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Purpose: We assess the application and advantages of modifying the trans-unco-discal (TUD) approach which we underwent for cervical myelo-radiculopathy. We present the surgical techniques of the modified TUD approach. Materials and Methods: The material was 180 cases where anterior cervical decompression (ACD) was performed by the modified TUD approach. We classified the material into four groups based on the location of the nerve root and/or spinal cord compression: I, compression of the root at intervertebral foramen (IVF); II, compression of the posterior margin of the vertebral body; III, compression of the IVF and posterior margin of the vertebral body; IV, compression of the bilateral IVF and posterior margin of the vertebral body. We applied the modified TUD approach to these four types. We present the surgical procedures and techniques for the modified TUD approach. The Japanese orthopedic association (JOA) score and neuroradiological alignment were examined. Results: The improvement rate of the JOA score was 78.4% at 6 months post-surgery and 77.5% in the most recent examinations. By the modified TUD approach, compressive lesions of the spinal cord and/or nerve roots were removed, and good alignment was acquired and sustained. Conclusions: ACD by the modified TUD approach safely achieved appropriate decompression for the spinal cord and/or nerve roots, and the patients had a high improvement rate and good alignment. Complications were less common than with other surgical procedures. If the TUD approach and endoscopic approaches can be combined, their application to new area is anticipated.
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INTRODUCTION: We report our experience regarding the clinical features and pathological findings of the calcification of the ligamentum flavum (CLF) and ossification of the ligamentum flavum (OLF) in the spine. In addition, we reviewed the previous studies on CLF and OLF to enhance the understanding of these conditions. MATERIALS AND METHODS: We compared the clinical, radiological, and histopathological features of CLF and OLF. RESULTS: In CLF, a computed tomography (CT) scan showed egg-shaped or speck-like calcification in the ligamentum flavum. Magnetic resonance (MR) imaging demonstrated spinal cord compression due to a thickened ligamentum flavum, which appeared as a low-intensity mass. Pathological findings demonstrated fused islands of calcification resembling sand-like calcification. In OLF, CT showed beak-like ossification extending into the intervertebral foramen. MR imaging demonstrated spinal cord compression by a low-intensity mass. Pathological findings revealed laminar ossification of LF with chondrocytes near the calcification and laminar hyaline cartilage. CONCLUSIONS: CLF and OLF appear to be distinct entities based on their clinical, neuroradiological, histopathological, and pathogenetic features. We suggest that the causes of CLF include both metabolic and dystrophic factors, while the pathogenesis of OLF is characterized by enchondral ossification induced by a genetic cascade triggered by shearing/tension stress.
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To image cerebral neural activity in ischemic areas, we proposed a novel technique to analyze spontaneous neuromagnetic fields based on standardized low-resolution brain electromagnetic tomography modified for a quantifiable method (sLORETA-qm). Using a 160-channel whole-head-type magnetoencephalographic system, cerebral magnetic fields were obtained pre- and postoperatively from 5 patients with unilateral internal carotid artery occlusive disease and 16 age-matched healthy volunteers. For quantitative imaging, voxel-based time-averaged intensities of slow waves in 4 frequency bands (0.3-2 Hz, 2-4 Hz, 4-6 Hz and 6-8 Hz) were obtained by the proposed technique based on sLORETA-qm. Positron emission tomography with (15)O gas inhalation ((15)O-PET) was also performed in these patients to evaluate cerebral blood flow and metabolism. In all 5 patients, slow waves in every frequency band were distributed in the area of cerebrovascular insufficiency, as confirmed by (15)O-PET preoperatively. In 4 patients, slow-wave intensities in theta bands (4-6 Hz, 6-8 Hz) decreased postoperatively along with improvements in cerebral blood flow and metabolism, whereas delta bands (0.3-2 Hz, 2-4 Hz) showed no significant differences between pre- and postoperatively. One patient with deterioration of cerebral infarction after surgery showed marked increases in slow-wave intensities in delta bands (0.3-2 Hz, 2-4 Hz) postoperatively, with distribution close to the infarct region. The proposed quantitative imaging of spontaneous neuromagnetic fields enabled clear visualization and alternations of cerebral neural conditions in the ischemic area. This technique may offer a novel, non-invasive method for identifying cerebral ischemia, although further studies in a larger number of patients are warranted.
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Isquemia Encefálica/patología , Procesamiento de Imagen Asistido por Computador/métodos , Imagen por Resonancia Magnética/métodos , Anciano , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Isquemia Encefálica/diagnóstico por imagen , Corteza Cerebral/patología , Corteza Cerebral/fisiopatología , Infarto Cerebral/diagnóstico por imagen , Infarto Cerebral/patología , Disartria/patología , Electroencefalografía , Humanos , Magnetoencefalografía , Masculino , Persona de Mediana Edad , Paresia/patología , Tomografía de Emisión de PositronesRESUMEN
OBJECTIVE: The objective of this retrospective study is to analyze whether preoperative functional imaging studies using FDG-PET and MEG enable prediction of postoperative seizure outcomes. METHODS: Thirty-six patients with intractable temporal lobe epilepsy were studied. Asymmetry index of tCMRgluc (PET-AI) and the equivalent current dipole intensity of first response of SEF (SEF-AI) were determined preoperatively using (18)F-fluorodeoxyglucose-positron emission tomography (FDG-PET) and magnetoencephalography (MEG), respectively. Seizure outcomes were evaluated according to the classification proposed by the International League Against Epilepsy (ILAE) at least 24 months after resection of epileptic focuses. Twelve healthy volunteers were included in this study to determine the normal value. RESULTS: Quantitative analysis revealed mean PET-AI in the patients was 5.4+/-5.2% (significantly different from normal controls); mean SEF-AI was 25.2+/-20.6% (not significantly different). PET-AI was positive (indicative of epileptic focus) in 29 of 36 patients (80.6%), while SEF-AI was positive in 17 of 36 patients (47.2%). Although no significant correlation between PET-AI and SEF-AI was noted (r=0.43), concordant asymmetry in both PET-AI and SEF-AI was significantly associated with better seizure outcome than discordant or paradoxical asymmetry of both factors (p<0.01). CONCLUSIONS: The results suggest that quantitative analysis of tCMRgluc with SEF may be helpful in characterizing the preoperative epileptogenic condition and predicting postoperative seizure outcome in patients with temporal lobe epilepsy, although a constellation of developmental brain abnormalities and environmental factors that together produce epilepsy need to be further explored.
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Epilepsia del Lóbulo Temporal/patología , Epilepsia del Lóbulo Temporal/fisiopatología , Epilepsia del Lóbulo Temporal/cirugía , Glucosa/metabolismo , Tálamo/metabolismo , Adulto , Niño , Epilepsia del Lóbulo Temporal/diagnóstico por imagen , Femenino , Fluorodesoxiglucosa F18 , Humanos , Imagen por Resonancia Magnética , Magnetoencefalografía/métodos , Masculino , Persona de Mediana Edad , Tomografía de Emisión de Positrones/métodos , Periodo Posoperatorio , Valor Predictivo de las Pruebas , Estadísticas no Paramétricas , Tálamo/diagnóstico por imagen , Resultado del Tratamiento , Adulto JovenRESUMEN
RCAS1, one of the tumor cell surface antigens, is strongly expressed in aggressive tumors. RCAS1 suppresses the in vitro growth of immune effector cells. We investigated the expression of RCAS1 in 57 gliomas using immunohistochemistry. Furthermore, we examined the association of the RCAS1 expression with the infiltration of tumor infiltrating lymphocyte (TIL). RCAS1 overexpression was significantly correlated with high histological grade and poor prognosis. Reduced infiltration and increased apoptosis of TILs was observed in RCAS1-positive regions. Apoptotsis of TILs appeared to be induced by RCAS1. RCAS1 expression in gliomas may play roles in tumor progression and tumor immune escape.
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Antígenos de Neoplasias/biosíntesis , Glioma/patología , Linfocitos Infiltrantes de Tumor/patología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Análisis de Varianza , Apoptosis , Niño , Femenino , Glioma/inmunología , Glioma/metabolismo , Humanos , Inmunohistoquímica/métodos , Inmunohistoquímica/estadística & datos numéricos , Estimación de Kaplan-Meier , Antígeno Ki-67/análisis , Antígenos Comunes de Leucocito/análisis , Masculino , Persona de Mediana Edad , Pronóstico , Modelos de Riesgos Proporcionales , Linfocitos T/metabolismo , Linfocitos T/patologíaRESUMEN
Surgical procedures for cerebral hemispherotomy may be broadly divided into those using a lateral and those using a vertical approach. However, careful study of surgical procedures using the lateral approach described in the literature shows differences in the approach to the ventricles. We discuss the application of transventricular hemispherotomy as a technique which provides relatively easy ventricular access for cerebral hemispherotomy. Transventricular hemispherotomy was successfully performed in a 36-year-old woman who was diagnosed with intractable epilepsy due to Sturge-Weber disease, and in a 25-year-old man who had developed intractable post-traumatic seizures after suffering cerebral contusion in a traffic accident as a child. These patients had no seizures or complications after surgery, and both patients have been weaned from antiepileptic drugs. The transventricular approach, as compared with other lateral approaches described in the literature, provides easy access to the ventricular cavity. Transventricular hemispherotomy proved to be a useful approach that allowed the following four common steps in cerebral hemispherotomy to be performed safely: (i) interruption of the internal capsule and corona radiata; (ii) resection of the medial temporal structures; (iii) transventricular corpus callosotomy; and (iv) disruption of the frontal horizontal fibers.
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Lesiones Encefálicas/complicaciones , Ventrículos Cerebrales/cirugía , Epilepsia/cirugía , Hemisferectomía/métodos , Síndrome de Sturge-Weber/complicaciones , Adulto , Lesiones Encefálicas/cirugía , Craneotomía/métodos , Epilepsia/etiología , Femenino , Humanos , Masculino , Resultado del TratamientoRESUMEN
In the management of skull base chordomas, surgical treatment is essential to achieve long-term control. A petroclival chordoma growing laterally in the skull base is one of the most challenging tumors for neurosurgeons. We have treated petroclival chordomas based on the principle of maximal surgical resection of the tumor with minimal morbidity. Lateral skull base approaches were used to approach petroclival chordomas in eight patients. The surgical procedure involved removal of soft tumor tissue and extensive drilling of adjacent bony structures. Gross total resection of the tumor was achieved in six patients. Subtotal resection in the remaining two patients was associated with acceptable morbidity. In cases of petroclival chordomas, lateral skull base approaches can be used as a primary procedure, although those approaches may be associated with high rates of morbidity and mortality.
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We present a rare case of skull base chordoma of extraosseous intradural type that presented as acute intratumoral hemorrhage. Surgical removal of the tumor was accomplished using a skull base approach.
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Cordoma/complicaciones , Neoplasias de la Coroides/complicaciones , Hemorragia Subaracnoidea/etiología , Adulto , Cordoma/cirugía , Neoplasias de la Coroides/cirugía , Femenino , Humanos , Literatura de Revisión como Asunto , Hemorragia Subaracnoidea/cirugíaRESUMEN
OBJECTIVE: We report three cases with ventral cervical intramedullary cavernous angiomas. An anterolateral partial vertebrectomy was performed to surgically approach and successfully resect these lesions. MATERIALS AND METHODS: All three patients presented with numbness in the upper extremities and investigation revealed a cavernous angioma on the ventral aspect of the spinal cord in each case. The lesion was exposed by an anterolateral partial vertebrectomy in all cases. Postoperative stabilization was achieved by using autografted iliac bone in all patients. In two patients, locking screws and plates were also used. RESULTS: Complete resection of the cavernous angioma was performed in all patients. There was symptomatic relief in all cases, and there was no postoperative morbidity. CONCLUSION: Anterolateral partial vertebrectomy provides direct exposure and is probably an ideal approach for selected cases with ventrally located intramedullary cavernous angiomas.
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Vértebras Cervicales/cirugía , Hemangioma Cavernoso del Sistema Nervioso Central/cirugía , Procedimientos Neuroquirúrgicos/métodos , Neoplasias de la Médula Espinal/cirugía , Procedimientos Quirúrgicos Vasculares/métodos , Adulto , Placas Óseas , Trasplante Óseo/métodos , Vértebras Cervicales/anatomía & histología , Duramadre/anatomía & histología , Duramadre/cirugía , Femenino , Hemangioma Cavernoso del Sistema Nervioso Central/fisiopatología , Humanos , Disco Intervertebral/anatomía & histología , Disco Intervertebral/cirugía , Procedimientos Neuroquirúrgicos/instrumentación , Complicaciones Posoperatorias/prevención & control , Neoplasias de la Médula Espinal/fisiopatología , Fusión Vertebral/instrumentación , Fusión Vertebral/métodos , Resultado del Tratamiento , Procedimientos Quirúrgicos Vasculares/instrumentaciónRESUMEN
The histological features of arterialized medullary vein (MV) in spinal dural arteriovenous fistulas (SDAVF) were studied in five consecutive patients who presented with progressive congestive myelopathy. Retrograde venous filling on preoperative angiography was recognized as being severe in 3 cases and moderate in 2 cases. Direct intradural interruption of the arterialized MV was performed in all patients. The arterialized MV was sampled and examined histologically to determine the percentage of the hyperplasia of venous wall (hypertrophic ratio). Histological examination of arterialized MV showed that hypertrophic alteration of venous wall structure was due to hyperplasia of elastic fibers, ranging from 41 to 82%. Patients with angiographically severe venous hypertension tended to have a higher hypertrophic ratio than patients with moderate venous hypertension. Our observations support the clinical concept that long-standing arterial stress in the spinal venous circulation causes histological alterations of spinal vascular structure associated with the progression of venous hypertension. We suggested that possibly the histological parameter can be used for predicting neurological recovery after occlusion of the fistulas.
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Fístula Arteriovenosa/patología , Bulbo Raquídeo/patología , Angiografía , Arterias/patología , Circulación Cerebrovascular/fisiología , Trastornos Neurológicos de la Marcha/etiología , Trastornos Neurológicos de la Marcha/patología , Humanos , Imagen por Resonancia Magnética , Masculino , Bulbo Raquídeo/irrigación sanguínea , Persona de Mediana Edad , Médula Espinal/patología , Venas/patologíaRESUMEN
Transsylvian selective amygdalohippocampectomy (TSA) is an operative technique designed to spare unaffected brain tissue during surgical treatment for mesial temporal lobe epilepsy. In contrast to standard anterior temporal lobectomy (ATL), the advantages of TSA with respect to postoperative cognitive outcome are equivocal. We compared cognitive function before and after surgery in 49 patients with unilateral mesial temporal lobe seizures who underwent either ATL (n=17) or TSA (n=32). All patients received neuropsychological testing before and 1 year after surgery. The intelligence quotient (IQ) increased postoperatively in both surgical groups. Memory evaluation in the ATL group revealed a postoperative decline in nonverbal memory after right-sided resection and a postoperative decline in verbal memory after left-sided resection. In the TSA group, there was a slight postoperative decline only in verbal memory after left-sided resection, but other memory function was well preserved. In particular, there was significant postoperative improvement in verbal memory after right-sided resection. Overall, memory function was better preserved in the TSA group than in the ATL group.
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Amígdala del Cerebelo/cirugía , Lobectomía Temporal Anterior , Epilepsia del Lóbulo Temporal/cirugía , Hipocampo/cirugía , Inteligencia , Memoria , Adulto , Amígdala del Cerebelo/patología , Epilepsia del Lóbulo Temporal/patología , Epilepsia del Lóbulo Temporal/psicología , Femenino , Estudios de Seguimiento , Hipocampo/patología , Humanos , Masculino , Análisis Multivariante , Resultado del TratamientoRESUMEN
Soluble amyloid beta protein (Abeta) oligomers have been considered recently to be responsible for the cognitive dysfunction that sets in prior to senile plaque formation in the Alzheimer's disease (AD) brain. By using the newly prepared antibody against oligomer Abeta, rather than fibrillar or monomer Abeta, we observed that oligomer Abeta in AD brains was localized as clusters ofdot-likeimmunostains in the neurons in a manner different from that in senile plaques. The relationship of oligomer Abeta with synaptophysin, a synaptic molecular marker, was examined because oligomer Abeta is widely believed to be related to synaptic failure. We observed that immunostainings for synaptophysin were absent near neurons bearing clusters of oligomer Abeta. The present study provides morphological evidence to support the idea that accumulated oligomer Abeta, but not fibrillar Abeta, is closely associated with synaptic failure, which is the major cause of cognitive dysfunction.
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Enfermedad de Alzheimer/metabolismo , Enfermedad de Alzheimer/fisiopatología , Corteza Cerebral/metabolismo , Corteza Cerebral/fisiopatología , Neuronas/metabolismo , Sinaptofisina/metabolismo , Péptidos beta-Amiloides/análisis , Péptidos beta-Amiloides/inmunología , Péptidos beta-Amiloides/metabolismo , Anticuerpos , Especificidad de Anticuerpos/inmunología , Biomarcadores/análisis , Biomarcadores/metabolismo , Corteza Cerebral/patología , Regulación hacia Abajo/fisiología , Humanos , Inmunohistoquímica/métodos , Degeneración Nerviosa/metabolismo , Degeneración Nerviosa/fisiopatología , Neuronas/inmunología , Neuronas/patología , Placa Amiloide/inmunología , Placa Amiloide/metabolismo , Placa Amiloide/patología , Sinapsis/metabolismo , Sinapsis/patología , Sinaptofisina/análisis , Sinaptofisina/inmunologíaRESUMEN
A 25-year-old man presented with intractable post-traumatic seizures after suffering cerebral contusion in a traffic accident at age 5 years. Cerebral hemispherotomy was performed to transect the neuronal fibers to interrupt connections between seizure foci in wide areas of the brain, and to minimize the resected brain parenchyma. His seizures resolved and behavioral disorders improved, which had been impaired since age 8 years. Increased glucose metabolism in the normal frontal lobe detected by interictal fluorodeoxyglucose-positron emission tomography was correlated with the improvements in behavioral disorders. These findings suggest that the effects of seizures may be reversible in brain areas connected with, but remote from, the epileptogenic cortex.
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Conmoción Encefálica/complicaciones , Epilepsia Postraumática/cirugía , Epilepsia Tónico-Clónica/cirugía , Lóbulo Frontal/lesiones , Hemisferectomía , Complicaciones Posoperatorias/cirugía , Trastorno de la Conducta Social/cirugía , Adulto , Glucemia/metabolismo , Conmoción Encefálica/fisiopatología , Niño , Preescolar , Craneotomía , Electroencefalografía , Epilepsia Postraumática/fisiopatología , Epilepsia Tónico-Clónica/fisiopatología , Fluorodesoxiglucosa F18 , Lóbulo Frontal/fisiopatología , Lóbulo Frontal/cirugía , Humanos , Imagen por Resonancia Magnética , Masculino , Examen Neurológico , Lóbulo Parietal/fisiopatología , Lóbulo Parietal/cirugía , Tomografía de Emisión de Positrones , Complicaciones Posoperatorias/fisiopatología , Trastorno de la Conducta Social/fisiopatología , Resultado del TratamientoRESUMEN
OBJECT: The authors evaluated their surgical experience over 20 years with 14 treated falcotentorial meningiomas. METHODS: In the past 20 years, 14 patients with falcotentorial junction meningiomas were surgically treated. There were seven men and seven women, whose ages ranged from 34 to 79 years. On the basis of neuroimaging studies, the authors analyzed the influence of the anatomical relationship of the tumor to the vein of Galen, patency of the vein of Galen, tumor size, and the signal intensities on the magnetic resonance images to determine possible difficulties that might be encountered during surgery and to prognosticate the outcome of surgery. Depending on the relationship with the vein of Galen, tumors were labeled as either a superior or an inferior type. All tumors were resected via an occipital transtentorial approach. The surgical outcome in eight patients was excellent; in the remaining six patients, it was fair. Of the prognostic factors, tumor location especially seemed to be the most important (p < 0.01, Fisher exact test). The outcome associated with the inferior type of tumor was significantly less optimal probably due to the relationship to the deep veins and the brainstem. In this series, the occlusion of deep veins did not significantly influence outcome. CONCLUSIONS: . Classification of the tumor location by preoperative neuroimaging studies can be helpful in estimating the surgical difficulty that might be encountered in treating the falcotentorial junction meningioma.
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Neoplasias Meníngeas/cirugía , Meningioma/cirugía , Adulto , Anciano , Venas Cerebrales/cirugía , Femenino , Humanos , Angiografía por Resonancia Magnética , Imagen por Resonancia Magnética , Masculino , Neoplasias Meníngeas/patología , Meningioma/patología , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
OBJECTIVES: Experimental spinal cord injury (SCI) with focal spinal tissue scarring was studied to better understand the progressive post-traumatic myelomalacia (PPM). METHODS: Using a stereotactic device, the authors developed an acute compression of spinal cord at Th-10 in the adult rat. In Group A, the rat thoracic spinal cord was compressed epidurally with preservation of local cerebrospinal fluid (CSF) dynamics. In Group B (spinal tissue scarring), the rat thoracic spinal cord was compressed directly after disruption of meninges of dura mater and arachnoid membrane, followed by gelatine sponge sealing. All rats were maintained for 12 weeks after injury. RESULTS: Imaging analysis revealed the significant increase of cystic cavitation in the contused spinal cord in Group B compared with Group A. Anterograde axonal tracing demonstrated that the labeled corticospinal axons had extended axonal sprouting into the nearby gray matter and displayed prominent dieback at the rostral interface of the spinal cord lesion in both groups. No significant difference in motor and sensory functions between the two groups was noted. DISCUSSION: The new experimental model of PPM formation was devised. Spinal tissue scarring at the injury site may cause a tethering effect on the spinal cord, which may lead to significant alteration of the spinal cord parenchyma. Although the devastating effect of PPM on motor and sensory functions is still not resolved completely, the results in the present study suggest the importance of careful analysis in the treatment of PPM after SCI.
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Cicatriz/fisiopatología , Enfermedades de la Médula Espinal/patología , Enfermedades de la Médula Espinal/fisiopatología , Médula Espinal/patología , Animales , Axones/metabolismo , Axones/patología , Biotina/análogos & derivados , Dextranos , Modelos Animales de Enfermedad , Espacio Epidural/patología , Femenino , Hiperalgesia/fisiopatología , Actividad Motora/fisiología , Desempeño Psicomotor/fisiología , Tractos Piramidales/patología , Ratas , Ratas Endogámicas F344 , Estadísticas no Paramétricas , Factores de TiempoAsunto(s)
Neoplasias Encefálicas/diagnóstico por imagen , Radioisótopos de Carbono , Metionina , Tomografía de Emisión de Positrones , Radiofármacos , Adolescente , Astrocitoma/diagnóstico por imagen , Niño , Preescolar , Femenino , Germinoma/diagnóstico por imagen , Glioma/diagnóstico por imagen , Humanos , Lactante , Masculino , Meduloblastoma/diagnóstico por imagen , Tomografía de Emisión de Positrones/métodos , Estudios RetrospectivosAsunto(s)
Neoplasias Encefálicas/cirugía , Neuronavegación/instrumentación , Neoplasias de la Base del Cráneo/cirugía , Base del Cráneo/cirugía , Adulto , Anciano , Neoplasias Encefálicas/patología , Femenino , Humanos , Imagen por Resonancia Magnética , Persona de Mediana Edad , Neuronavegación/métodos , Base del Cráneo/anatomía & histología , Neoplasias de la Base del Cráneo/patologíaRESUMEN
Cathepsin B, one of the lysosomal cysteine proteases, has been related to tumor invasiveness. Cystatin C is the strongest inhibitor of cathepsin B. Knowledge of its participation in the progression of gliomas is limited. We investigated the expression of cystatin C and its association with the clinicopathologic features of 57 gliomas. Cystatin C and cathepsin B expressions were evaluated by immunohistochemical methods and by semiquantitative real-time polymerase chain reaction analysis for the corresponding messenger RNA. Disease-free survival was analyzed by the Kaplan-Meier method. Tumors with low cystatin C protein expression and high cathepsin B protein expression were significantly more likely to be of high grade, and this pattern was significantly correlated with high Ki-67 LI and tumor recurrence. Depressed expression of cystatin C messenger RNA in glioblastomas compared with low-grade astrocytomas was demonstrated. Multivariate analysis demonstrated high tumor grade, high Ki-67 labeling index, high cathepsin B expression, and low cystatin C expression correlated significantly with shorter disease-free survival. These results suggest that gliomas in patients with an unfavorable clinical outcome are characterized by depressed expression of cystatin C. Evaluation of cystatin C expression in gliomas provides useful clinical information, especially as a prognostic indicator.
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Neoplasias Encefálicas/metabolismo , Cistatinas/metabolismo , Glioma/metabolismo , Adolescente , Adulto , Anciano , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/patología , Catepsina B/metabolismo , Niño , Preescolar , Cistatina C , Supervivencia sin Enfermedad , Femenino , Glioma/diagnóstico , Glioma/patología , Humanos , Inmunohistoquímica , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Pronóstico , Análisis de SupervivenciaRESUMEN
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a rare hereditary disease characterized by recurrent transient ischemic attacks (TIA) and strokes, and vascular dementia caused by point mutations of the Notch 3 gene. Here, we report a Japanese CADASIL case who displayed limited dementia and had the Notch 3 R141C mutation. The relationship between pathogenesis and the mutation site in Notch 3 is discussed based on the case presented here.
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CADASIL/genética , Demencia por Múltiples Infartos/genética , Mutación Puntual/genética , Proteínas Proto-Oncogénicas/genética , Receptores de Superficie Celular/genética , Femenino , Humanos , Imagen por Resonancia Magnética , Persona de Mediana Edad , Mutación MissenseRESUMEN
We report two rare cases of progressive congestive myelopathy caused by dural arteriovenous fistulae (DAVF) at the foramen magnum. The first, a 69-year-old male with a 2-year history of progressive myelopathy, had symptoms unrecognized due to a past history that included spinal caries and congenital dislocation of the hip. The second, a 60-year-old male, had a recurrence of the myelopathy three months after endovascular occlusion of the DAVF feeding artery. Both patients were successfully treated by direct microsurgical interruption of the arterialized medullary vein with functional and radiological improvement. The clinical manifestations of DAVF at the foramen magnum are nonspecific, mimicking those of cervical spondylotic myelopathy or cervical neoplasm. Accurate and early diagnosis followed by complete obliteration of the fistula is mandatory to avoid permanent neurological deficit.