepsia Parcial Contínua , Humanos , Pré-Escolar , Criança , Adolescente , Adulto Jovem , Adulto , Epilepsia Parcial Contínua/diagnóstico , Epilepsia Parcial Contínua/etiologia , Estudos Retrospectivos , Encefalite/diagnóstico , Encefalite/diagnóstico por imagem , Convulsões , Imageamento por Ressonância Magnética , Atrofia , EletroencefalografiaRESUMO
In this study, we have evaluated the underlying aetiologies, yield of genetic testing and long-term outcomes in patients with early-infantile developmental and epileptic encephalopathies. We have prospectively studied patients with seizure onset before 3 months of age. Based on the clinical details, neuroimaging, metabolic testing and comprehensive genetic evaluation, patients were classified into different aetiological groups. The phenotypic differences between genetic/unknown groups and remaining aetiologies were compared. Factors that could affect seizure control were also assessed. A total of 80 children (M:F ratio-1.5:1) were recruited. The median seizure onset age was 28 days (range, 1-90 days). The aetiologies were confirmed in 66 patients (83%). The patients were further classified into four aetiological groups: genetic (50%), structural (19%), metabolic (14%; all were vitamin responsive) and unknown (17%). On comparing for the phenotypic differences between the groups, children in the 'genetic/unknown' groups were more frequently observed to have severe developmental delay (Odds Ratio = 57; P < 0.0001), autistic behaviours (Odds Ratio = 37; P < 0.0001), tone abnormalities (Odds Ratio = 9; P = 0.0006) and movement disorder (Odds Ratio = 19; P < 0.0001). Clonic seizures were more common in the vitamin responsive/structural groups (Risk Ratio = 1.36; P = 0.05) as compared to patients with 'genetic/unknown' aetiologies. On the contrary, vitamin responsive/structural aetiology patients were less likely to have tonic seizures (Risk Ratio = 0.66; P = 0.04). Metabolic testing was diagnostic in three out of 41 patients tested (all three had biotinidase deficiency). MRI was abnormal in 35/80 patients (malformation observed in 16/35; 19/35 had non-specific changes that did not contribute to underlying aetiology). A molecular diagnosis was achieved in 53 out of 77 patients tested (69%). Next-generation sequencing had a yield of 51%, while microarray had a yield of 14%. STXBP1 was the most common (five patients) single-gene defect identified. There were 24 novel variants. The mean follow-up period was 30 months (range, 4-72 months). On multivariate logistic regression for the important factors that could affect seizure control (seizure onset age, time lag of first visit to paediatric neurologist and aetiologies), only vitamin responsive aetiology had a statistically significant positive effect on seizure control (P = 0.02). Genetic aetiologies are the most common cause of early-infantile developmental and epileptic encephalopathies. Patients in the genetic/unknown groups had a more severe phenotype. Patients with vitamin responsive epilepsies had the best probability of seizure control.
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INTRODUCTION: Post operative pituitary apoplexy after partial resection of a giant pituitary adenoma is mostly fatal, despite early and best management. Pathophysiology, clinical presentation and preoperative consideration toward prevention of apoplexy are discussed. MATERIAL AND METHODS: Patients with post operative pituitary apoplexy were critically reviewed for clinical presentation, endocrine status, preoperative imaging and post operative course with outcome. Operative findings and histopathology were correlated. RESULTS: Thirteen patients over 11 years with a mean age of 36 years were reviewed. All patients had giant pituitary adenomas. Four patients had functional adenomas. All patients were optimized for endocrine status before surgery. Twelve patients underwent transsphenoidal excision of the tumor. Only partial excision could be achieved in all cases. Deterioration of consciousness (9), visual deterioration (3), delayed reversal and excessive bleeding (1) were the primary indicators toward apoplexy. Ten patients were reexplored within 24 h of first surgery. All except one were explored transcranially a second time. Twelve patients died with variable post operative course. Hypothalamic dysfunction and dyselectolytemia (9) were leading causes of death, followed by meningitis and raised intracranial pressure. CONCLUSIONS: Post operative pituitary apoplexy is associated with high mortality, despite early and best management. Partial resection of the giant pituitary adenoma is directly responsible for post operative apoplexy. Maximum possible resection of the tumor by suitable exposure should be the optimal goal of surgery. Surgical exposure, either transcranial or transsphenoidal, should be dictated by tumor configuration on preoperative imaging. Endocrine status, histology of the tumor and clinical presentation do not appear to contribute to post operative pituitary apoplexy.
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Adenoma/cirurgia , Apoplexia Hipofisária/prevenção & controle , Neoplasias Hipofisárias/cirurgia , Complicações Pós-Operatórias/prevenção & controle , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Cuidados Pré-Operatórios/métodos , Estudos Retrospectivos , Análise de Sobrevida , Resultado do Tratamento , Adulto JovemRESUMO
Brain Source Localization (BSL) using Electroencephalogram (EEG) has been a useful noninvasive modality for the diagnosis of epileptogenic zones, study of evoked related potentials, and brain disorders. The inverse solution of BSL is limited by high computational cost and localization error. The performance is additionally limited by head shape assumption and the corresponding harmonics basis function. In this work, an anatomical harmonics basis (Spherical Harmonics (SH), and more particularly Head Harmonics (H2)) based BSL is presented. The spatio-temporal four shell head model is formulated in SH and H2 domain. The anatomical harmonics domain formulation leads to dimensionality reduction and increased contribution of source eigenvalues, resulting in decreased computation and increased accuracy respectively. The performance of spatial subspace based Multiple Signal Classification (MUSIC) and Recursively Applied and Projected (RAP)-MUSIC method is compared with the proposed SH and H2 counterparts on simulated data. SH and H2 domain processing effectively resolves the problem of high computational cost without sacrificing the inverse source localization accuracy. The proposed H2 MUSIC was additionally validated for epileptogenic zone localization on clinical EEG data. The proposed framework offers an effective solution to clinicians in automated and time efficient seizure localization.
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Algoritmos , Epilepsia , Encéfalo , Eletroencefalografia/métodos , Epilepsia/diagnóstico , Cabeça , HumanosRESUMO
INTRODUCTION: Application of stereotaxy frame for gamma knife under local anesthesia creates anxiety and phobia in many patients. Vasovagal syncopal attacks and events of bradycardia are not infrequently seen. We tried to analyse the various factors affecting these events and set standards and safety recommendations for the procedure. MATERIALS AND METHODS: This prospective study was carried out in Gamma Knife Centre, AIIMS, New Delhi over a 6 month period between June, 2021- November, 2021. All patients coming to Gamma knife for various etiologies were asked to fill Hamilton Anxiety Rating Scale (HAM-A) before frame application. Baseline pulse rate was evaluated and with every pin application pulse rate was recorded. A VAS (visual analogue scale) for pain was also administered after the procedure. Twenty percent fall in pulse rate were considered as significant bradycardia. Intraprocedural syncopal events were also recorded. RESULTS: A total of 141 patients were studied of which 6% patients had intraprocedural syncopal attacks requiring medical treatment while 32% patients had bradycardia. Having previous surgery, having breakfast before the procedure, duration of pin application, quantity of local anaesthetia, primary diagnosis, sex of patients were not found to alter the syncope or bradycardia events. Patients who were found anxious (score > 18 on HAM-A) had a significantly higher risk of having a procedural event. Previous history of syncope was also associated with 1.7 times more chances of developing Intraprocedural bradycardia. CONCLUSIONS: Frame application for gamma-knife is a relatively safe procedure however procedure rooms should be equipped with emergency drugs and fluids for management of syncope. A Simple anxiety questionnaire can predict 'at risk' patients' for procedural events.
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Doenças do Sistema Nervoso , Radiocirurgia , Ansiedade/etiologia , Bradicardia/etiologia , Encéfalo , Seguimentos , Humanos , Doenças do Sistema Nervoso/etiologia , Estudos Prospectivos , Radiocirurgia/efeitos adversos , Radiocirurgia/métodos , Estudos Retrospectivos , Síncope/etiologia , Resultado do TratamentoRESUMO
Rasmussen's encephalitis (RE) is a rare chronic inflammatory disease of the brain resulting in unilateral hemispheric atrophy with drug-resistant focal epilepsy associated with a variable degree of progressive hemiparesis and cognitive decline. The precise etiology of RE is unknown but presumed to have a neuroinflammatory pathobiological basis. Only surgery halts progression of the disease, but may occur at the expense of a fixed but otherwise inevitable neurological deficit. Therefore, the question of medical management is an important consideration. Reports of rituximab use in patients with RE were presented at the American Epilepsy Society annual meeting in 2008. Good published evidence for its usage has been very slow to emerge since then. However, rituximab continues to be listed in discussions of treatment options for patients with RE, though other monoclonal antibodies have since been used with comparable outcomes. We describe a series of nine patients including two with adult-onset RE. Rituximab was used early in the disease course (range 1-108 months; mean 32 months). Of nine patients with RE, there was significant benefit in their seizure burden with rituxamab. Seizure freedom occurred in 3 patients. Epilepsia partialis continua (EPC) was present in 4/9 and no focal motor deficit noted in 4/9. No progression of a neurological deficit was present in 2/9 and evidence of progression with neuroimaging was terminated with rituxamab in 5/9 supporting early use of rituxamab in patients with RE.
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Objective: Although rare, temporal encephalocele is an important causative agent in surgically remediable drug-refractory epilepsy. The ideal treatment for temporal encephalocele remains unclear with a variety of resective surgeries recommended. Here, we analyse patient data on temporal encephalocele with a view to highlighting diagnostic clues and management strategies. Methods: Comprehensive databases at Deenanath Mangeshkar Hospital, Pune from January 2015 to June 2019 were reviewed for this observational study. Of 107 temporal lobe epilepsy surgery patients, nine individuals with temporal encephalocele were identified, who formed the study cohort. Their clinical, neuropsychological, EEG, imaging and long-term outcome data were analysed. Results: The study cohort consisted of seven males and two females with a mean age of 22 years. Epilepsy onset age varied from 4.5 to 19 years. Seven patients had focal non-motor seizures with impaired awareness, while two patients had focal motor seizures. Temporal encephalocele detection by MRI was reported in only two patients, and was missed in seven individuals. Three patients underwent standard anterior temporal lobectomy while the remaining six underwent resection of the temporal encephalocele with surrounding temporal pole. Eight patients showed Engel Class I outcome and one showed Class IIa outcome after a mean follow-up duration of 27 months (17-44 months). Histopathology confirmed gliosis in seven, hippocampal sclerosis type I in one and suspicious dyslamination with prominent gliosis in one patient. Six of eight patients reported an improvement in their psychological state (mood, anxiety and motivation) over time. Significance: A careful review of MRI in patients with temporal lobe epilepsy is necessary, followed by investigations for the presence of an encephalocele. When temporal lobe epilepsy is associated with encephalocele, tailored resection of the encephalocele and the surrounding temporal pole, sparing mesial temporal structures, demonstrates excellent long-term clinical and neuropsychological outcome.
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Epilepsia do Lobo Temporal , Adolescente , Adulto , Criança , Pré-Escolar , Eletroencefalografia , Encefalocele/complicações , Encefalocele/diagnóstico , Encefalocele/cirurgia , Epilepsia do Lobo Temporal/diagnóstico , Epilepsia do Lobo Temporal/etiologia , Epilepsia do Lobo Temporal/cirurgia , Feminino , Gliose/complicações , Humanos , Índia , Imageamento por Ressonância Magnética , Masculino , Estudos Retrospectivos , Convulsões/etiologia , Resultado do Tratamento , Adulto JovemRESUMO
INTRODUCTION: The present study aims to describe epilepsy surgery outcomes in the pediatric population from a tertiary center in India. METHODOLOGY: Children less than 18 years who underwent epilepsy surgery between June 2015 and December 2019 for whom at least a 1-year follow-up was available, were retrospectively evaluated for clinical presentation, radiology, surgical intervention, and seizure outcomes. OBSERVATIONS: Out of a total of 355 epilepsy surgeries performed, 242 were in the pediatric group (140 males, 80 females). The mean age at surgery was 9.4 years ±4.8 years (range 4 months-18 years). The mean duration of epilepsy was 5.64 years ±3.91 (range 2 months-17 years). 126 patients experienced daily seizures, 45 weekly and 39 reported monthly seizures. Six had refractory status epilepticus. All the patients were on multiple anti-epileptic drugs (AEDs): the mean number of AEDs was 3.27 ± 0.98 (range 2-7 AEDs). Focal seizure was the most common seizure type seen in 72.27% of children (159/220). The most frequent etiology was focal cortical dysplasia (70), followed by bilateral parieto-temporo-occipital gliosis (48). All the patients underwent standard pre-surgical evaluation. Eleven patients needed stage 2 evaluation (intracranial EEG). The different surgeries performed were electrocorticography (ECOG) and navigation-guided resection (65), anterior temporal lobectomy and hippocampectomy (ATLAH) (48), functional hemispherotomy (39), callosotomy (28), disconnection surgeries (16), and multilobar resection (12). Twelve patients underwent more than one surgery. The patients were followed up between a minimum of 12 months and a maximum of 66 months (median 32 months; IQR 20 months). Engel class 1 outcome was observed in 81.38% in definitive surgeries. Outcomes of temporal lobe (TL) surgeries (92.3%) were better compared to hemispheric (87.17%) and extratemporal lobe (ETL) surgeries (75.32%). We encountered unexpected transient motor deficits in 2 patients and culture-proven meningitis in 8 patients. Post-surgery drug freedom (P-value 0.003) was the most important factor for better developmental, cognitive and behavioral outcomes. CONCLUSION: Epilepsy surgeries are safe and seizure outcomes are excellent in properly selected cases with thorough presurgical evaluation. Early referral to a tertiary epilepsy center is needed for timely intervention.
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Epilepsia Resistente a Medicamentos , Epilepsia , Malformações do Desenvolvimento Cortical , Criança , Epilepsia Resistente a Medicamentos/complicações , Epilepsia Resistente a Medicamentos/cirurgia , Eletroencefalografia , Epilepsia/etiologia , Epilepsia/cirurgia , Feminino , Humanos , Lactente , Masculino , Malformações do Desenvolvimento Cortical/complicações , Estudos Retrospectivos , Convulsões/complicações , Resultado do TratamentoRESUMO
BACKGROUND: To study surgical outcomes in pharmaco-resistant epilepsy associated with posterior cortex ulegyria secondary to perinatal insults. METHODS: A cohort was analysed for clinico-radiological charectaristics, surgical interventions and seizure outcomes. OBSERVATIONS: A total of 38 patients underwent surgery, divided as group A - curative surgeries (n = 20) and group B - palliative surgeries (n = 18). Mean age of onset of epilepsy in group A was 5.2 ± 3.4 years against 2.7 ± 2.4 years in group B (p < 0.01). Electroclinical Lennox Gastaut Syndrome was encountered in 9/20 patients in group A, against all 18 patients in group B. Disabling reflex epilepsy was seen in 10 (26 %) patients. Interictal electrophysiology localized in the posterior cortex in all patients in group A, but ictal onsets contributed in only 7/20 patients. Nine patients from group A had unilateral parieto-occipital ulegyria while bilateral in 11/20 patients, and 16/18 from group B. Group A patients underwent parieto-occipital resection (n = 10) and temporo-parieto-occipital disconnection (n = 10) while group B underwent complete corpus callosotomy (n = 18). In group A, Engel Ia outcome was achieved in 15/20 patients (75 %) at mean follow up of 23.5 ± 7.9 months. Group B patients experienced cessation of head drops in all 18 patients, with two-third reduction in seizure frequency at 29.2± 12.4 months of mean follow up. Reflex seizures responded completely in both groups. CONCLUSIONS: Epilepsy surgeries for posterior cortex ulegyria results in excellent seizure outcomes. Corpus callosotomy appears highly effective as a palliation for head drop as well as disabling reflex seizures in a well selected cohort.
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Epilepsia Resistente a Medicamentos , Córtex Cerebral/diagnóstico por imagem , Córtex Cerebral/cirurgia , Criança , Pré-Escolar , Epilepsia Resistente a Medicamentos/diagnóstico por imagem , Epilepsia Resistente a Medicamentos/etiologia , Epilepsia Resistente a Medicamentos/cirurgia , Eletroencefalografia , Humanos , Lactente , Imageamento por Ressonância Magnética , Resultado do TratamentoRESUMO
INTRODUCTION: Status epilepticus (SE) is one of the most common medical emergencies, requiring urgent treatment; nearly 30 % patients develop refractory SE. The role of epilepsy surgery (ES) for refractory SE however remains unclear with empirical evidence limited to single case reports and small case series. The aim of the present study was to determine the clinical presentation, imaging characteristics and outcome of children with refractory SE who underwent emergency ES for refractory SE. MATERIAL AND METHOD: Patients who had SE, failed to respond to escalating medical treatment of SE with/ without pharmacological suppression therapy, and eventually underwent ES were included. RESULTS: There were ten children, 7 boys and 3 girls (range 6 months to 14 years). The age of onset of epilepsy varied from day 2 of life to 12.8 years. The duration of SE prior to surgery was 2-6 days (mean 3.7 days). Four patients had hemimegalencephaly, 3 had focal cortical dysplasia, 2 had Rasmussen's encephalitis, and one had hemispheric porencephalic cyst. The time interval between onset of seizures and ES ranged from 2 months to 8 years (mean 3.1 year). Seven patients underwent hemispherotomy, resection of dysplasia in two and temporo-parieto-occipital disconnection in one. Nine had Engel I outcome and Engel IIIa in one, at follow up of 12-44 months (mean 31 months). CONCLUSION: Emergency ES is an effective treatment option for termination of refractory SE in children with structural pathology, after failure of medical treatment. Patients with refractory SE with focal or hemispheric structural abnormality on MRI, and concordant semiology with/without concordant EEG can be surgical candidates with or without invasive monitoring.
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Epilepsia Resistente a Medicamentos , Epilepsia , Hemisferectomia , Malformações do Desenvolvimento Cortical , Estado Epiléptico , Criança , Epilepsia Resistente a Medicamentos/complicações , Epilepsia Resistente a Medicamentos/diagnóstico por imagem , Epilepsia Resistente a Medicamentos/cirurgia , Eletroencefalografia/efeitos adversos , Epilepsia/tratamento farmacológico , Feminino , Hemisferectomia/métodos , Humanos , Imageamento por Ressonância Magnética , Masculino , Malformações do Desenvolvimento Cortical/cirurgia , Estudos Retrospectivos , Estado Epiléptico/complicações , Estado Epiléptico/diagnóstico por imagem , Estado Epiléptico/cirurgia , Resultado do TratamentoRESUMO
OBJECTIVE: To study the factors responsible for failure of hemispherotomy and outcomes of revision surgery. The effect of the surgeon's learning curve on failures was also analyzed. METHODS: Forty consecutive patients, who underwent functional hemispherotomy through a 4-year period, from the inception of the single-surgeon epilepsy surgery program, were analyzed. RESULTS: A total of 47 functional hemispherotomies were performed in the study period in 40 patients (7 revision surgeries on 6 patients). Mean age of the cohort was 9.45 ± 14.84 years and it included 7 infants (<2 years). Of the 9 patients (23.5%) who failed the first procedure, 6 qualified for revision surgery, all of whom belonged to the cohort of the first 15 patients treated during the first 2 years of the program. Hemimegalencephaly was the most common disease (n = 4). Ipsilateral temporal stem (n = 3), frontobasal connections (n = 2), splenium of corpus callosum (n = 2), and posterior insula (n = 2) were residual undisconnected substrates identified for revision on imaging. The substrates for failure were obvious in 5/6 patients and resulted from incomplete disconnection, implying surgical inadequacy. At the mean follow-up of 30 ± 13.17 months (range, 13-55 months), 35 of 40 patients (87.5%) remained seizure free (Engel class Ia), including 4/6 patients who underwent redo surgery. Revision did not benefit the remaining 2 patients (Engel class III). There was no mortality. CONCLUSIONS: Surgical revision is more common in hemimegalencephaly and in the early days of a surgical program. Affirmative neuroimaging improves the outcomes of subsequent revision surgery.
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Corpo Caloso/cirurgia , Epilepsia Resistente a Medicamentos/cirurgia , Hemisferectomia/métodos , Reoperação , Adolescente , Adulto , Criança , Pré-Escolar , Epilepsia Resistente a Medicamentos/diagnóstico por imagem , Epilepsia Resistente a Medicamentos/etiologia , Epilepsia Resistente a Medicamentos/fisiopatologia , Eletroencefalografia , Encefalite/complicações , Encefalite/diagnóstico por imagem , Feminino , Gliose/complicações , Gliose/diagnóstico por imagem , Hemimegalencefalia/complicações , Hemimegalencefalia/diagnóstico por imagem , Humanos , Lactente , Imageamento por Ressonância Magnética , Masculino , Malformações do Desenvolvimento Cortical/complicações , Malformações do Desenvolvimento Cortical/diagnóstico por imagem , Espasmos Infantis/complicações , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/diagnóstico por imagem , Falha de Tratamento , Adulto JovemRESUMO
OBJECTIVES: To analyze the outcome of a pediatric population with traumatic basal ganglia hematoma (TBGH). METHODOLOGY: Patients < 15 years of age with TBGH were studied for mode of injury, severity of injury on admission, Glasgow coma scale (GCS) score on admission, radiology, intervention and overall outcome. OBSERVATIONS: Twenty-one patients (male:female = 4:1) with a mean age of 7.2 ± 3.7 years (range 0.33-15 years) were studied. High-velocity trauma (52%) followed by fall from height (38%) were the leading causes. Seventy-six percent of the patients had severe head injury. The mean GCS score on admission was 6.0 ± 2.5 (range 3-12), while the mean GCS score of the group with severe head injury was 4.81 ± 1.7 (range 3-7). Eleven (52.4%) patients had isolated basal ganglia hematoma while 10 (47.6%) had other associated intracranial injuries. Only 3 patients required surgical interventions. Eleven patients (52.4%) expired during their hospital stay. At discharge, 9 (42.9%) had a poor, nonfunctional outcome (Glasgow outcome scale, GOS 2, 3). CONCLUSION: The severity of head injuries and GCS score on admission mainly determined the ultimate outcome in pediatric TBGH. The mode of injury or associated intracranial injuries did not change the outcome. The presence of TBGH in severely head-injured patients worsens the prognosis and outcome.
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Hemorragia dos Gânglios da Base/diagnóstico por imagem , Hemorragia dos Gânglios da Base/mortalidade , Lesões Encefálicas/diagnóstico por imagem , Lesões Encefálicas/mortalidade , Adolescente , Hemorragia dos Gânglios da Base/fisiopatologia , Lesões Encefálicas/fisiopatologia , Criança , Pré-Escolar , Feminino , Seguimentos , Escala de Resultado de Glasgow , Mortalidade Hospitalar , Humanos , Lactente , Masculino , Valor Preditivo dos Testes , Prognóstico , Recuperação de Função Fisiológica , Tomografia Computadorizada por Raios XRESUMO
Focal neuronal lipofuscinosis is a unique neuronal pathology, characterised by accumulation of lipofuscin within dysmorphic neurons. We report a case of 12-year-old female with drug resistant epilepsy since one and a half years of age. MRI brain showed right frontal dysplasia, and PET showed right frontal hypometabolism. She underwent electrocorticography-guided resection of the lesion. Histopathology revealed cortical dyslamination with several hypertrophic dysmorphic neurons showing intracytoplasmic granular accumulation of lipofuscin which was positive for Periodic acid-Schiff, Luxol fast blue, and autofluorescent. Ultrastructural examination revealed intracytoplasmic, non-membrane bound, electron dense material with characteristics of lipofuscin filling the neuronal soma. On immunohistochemistry, the neurons showed ring-like non-phosphorylated and phosphorylated neurofilaments enveloping the lipofuscin material, few being positive for ubiquitin. It is important to be aware of this rare entity as it can be associated with family history of seizures and has a distinct pathobiology.
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Gyratory seizures (GS) are rare and have been reported in focal (frontal and temporal) as well as generalized epilepsies. The exact neuroanatomical substrate of gyration during a seizure is not clearly understood, but is thought to be due to the involvement of the basal ganglia. The direction of gyration occurring without head version is thought to have ipsilateral cerebral origins, while gyrational seizures preceded by forced head version are suggestive of seizure onset contralateral to the direction of gyration or the presence or absence of forced head version is thought to have important implications for lateralization of seizure origins from the direction of gyration. This case describes gyratory seizures in a young boy with a temporal encephalocele. Report of MRI brain initially indicated no abnormalities, but careful review revealed a left temporal encephalocele. PET scan showed left temporal hypometabolism. The patient underwent a left anterior temporal resection with amygdalohippocampectomy (ATL + AH) and is seizure-free for 18 months. Temporal encephalocele is the most commonly iatrogenic but may also be spontaneous, post-traumatic, or in relation to chronic otitis media. This case suggests that gyratory seizures may be a unique presentation of temporal encephalocele and this possibility warrants investigation in patients with medically refractory epilepsy.
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Radiation is a well-known etiology for many CNS tumors, including meningiomas, sarcomas and gliomas. However, occurrence of pituitary adenoma after radiation exposure is rare. We report a 21-year-old man with a pituitary adenoma, who was treated with post-operative radiotherapy for pontine glioma at 6 years of age. The role of radiation exposure in the etiology of pituitary adenoma is discussed, along with a literature review of radiation-induced tumors.
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Neoplasias Induzidas por Radiação , Neoplasias Hipofisárias/etiologia , Prolactinoma/etiologia , Radioterapia de Alta Energia/efeitos adversos , Neoplasias do Tronco Encefálico/radioterapia , Glioma/radioterapia , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Neoplasias Hipofisárias/diagnóstico por imagem , Prolactinoma/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Adulto JovemAssuntos
Falha de Equipamento , Hidrocefalia/patologia , Hidrocefalia/terapia , Imageamento por Ressonância Magnética , Derivação Ventriculoperitoneal/efeitos adversos , Adolescente , Algoritmos , Criança , Pré-Escolar , Serviço Hospitalar de Emergência , Feminino , Humanos , Hidrocefalia/complicações , Lactente , Masculino , Valor Preditivo dos Testes , Reprodutibilidade dos TestesRESUMO
OBJECTIVE: Gangliogliomas (GG) are benign, primary neoplasms most commonly noted in young adults. Intraventricular location is rare. We report a case of a multicentric intraventricular GG posing diagnostic and therapeutic challenges and in addition provide a detailed literature review of intraventricular GG. METHODS: A 15-year-old girl presented with features of raised intracranial pressure of short duration. Imaging revealed 2 separate lesions situated in the anterior and posterior third ventricle with hydrocephalus. The patient underwent ventriculoperitoneal shunt insertion, followed by excision of lesions in 2 stages. Both specimens revealed features of GG and the patient had an uneventful recovery. For the literature review, only pure intraventricular GG were included and intraventricular extensions of paraventricular/extraventricular GG were not considered. A brief comparison of clinicoradiologic characteristics between intraventricular and parenchymal GG is provided. RESULTS: Including ours, 21 cases were identified. Male/female ratio was 1.3:1. Peak age of occurrence was the third to fourth decade. Features of increased intracranial pressure were the most common presenting features and seizures were noted in one quarter of cases. Gross total resection was achieved in 90% and recurrences and mortality were noted in 10% each. CONCLUSIONS: Intraventricular GG are rare tumors. Complete surgical excision achieves excellent results. The role of adjuvant therapy is controversial.
Assuntos
Neoplasias do Ventrículo Cerebral/diagnóstico , Neoplasias do Ventrículo Cerebral/cirurgia , Ganglioglioma/diagnóstico , Ganglioglioma/cirurgia , Hidrocefalia/cirurgia , Terceiro Ventrículo , Derivação Ventriculoperitoneal , Adolescente , Adulto , Neoplasias do Ventrículo Cerebral/complicações , Neoplasias do Ventrículo Cerebral/diagnóstico por imagem , Neoplasias do Ventrículo Cerebral/patologia , Meios de Contraste , Feminino , Ganglioglioma/complicações , Ganglioglioma/diagnóstico por imagem , Ganglioglioma/patologia , Humanos , Hidrocefalia/etiologia , Hipertensão Intracraniana/etiologia , Imageamento por Ressonância Magnética , Masculino , Recidiva Local de Neoplasia/diagnóstico por imagem , Recidiva Local de Neoplasia/prevenção & controle , Convulsões/etiologia , Terceiro Ventrículo/patologia , Terceiro Ventrículo/cirurgia , Tomografia Computadorizada por Raios X/métodosRESUMO
AIMS AND OBJECTIVES: Leukocytosis after intracranial surgery may create concern about possible infection, especially when associated with fever. Knowledge of the expected degree of leukocytosis after surgery would assist in the interpretation of leukocytosis. It was hypothesized that the degree of leukocytosis after intracranial surgery correlated with the extent of brain damage inflicted during the surgery. MATERIALS AND METHODS: In this prospective study conducted over 6 months, consecutive patients undergoing either elective resections of brain tumors (having significant collateral brain damage) or aneurysm clipping (with minimal collateral brain damage) were studied. Total blood leukocyte count was checked daily in the morning for the first five postoperative days in both the groups. The mean of the leukocyte count ratio (postoperative leukocyte count/preoperative leukocyte count) on each day was calculated for each group. RESULTS: There were 76 patients, 46 in the test group and 30 controls. Both groups were well matched in age, sex, duration of surgery, and intraoperative fluid balance. The mean leukocyte count ratio on POD1 in the tumor group was significantly higher (1.87) as compared to 1.1 in the aneurysm group (P = 0.001). This difference in the leukocyte count ratio between the groups was maintained on the second and third postoperative days, with decreasing level of significance after the third day. CONCLUSIONS: This study shows that intraoperative brain injury is associated with leukocytosis in the immediate postoperative period. This can assist in the interpretation of leukocytosis after intracranial surgeries and could be a quantitative marker for brain injury in patients undergoing intracranial surgery.