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Nasal Glioma (NG) represents a rare congenital abnormality of the neonate, which can be associated with skull defects or even a direct communication to the central nervous system. MRI serves valuable information for differentiation from encephalocele, dermoid cyst and congenital hemangioma. Complete resection remains the treatment of choice. We present two cases of NG, which were both suspected during prenatal ultrasound and MRI. In the first case, postnatal MRI showed a transcranial continuity. Mass excision was performed and the defect was covered by a glabellar flap allowing a good cosmetic result. Postnatal MRI excluded a trans-glabellar communication in the second case. After surgical excision, the resulting skin defect was covered with a full thickness skin graft harvested from the right groin. In cases of NGs complete resection and cosmetic appealing results can be achieved and might necessitate a multidisciplinary approach.
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Hemangioma , Enfermedades Nasales , Femenino , Humanos , Recién Nacido , Imagen por Resonancia Magnética , EmbarazoRESUMEN
STUDY OBJECTIVES: Self-reported perception of sleep often differs from objective sleep study measures, but factors predicting the discrepancy between self-reported and objective sleep parameters are controversial, and a comparison of laboratory vs ambulatory polysomnography (PSG) is lacking. METHODS: We retrospectively analyzed PSGs conducted between 2012 and 2016. Linear regression was applied to predict the discrepancy between self-reported and objective sleep parameters (total sleep time, sleep efficiency, sleep latency, using age, sex, arousal index, type of sleep disorder, and PSG type [laboratory vs ambulatory] as regressors). RESULTS: A total of 303 PSGs were analyzed (49% women, median age 48 years), comprising patients with insomnia (32%), sleep-related breathing disorders (27%), sleep-related movement disorders (15%), hypersomnia/narcolepsy (14%), and parasomnias (12%). Sleep disorder was the best predictor of discrepancy between self-reported and objective total sleep time, and patients with insomnia showed higher discrepancy values compared to all other patient groups (P < .001), independent of age and PSG type (P > .05). Contributory effects for higher discrepancy values were found for lower arousal index. Patients with insomnia underestimated both total sleep time (median discrepancy: 46 minutes, P < .001) and sleep efficiency (median discrepancy: 11%, P < .001). No significant predictor for discrepancy of sleep latency was found. CONCLUSIONS: Misperception of sleep duration and efficiency is common in sleep lab patients, but most prominent in insomnia, independent of age, sex, or laboratory vs ambulatory recording setting. This underlines the role of PSG in patients with a clinical diagnosis of insomnia and its use in cognitive behavioral therapy.
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Trastornos del Inicio y del Mantenimiento del Sueño , Sueño , Femenino , Humanos , Masculino , Persona de Mediana Edad , Percepción , Estudios Retrospectivos , AutoinformeRESUMEN
Here we present a case of a patient who got trapped in an elevator; on initial evaluation patient was found with bradycardia; on further evaluation electrocardiogram (EKG) showed new onset 2nd-degree Mobitz type 2 AV block. On admission patient developed ischemic changes on EKG and troponin elevation. Transthoracic echocardiogram showed reduced ejection fraction as well as apical inferior, anterior, lateral, and septal hypokinesia. Coronary angiography showed nonobstructive coronary artery disease and ventriculogram demonstrated anterolateral and apical hypokinesia suggesting takotsubo cardiomyopathy (TCM). Atrioventricular block (AV) is rarely seen as initial presentation of TCM and has a prevalence of about 2.9%. AV block during early presentation of TCM poses a therapeutic dilemma with regard to the timing and the need to place a temporary or permanent pacemaker. The decision to place a permanent pacemaker may be on a case-by-case basis and more research is needed on formulating standardized recommendations in patients with TCM and conduction tissue abnormalities.
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Right ventricular loading/pressure influences left ventricular function because the two ventricles pump in series and because they are anatomically arranged in parallel, sharing the common ventricular septum. Flattening of the interventricular septum detected during echocardiographic examination is called D-shaped left ventricle. We present a case of an elderly male of African descent, who presented with increased shortness of breath. Transthoracic echocardiogram showed flattening and left sided deviation of interventricular septum causing a decreased size in left ventricle, secondary to volume/pressure overload in the right ventricle. While patient received hemodialysis therapy and intravascular volume was removed, patient blood pressure was noted to increase, paradox. Repeated transthoracic echocardiogram demonstrated less left deviation of interventricular septum compared with previous echocardiogram. We consider that it is important for all physicians to be aware of the anatomic and physiologic implication of D-shaped left ventricle and how right ventricle pressure/volume overload affects its function and anatomy.
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Thrombotic thrombocytopenic purpura (TTP) is a part of a spectrum of thrombotic microangiopathy syndromes which are mainly characterized by platelet aggregation causing microangiopathic hemolytic anemia, thrombocytopenia and microvascular occlusion. In literature, very few cases expressing a direct association between pre-existing Grave's disease and TTP have been described. A 37-year-old African-American woman with past medical history of Grave's disease and polysubstance abuse who presented with complaints of dyspnoea at rest and chest pain was diagnosed to have TTP on further evaluation. Patient also showed severely elevated thyroid hormones and suppressed thyroid stimulating hormone levels indicating severe thyrotoxicosis. Initiation of prompt management of TTP and thyrotoxicosis led to a favorable patient outcome. In conclusion, patients presenting with thyrotoxicosis, thrombocytopenia and microangioapthic hemolytic anemia without an alternative cause should be treated and screened for TTP due to the high fatality associated with untreated or untimely detection of this disease.
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BACKGROUND: Glioblastoma multiforme (GBM) is a highly aggressive tumor of the central nervous system with a dismal prognosis for affected patients. Aberrant protein kinase C (PKC) signaling has been implicated in gliomagenesis, and a member of the PKC-activated protein kinase D (PRKD) family, PRKD2, was identified as mediator of GBM growth in vitro and in vivo. METHODS: The outcome of PRKD2 silencing and pharmacological inhibition on glioma cell proliferation was established with different glioma cell lines. Western blotting, senescence assays, co-immunoprecipitation, fluorescence activated cell sorting, quantitative PCR, and immunofluorescence microscopy were utilized to analyze downstream signaling. RESULTS: RNA-interference (21-mer siRNA) and pharmacological inhibition (CRT0066101) of PRKD2 profoundly inhibited proliferation of p53(wt) (U87MG, A172, and primary GBM2), and p53(mut) (GM133, T98G, U251, and primary Gli25) glioma cells. In a xenograft experiment, PRKD2 silencing significantly delayed tumor growth of U87MG cells. PRKD2 silencing in p53(wt) and p53(mut) cells was associated with typical hallmarks of senescence and cell cycle arrest in G1. Attenuated AKT/PKB phosphorylation in response to PRKD2 silencing was a common observation made in p53(wt) and p53(mut) GBM cells. PRKD2 knockdown in p53(wt) cells induced upregulation of p53, p21, and p27 expression, decreased phosphorylation of CDK2 and/or CDK4, hypophosphorylation of retinoblastoma protein (pRb), and reduced transcription of E2F1. In p53(mut) GM133 and primary Gli25 cells, PRKD2 silencing increased p27 and p15 and reduced E2F1 transcription but did not affect pRb phosphorylation. CONCLUSIONS: PRKD2 silencing induces glioma cell senescence via p53-dependent and -independent pathways.
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Neoplasias Encefálicas/metabolismo , Senescencia Celular/fisiología , Glioma/metabolismo , Proteínas Quinasas/metabolismo , Proteína p53 Supresora de Tumor/metabolismo , Animales , Western Blotting , Línea Celular Tumoral , Citometría de Flujo , Silenciador del Gen , Xenoinjertos , Humanos , Inmunoprecipitación , Ratones , Microscopía Fluorescente , Proteína Quinasa D2 , Interferencia de ARN , Reacción en Cadena en Tiempo Real de la Polimerasa , Transducción de Señal/fisiología , TransfecciónRESUMEN
Glioblastoma multiforme (GBM) is the most common malignant primary brain tumor and is invariably fatal to affected patients. Oxysterols belong to a class of bioactive lipids that are implicated in neurological disease and are associated with various types of cancer. Here, we investigated expression and transcriptional regulation of cholesterol 25-hydroxylase (CH25H) in human U87MG and GM133 glioblastoma cell lines. We demonstrate that in both cell lines transcription and translation of CH25H are increased in response to TNFα and IL1ß. In parallel, both cell lines upregulate 25-hydroxycholesterol (25-OHC) synthesis and secretion to levels comparable to bone marrow-derived mouse macrophages under inflammatory conditions. To determine whether 25-OHC acts as chemoattractant for tumor-associated macrophages, the human THP-1 monoblastic leukemia cell line was treated with varying amounts of the oxysterol. Experiments revealed that 25-OHC and lipid extracts isolated from GM133-conditioned medium (containing 7-fold higher 25-OHC concentrations than U87MG medium) induce chemotactic migration of THP-1 cells. Of note, 25-OHC also induced the migration of primary human peripheral blood monocytes. In response to exogenously added 25-OHC, THP-1 cells reorganized intermediate filament-associated vimentin to more cortical and polarized structures. Chemotactic migration of monocytes in response to 25-OHC was pertussis toxin-sensitive, indicating the involvement of G protein-coupled receptors. Using RNA interference we demonstrated that G protein-coupled receptor 183 (EBI2) contributes to 25-OHC-mediated chemotactic migration of THP-1 cells. These in vitro data indicate that GBM-derived and secreted 25-OHC may be involved in the recruitment of immune-competent cells to a tumor via EBI2.
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Neoplasias Encefálicas/metabolismo , Quimiotaxis/efectos de los fármacos , Glioblastoma/metabolismo , Hidroxicolesteroles/metabolismo , Monocitos/fisiología , Línea Celular Tumoral , Humanos , Biosíntesis de Proteínas , ARN Interferente Pequeño , Receptores Acoplados a Proteínas G/genética , Receptores Acoplados a Proteínas G/metabolismo , Esteroide Hidroxilasas/genética , Esteroide Hidroxilasas/metabolismo , Esteroles/farmacología , Transcripción Genética , Regulación hacia Arriba , Vimentina/metabolismoRESUMEN
The perception of fear aura in complex partial seizures is linked to epileptic discharges within mesial temporal lobe structures. Although selective amygdalohippocampectomy often leads to favorable seizure control, persistence of fear auras after surgery can hamper quality of life significantly. We describe two patients with persistent fear auras after selective amygdalohippocampectomy who had to be reevaluated for a second operative procedure. In one patient, ictal SPECT revealed focal hyperperfusion within the left temporal pole. In the other patient, localization of the focus was possible with ictal scalp EEG, which revealed closely time-related focal theta activity in the right frontotemporal electrodes. Both patients underwent a second surgery leading to complete remission. The persistence of fear auras after selective amygdalohippocampectomy provides an example of involvement of a complex neuronal network in the generation of this emotional state during mesiotemporal lobe seizures. Ictal SPECT or ictal scalp EEG may be valuable in identifying the involved areas and in guiding the surgeon to render these patients seizure free.
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Amígdala del Cerebelo/diagnóstico por imagen , Epilepsia , Miedo , Hipocampo/diagnóstico por imagen , Adulto , Amígdala del Cerebelo/patología , Amígdala del Cerebelo/cirugía , Electroencefalografía , Epilepsia/diagnóstico por imagen , Epilepsia/fisiopatología , Epilepsia/cirugía , Femenino , Hipocampo/patología , Hipocampo/cirugía , Humanos , Imagen por Resonancia Magnética , Masculino , Procedimientos Neuroquirúrgicos/métodos , Radiografía , Cuero Cabelludo , Tomografía Computarizada de Emisión de Fotón ÚnicoRESUMEN
We report the case of a 14-year-old girl who presented with an 8-week history of unilateral pansinusitis. In the course of the disease a right-side frontal swelling was observed, which disappeared following antibiotic treatment. Four weeks later, however, the swelling reappeared and was now diagnosed as recurrent Pott's puffy tumor. Interestingly, C-reactive protein levels were in the normal range throughout. Following functional endoscopic frontal sinusotomy, antibiotic treatment with ceftriaxone was administered over 3 weeks and led to complete remission of the lesion. Pott's puffy tumor is a subperiostal abscess of the frontal bone, usually presenting as localized swelling of the soft tissue in the overlying region of the forehead, and is associated with localized osteomyelitis and occasionally with intracranial epidural abscess. The entity has been known since 1768, although recurrent cases with normal inflammation parameters have not been published previously.
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Absceso/diagnóstico , Absceso/terapia , Ceftriaxona/administración & dosificación , Hueso Frontal , Osteomielitis/diagnóstico , Osteomielitis/terapia , Adolescente , Antibacterianos/administración & dosificación , Terapia Combinada , Endoscopía , Femenino , Humanos , Enfermedades Raras/diagnóstico , Prevención Secundaria , Resultado del TratamientoRESUMEN
A 4(3/4)-year-old male with Down syndrome (DS) presented with unsteady gait and fatigue. Neuroimaging revealed a cerebellar mass with concomitant obstructive hydrocephalus and additional metastatic lesions. He was successfully treated and is still in complete remission 5 years from diagnosis. The present case illustrates that, although not yet reported, medulloblastoma can also occur in patients with DS.
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Síndrome de Down/complicaciones , Meduloblastoma/terapia , Protocolos de Quimioterapia Combinada Antineoplásica , Preescolar , Terapia Combinada , Irradiación Craneana , Humanos , Imagen por Resonancia Magnética , Masculino , Meduloblastoma/diagnóstico , Inducción de RemisiónRESUMEN
BACKGROUND AND PURPOSE: Radiotherapy is an integral part of various therapeutic regimens in pediatric and adult oncology. Endocrine dysfunction, neurologic and psychiatric deficits, secondary malignancies and radiation-induced necrosis are well-known possible late effects of cranial irradiation. However, only sporadic cases of radiation-induced cavernous hemangiomas (RICH) have been reported so far. PATIENTS AND METHODS: Pediatric patients who underwent cranial radiation therapy for malignant diseases between January 1980 and December 2003 were retrospectively analyzed. After the end of therapy they entered a detailed follow-up program. RESULTS: Of 171 patients, eight (three patients with medulloblastoma, three patients with acute lymphoblastic leukemia, and one patient each with ependymoma and craniopharyngioma) developed intracerebral cavernoma 2.9-18.4 years after irradiation representing a cumulative incidence (according to the Kaplan-Meier method) of 2.24%, 3.86%, 4.95%, and 6.74% within 5, 10, 15, and 20 years following radiation therapy, respectively. In patients treated in the first 10 years of life, RICH occurred with shorter latency and significantly more often (p = 0.044) resulting in an even higher cumulative incidence. CONCLUSION: These findings and previously published cases show that cavernous hemangiomas may occur after irradiation of the brain several years after the end of therapy irrespective of the radiation dose and type of malignancy. Particularly children < 10 years of age at the time of irradiation are at higher risk. Since patients with RICH frequently do not show symptoms but hemorrhage is a possible severe complication, imaging of the central nervous system should be performed routinely for longer follow- ups, particularly in patients who were treated as young children.
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Neoplasias Encefálicas/etiología , Irradiación Craneana/efectos adversos , Hemangioma Cavernoso del Sistema Nervioso Central/etiología , Neoplasias Inducidas por Radiación/etiología , Adolescente , Adulto , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/radioterapia , Neoplasias Cerebelosas/radioterapia , Niño , Preescolar , Craneofaringioma/radioterapia , Ependimoma/radioterapia , Femenino , Estudios de Seguimiento , Lóbulo Frontal/patología , Lóbulo Frontal/efectos de la radiación , Hemangioma Cavernoso del Sistema Nervioso Central/diagnóstico , Humanos , Lactante , Imagen por Resonancia Magnética , Masculino , Meduloblastoma/radioterapia , Neoplasias Inducidas por Radiación/diagnóstico , Lóbulo Parietal/patología , Lóbulo Parietal/efectos de la radiación , Neoplasias Hipofisarias/radioterapia , Leucemia-Linfoma Linfoblástico de Células Precursoras/radioterapia , Dosificación Radioterapéutica , Factores de Riesgo , Lóbulo Temporal/patología , Lóbulo Temporal/efectos de la radiación , Tomografía Computarizada por Rayos XRESUMEN
OBJECTIVE: To present clinical and morphological findings before and after surgery in a child with Chairi I malformation (CMI) and intra-cranial hypertension (IH). The literature is reviewed and pathophysiologic factors are discussed. CLINICAL PRESENTATION: A 13-year-old obese boy with a 3-week history of headaches, neck pain, torticollis and progressive visual deterioration was admitted. Bi-lateral chronic papilloedema and decrease in visual acuity were found in the presence of a previously diagnosed CMI. INTERVENTION AND FOLLOW-UP: Intra-cranial pressure monitoring demonstrating increased pressure levels was followed by a sub-occipital decompression, C1 laminectomy and duroplasty. Post-operatively, the boy improved markedly, the 6 months follow-up opthalmological examination demonstrated resolution of papilloedema, but consecutive bi-lateral optic nerve atrophy. CONCLUSION: IH with progressive visual deterioration represents one of the varying clinical presentations of CMI and may be classified as a secondary form of idiopathic IH. Neuro-ophthalmological examination in all patients with CMI is recommended to identify the real incidence of this presentation. Altered CSF dynamics, venous hypertension and obesity as co-factors may be causative pathophysiologic factors.
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Malformación de Arnold-Chiari/complicaciones , Hipertensión Intracraneal/etiología , Malformación de Arnold-Chiari/fisiopatología , Malformación de Arnold-Chiari/cirugía , Atrofia , Niño , Humanos , Hipertensión/complicaciones , Hipertensión Intracraneal/fisiopatología , Hipertensión Intracraneal/cirugía , Presión Intracraneal/fisiología , Imagen por Resonancia Magnética , Masculino , Procedimientos Neuroquirúrgicos , Obesidad/complicaciones , Enfermedades del Nervio Óptico/complicaciones , Enfermedades del Nervio Óptico/patología , Papiledema/complicaciones , Papiledema/patología , Trastornos de la Visión/etiología , Agudeza VisualRESUMEN
PURPOSE: In the presurgical evaluation of patients with partial epilepsy, the ictal single photon emission computed tomography (SPECT) is a useful noninvasive diagnostic tool for seizure focus localization. To achieve optimal SPECT scan quality, ictal tracer injection should be carried out as quickly as possible after the seizure onset and under highest safety conditions possible. Compared to the commonly used manual injection, an automatic administration of the radioactive tracer may provide higher quality standards for this procedure. In this study, therefore, we retrospectively analyzed efficiency and safety of an automatic injection system for ictal SPECT tracer application. METHODS: Over a 31-month period, 26 patients underwent ictal SPECT by use of an automatic remote-controlled injection pump originally designed for CT-contrast agent application. Various factors were reviewed, including latency of ictal injection, radiation safety parameters, and ictal seizure onset localizing value. RESULTS: Times between seizure onset and tracer injection ranged between 3 and 48 s. In 21 of 26 patients ictal SPECT supported the localization of the epileptogenic focus in the course of the presurgical evaluation. In all cases ictal SPECT tracer injection was performed with a high degree of safety to patients and staff. CONCLUSIONS: Ictal SPECT by use of a remote-controlled CT-contrast agent injection system provides a high scan quality and is a safe and confirmatory presurgical evaluation technique in the epilepsy-monitoring unit.
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Mapeo Encefálico , Corteza Cerebral/diagnóstico por imagen , Medios de Contraste/administración & dosificación , Epilepsias Parciales/diagnóstico por imagen , Radiofármacos/administración & dosificación , Robótica/instrumentación , Exametazima de Tecnecio Tc 99m/administración & dosificación , Tomografía Computarizada de Emisión de Fotón Único/métodos , Corteza Cerebral/fisiopatología , Corteza Cerebral/cirugía , Electroencefalografía/estadística & datos numéricos , Epilepsias Parciales/fisiopatología , Epilepsias Parciales/cirugía , Lateralidad Funcional/fisiología , Humanos , Bombas de Infusión , Inyecciones/instrumentación , Imagen por Resonancia Magnética , Cuidados Preoperatorios , Protección Radiológica , Estudios Retrospectivos , Tomografía Computarizada de Emisión de Fotón Único/instrumentaciónRESUMEN
PURPOSE: Anterior callosotomy is a surgical option for the treatment of generalized tonic or atonic seizures associated with drop attacks. Besides open surgery, a radiosurgical callosal disconnection using the gamma knife (GK) also can be performed, but reliable data about tolerability and efficacy are sparse. METHODS: Eight patients (three female, five male age range, 5 to 69 years) with severe generalized epilepsy associated with disabling drop attacks underwent GK callosotomy between 1993 and 2004. In six patients, the anterior third of the corpus callosum was radiosurgically disconnected. In one patient a second procedure with GK treatment of the middle third of the corpus callosum was added 17 months later. In two patients posterior GK callosotomy had followed partial hemispherotomy. RESULTS: Drop attacks (DAs) were completely abolished in three patients, and two patients had a marked DA seizure reduction of 60%. Two of four patients with additional generalized tonic-clonic seizures showed a reduction of 100%, and the remaining, a 50% and 60% decrease, respectively. Other seizure types responded less well to the radiosurgical treatment. In both patients with posterior GK callosotomy after hemispherotomy, partial seizures decreased. Beside transient headache in two patients, no immediate or long-term postradiosurgical side effects were observed. CONCLUSIONS: Palliative radiosurgical callosotomy is an efficient and safe noninvasive alternative to the open procedure with comparable results. No signs of postradiosurgical side effects were noted within an up to 12-year posttreatment period.
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Cuerpo Calloso/cirugía , Epilepsia Generalizada/cirugía , Radiocirugia/efectos adversos , Radiocirugia/métodos , Adolescente , Adulto , Anciano , Niño , Preescolar , Cuerpo Calloso/patología , Epilepsia Generalizada/diagnóstico , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Necrosis/patología , Dosis de Radiación , Traumatismos por Radiación/patología , Índice de Severidad de la Enfermedad , Síncope/prevención & control , Resultado del TratamientoRESUMEN
The aim of the present study was to evaluate the spectrum of late effects in a large cohort of pediatric patients with low-grade gliomas (WHO grade I and II) during an observation period of 20 years. Eighty-seven patients with low-grade gliomas grouped according to tumor location (cerebellum: n=28; cerebral hemispheres: n=21; central midline: n=15; brainstem: n=12; tectum: n=5; other locations: n=6) were evaluated for tumor- and/or treatment-related late effects by analysis of medical and computer records, and personal interviews. Seventy patients underwent neurosurgery, 29 patients received additional radiotherapy and 20 additional chemotherapy. Median follow-up of survivors is 96 months with an overall survival of 79% (cerebellum: 89%; cerebral hemispheres: 95%; central midline: 80%; brainstem: 25%; tectum: 100%; other locations: 66%). Chronic medical problems (mild ataxia to multiple severe neuroendocrine deficits) are observed in 100% of patients with brainstem/central midline tumors and in 40-50% of patients with low-grade gliomas of other locations. Endocrine deficiencies were observed in 15/17 (88%) of long-term survivors who received radiotherapy. In contrast, none of the patients who underwent surgery only had endocrine deficiencies. Seven long-term survivors (10.1%) are severely disabled with permanent need of medical help. Tumor- and treatment-related late effects are common in patients with low-grade gliomas with the most severe occurring in patients with brainstem or central midline tumors. As long-term survival is excellent in patients with low-grade gliomas except for tumors located in the brainstem, future treatment studies should focus on avoiding long-term late effects.
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Neoplasias Encefálicas/terapia , Enfermedades del Sistema Endocrino/epidemiología , Glioma/terapia , Enfermedades del Sistema Nervioso/epidemiología , Adolescente , Adulto , Antineoplásicos/efectos adversos , Austria/epidemiología , Neoplasias Encefálicas/complicaciones , Niño , Preescolar , Estudios de Cohortes , Terapia Combinada/efectos adversos , Supervivencia sin Enfermedad , Enfermedades del Sistema Endocrino/etiología , Femenino , Estudios de Seguimiento , Glioma/complicaciones , Trastornos de la Audición/epidemiología , Trastornos de la Audición/etiología , Humanos , Lactante , Masculino , Enfermedades del Sistema Nervioso/etiología , Traumatismos por Radiación/epidemiología , Estudios Retrospectivos , Sobrevivientes/estadística & datos numéricos , Trastornos de la Visión/epidemiología , Trastornos de la Visión/etiologíaRESUMEN
OBJECTIVES: Gamma knife radiosurgery as a noninvasive procedure is increasingly used as a treatment option in patients with intractable seizures. We investigated efficacy and safety of gamma knife radiosurgery (GKRS) for callosotomy in children. MATERIALS AND METHODS: Between 2000 and 2004 three children between 4 and 14 years (mean 8 years) underwent radiosurgical callosotomy. One child presented with Lennox-Gastaut syndrome, two with hemispheric cortical dysplasia. These two children underwent functional hemispherotomy before GKRS. GKRS was performed with a marginal dose of 55-60 Gy on the 50% isodose. RESULTS: Mean follow-up was 35 months. Radiosurgical callosotomy was ineffective in one child with the Lennox-Gastaut syndrome, whereas in the remaining two children, a 100% seizure reduction of generalized tonic-clonic seizures, 20-70% reduction of partial seizures, and a progress in mental and physical development was achieved. No postradiosurgical side effects were observed in all children. CONCLUSION: Radiosurgical callosotomy might be offered after hemispherotomy to complete callosal resection. However, larger number of patients and longer follow-ups are needed to draw final conclusions.
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Cuerpo Calloso/cirugía , Epilepsia/patología , Epilepsia/cirugía , Radiocirugia/métodos , Adolescente , Niño , Preescolar , Resistencia a Medicamentos , Epilepsia/tratamiento farmacológico , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Antibodies against the glutamate receptor type 3-(GluR3) have been found in association with Rasmussen's encephalitis (RE) but were also detected in patients with non-inflammatory focal epilepsies. We report the case of an 18-year-old patient with treatment refractory left mesial temporal lobe epilepsy accompanied by high levels of GluR3 antibodies. Different from experiences in patients with RE immunomodulatory therapy by use of intravenous gammaglobulines neither altered GluR3 serum levels nor had any effect on seizure frequency in our patient. Interestingly, GluR3 serum levels remained positive after successful surgical intervention leading to patient's seizure freedom.
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Autoanticuerpos/sangre , Epilepsia del Lóbulo Temporal/tratamiento farmacológico , Inmunoglobulina G/uso terapéutico , Inmunoglobulinas Intravenosas/uso terapéutico , Factores Inmunológicos/uso terapéutico , Receptores AMPA/inmunología , Adolescente , Lobectomía Temporal Anterior , Autoanticuerpos/efectos de los fármacos , Epilepsia del Lóbulo Temporal/inmunología , Epilepsia del Lóbulo Temporal/cirugía , Humanos , Inmunoglobulina G/sangre , MasculinoRESUMEN
PURPOSE: The aim of this study was to report on children with cerebellar low-grade glioma (LGG), who were found to have progressive or nonprogresssive residual tumors or tumor recurrence after tumor resection. PATIENTS AND METHODS: Medical records and magnetic resonance imaging (MRI) studies of children (<16 years) with cerebellar LGG were retrospectively analyzed. RESULTS: Of 289 patients with CNS tumors referred between 1983 and 2003, 28 (9.7%) (15 male, 13 female; median age at diagnosis: 71 months) had cerebellar LGG (pilocytic astrocytoma grade I: n = 21; fibrillary astrocytoma grade II: n = 5; mixed hamartoma/pilocytic astrocytoma: n = 1; radiographic diagnosis: n = 1). Total resection was initially performed in 16 patients (57.1%), near total resection in 4 (14.3%), and partial resection in 6 patients (21.4%). One patient underwent biopsy. At a median follow-up of 112 months, 25 patients (89.3%) were alive, 18 of them being in complete remission. Three patients died, 2 due to symptoms related to brain stem compression/infiltration and 1 patient due to postoperative cerebral edema. Presently 5 patients have nonprogressive residual tumors and 2 patients developed nonprogressive recurrences 10 years and 20 months after initial total resection, respectively. None of them required second surgery and none received additional nonsurgical therapies. Only 1 additional patient had to undergo second surgery due to disease progression. CONCLUSIONS: A 'wait and see' strategy is justified in patients with nonprogressive recurrent or residual cerebellar LGG after primary tumor resection. However, long-term follow-up with repeated MRI is mandatory in these patients to detect disease progression. Second surgery is indicated only in patients with unequivocal disease progression, as documented by MRI.
Asunto(s)
Astrocitoma/cirugía , Neoplasias Cerebelosas/cirugía , Recurrencia Local de Neoplasia/cirugía , Neoplasia Residual/cirugía , Adolescente , Astrocitoma/diagnóstico , Astrocitoma/mortalidad , Neoplasias Cerebelosas/diagnóstico , Neoplasias Cerebelosas/mortalidad , Niño , Preescolar , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Lactante , Imagen por Resonancia Magnética , Masculino , Recurrencia Local de Neoplasia/diagnóstico , Neoplasia Residual/diagnóstico , Neoplasia Residual/mortalidad , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/mortalidad , Complicaciones Posoperatorias/cirugía , Reoperación , Estudios Retrospectivos , Tasa de SupervivenciaRESUMEN
INTRODUCTION: The treatment of brain abscess remains a challenging topic usually involving a multimodal concept. METHODS: We report our experience with hyperbaric oxygen (HBO) therapy in five children presenting with brain abscesses between 1995 and 2002 at the Department of Neurosurgery, Graz. Mean age was 14.8 (range 11-17 years). All abscesses were located supratentorially. One child had a single abscess and one had multilocated abscesses. Two other patients presented with both subdural empyema and brain abscess, one of them showing an epidural empyema as well. In another child, the brain abscess was associated with meningoencephalitis and subdural empyema. In all of them the underlying condition was spread of infection from the paranasal sinuses, except for one, who was immunocompromised due to cytotoxic chemotherapy for acute lymphocytic leukaemia. RESULTS: One single brain abscess and one of the multiple abscesses were drained. All subdural/epidural empyemas were treated surgically. Antibiotics were administered intravenously for 13 to 22 days (mean 22 days). All patients underwent HBO therapy; the number of treatments ranged from 26 to 45 "dives" (mean 30). Treatments were given once daily at 2.2 atmosphere absolutes for 60 min at 12 m. During the hospital stay all improved their clinical condition, with continued regression of abnormalities on magnetic resonance imaging (MRI). In the following weeks, other interventions were performed to treat the origin of the infections. At 6 months follow-up they were all in good clinical condition, either symptom free or with minor residual symptoms. MRI at this time showed no evidence of disease in three, a residual dural enhancement in one and a residual shrunken collection in the child with multilocated abscesses. No recurrence was observed during a mean follow-up of 21 months (range from 7 to 72 months). CONCLUSION: HBO therapy in children with brain abscesses seems to be safe and effective, even when they are associated with subdural or epidural empyemas. It provides a helpful adjuvant tool in the usual multimodal treatment of cerebral infections and may reduce the intravenous course of antibiotics and, consequently, the duration of hospitalization. Multidisciplinary management is recommended to optimize care for these critically ill children.
Asunto(s)
Absceso Encefálico/terapia , Oxigenoterapia Hiperbárica/métodos , Adolescente , Absceso Encefálico/diagnóstico , Niño , Femenino , Estudios de Seguimiento , Humanos , Imagen por Resonancia Magnética/métodos , Masculino , Procedimientos Neuroquirúrgicos/métodos , Estudios RetrospectivosRESUMEN
OBJECTIVES: Ventriculoperitoneal shunting is the most common treatment for hydrocephalus. Repeated shunt revisions and other previous surgical procedures can complicate the placement of the distal catheter. Occasionally, when conventional sites like the abdominal cavity and the right atrium are used up or unavailable, a ventriculopleural shunt can be inserted. MATERIALS AND METHODS: Between April 2001 and August 2002, 7 children ranging in age from 5 months to 17 years underwent ten endoscopically-assisted cerebrospinal fluid shunt insertions. All patients had had previous multiple shunt revisions and other abdominal operations. In addition 4 children had suffered from severe peritonitis, 1 of them with a subsequent adhesion ileus. In 9 cases the terminal portion of the shunt was inserted with laparoscopic assistance and in 1 case with thoracoscopic assistance. The latter had previously undergone two conventional intercostal thoracotomies for shunt insertions. Placement of the shunt was performed in a suitable area under direct laparoscopic or thoracoscopic vision by a peel-off needle into the peritoneal or pleural cavity. In 2 children repeated endoscopic approaches were necessary for shunt replacement due to infections. RESULTS: We used this procedure successfully in each case without operation-related complications. Neither new adhesions nor difficulties in laparoscopic replacement of the shunt were observed. CONCLUSION: Laparoscopic and thoracoscopic assistance in the placement of the distal catheter has the benefit of allowing a safe and precise insertion with visual control of shunt function, even in patients with prior surgical interventions. Additionally, this minimally invasive technique appears to prevent new adhesions arising in the peritoneal cavity.