RESUMO
Presentamos en nuestro trabajo, un caso de liponeurocitomacerebeloso, tumor poco frecuente, observado en unamujer de 62 años de edad. Dicho tumor mostró una imagenhistológica compatible con un tumor de células pequeñas,de escasa agresividad, positivo para marcadores neuronales(sinaptofisina y enolasa neuronal específica) con áreasfocales de diferenciación lipídica. Tras establecer un diagnósticodiferencial con otras neoplasias, comentamos brevementelas características de esta rara entidad(AU)
We report a case of cerebellar liponeurocytoma in a 62year old woman. Histologically, the tumour was of lowaggressiveness, composed of small cells with areas of lipidisedcells and was positive for neuronal markers (synaptophysinand neuron specific enolase). The principal featuresof this unusual entity are reviewed and the pathologicalaspects of its differential diagnosis are discussed(AU)
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Neoplasias Cerebelares/diagnóstico , Neoplasias Cerebelares/patologia , Diagnóstico Diferencial , Cerebelo/anatomia & histologia , Cerebelo/patologia , CerebeloRESUMO
Traumatic neuromas (TN) of the biliary tree causing strictures have only occasionally been described after liver transplantation. Herein, we have reported 15 cases of TN that were detected between 1 and 17 months after transplantation (median: 4 months) during surgery for obstructive jaundice (12 cases), after alterations of liver function tests (two cases), or incidentally discovered after retransplantation (n = 1) we resected the lesion and the biliary anastomosis. Pathological examination and immunostaining for S-100 protein were performed to study the nerve fascicles. After a median follow-up time of 64 months (range = 0-127), 10 patients are alive without any complication related to the previous biliary TN. We propose the following classification: type I: TN originating from and located in the main biliary tract wall, and type II: TN arising from the surrounding tissues next to the main biliary tract. We conclude that TN are not uncommon after liver transplantation and that they are sometimes symptomatic, causing a biliary stricture that requires surgical treatment. We propose a classification to help patient selection for surgery. In our opinion, resection of the TN is the operation of choice, together with resection of the involved biliary tract in type I TN.
Assuntos
Neoplasias dos Ductos Biliares/diagnóstico , Ductos Biliares Extra-Hepáticos , Transplante de Fígado/efeitos adversos , Neuroma/diagnóstico , Adolescente , Adulto , Idoso , Neoplasias dos Ductos Biliares/epidemiologia , Neoplasias dos Ductos Biliares/terapia , Colangiopancreatografia Retrógrada Endoscópica , Feminino , Seguimentos , Humanos , Imunossupressores/uso terapêutico , Transplante de Fígado/imunologia , Masculino , Pessoa de Meia-Idade , Neuroma/epidemiologia , Neuroma/terapia , Complicações Pós-Operatórias/epidemiologia , Reoperação , Estudos Retrospectivos , Fatores de Tempo , Adulto JovemAssuntos
Encéfalo/fisiopatologia , Eletroencefalografia , Doença por Corpos de Lewy/fisiopatologia , Idoso , Encéfalo/metabolismo , Encéfalo/patologia , Humanos , Doença por Corpos de Lewy/metabolismo , Doença por Corpos de Lewy/patologia , Estudos Longitudinais , Masculino , Periodicidade , Ubiquitina/metabolismoRESUMO
Introducción. La disección aguda tipo B de la aorta torácica es una entidad compleja y grave, asociada a una elevada morbilidad y mortalidad. Desarrollo. Tradicionalmente, el tratamiento médico se ha preferido para las disecciones no complicadas, y se ha reservado la reparación quirúrgica para las que presentan complicaciones isquémicas o rotura. El tratamiento endovascular con endoprótesis ha aportado mayores esperanzas para los casos complicados, a la vez que se ha postulado su aplicación en casos asintomáticos. Estos aspectos se revisan y se discuten a fondo en el presente documento. Asimismo, se analizan aspectos técnicos del tratamiento endovascular pertenecientes al pre, peri y posprocedimiento. Conclusión. Por el momento, el uso de endoprótesis sólo está justificado en el tratamiento de disecciones agudas complicadas o inestables, o en las crónicas con expansión aneurismática
Introduction. Acute type B dissection of the thoracic aorta is a complex, severe condition associated to a high rate of morbidity and mortality. Development. Medical treatment has traditionally been preferred for non complicated dissections while surgical repair has been reserved for those with ischaemic complications or rupture. Endovascular treatment with a stent has brought greater hope for complicated cases, and its application in asymptomatic cases has also been suggested. These issues are reviewed and discussed at length in this article. Likewise, technical aspects of the endovascular treatment belonging to the pre-, peri- and post-operative periods are also analysed. Conclusions. For the time being, the use of stents is only justified in the treatment of complicated or unstable acute dissections, or in chronic cases with aneurysmal expansion
Assuntos
Procedimentos Cirúrgicos Vasculares/métodos , Dissecação/métodos , Dissecação da Artéria Carótida Interna/diagnóstico , Dissecação da Artéria Carótida Interna/cirurgia , Isquemia/complicações , Próteses e Implantes , Eletrocardiografia/métodos , Radiografia Torácica/métodos , Aortografia/métodos , Cuidados Pós-Operatórios/métodos , Indicadores de Morbimortalidade , Eletrocardiografia/tendências , Eletrocardiografia , Tomografia Computadorizada de Emissão/métodos , Ecocardiografia Transesofagiana/métodos , Espectroscopia de Ressonância Magnética/métodosRESUMO
Objetivo. Estudiar los cambios morfológicos de la pared aórtica en la disección crónica tipo B de Stanford tratada mediante stents recubiertos. Pacientes y métodos. Fueron tratados 17 pacientes (edad media: 61 años; rango: 35-70 años) por disección crónica tipo B. Se implantaron endoprótesis Talent (n = 11) y Excluder (n = 6); abordaje femoral (n = 15) y retroperitoneal (n = 2); control intraoperatorio mediante fluoroscopia y ecocardiografía transesofágica; cobertura intencionada de la arteria subclavia en cinco casos. Resultados. Éxito técnico con cierre de la puerta de entrada (17/17); tiempo quirúrgico: 73 ± 22 min (rango: 55-160 min); sin conversiones ni paraplejía; estancia postoperatoria media: 48 horas; trombosis de la falsa luz torácica completa (n = 13) y parcial (n = 4). El diámetro mínimo de la luz verdadera (LV) aumentó de 18,1 ± 5,4 a 27,9 ± 5,06 mm (p < 0,01). La luz falsa (LF) se redujo de 39,22 ± 18 a 26,6 ± 12,7 mm (p < 0,01). Seguimiento medio: 18 meses (rango: 1-58 meses); sin migraciones, fugas u otras complicaciones relacionadas con la endoprótesis; tres pacientes necesitaron a posteriori cobertura de la reentrada distal. Conclusiones. El cierre de la puerta de entrada mediante stents recubiertos suele inducir la trombosis en la LF torácica, ello conlleva una remodelación aórtica: estabilización del diámetro aórtico máximo, con cambios estadísticamente significativos del índice LV/LF (reducción de la LF, aumento de la LV). La aorta abdominal suele permanecer inalterada por persistencia de reentradas paraviscerales. Los resultados preliminares sugieren que el tratamiento endovascular de la disección crónica tipo B es una opción terapéutica factible y efectiva, con excelentes resultados a medio plazo
Aim. To study the morphologic evolution in the aortic wall of the chronic aortic type B dissection treated by means of stent-graft. Patients and methods. 17 patients (average age: 61 years; range 35-70 years) were treated by chronic aortic type B dissection. The endoprosthesis implanted were Talent (n = 11) and Excluder (n = 6); femoral access (n = 15) and iliac (n = 2); intraoperative transesophageal ecocardiography; intentionally left subclavian artery coverture in five patients. Results. Closure of the entry tear in all cases; surgical time: 73 ± 22 min (range: 55-160 min); no conversions, no neurological complications; median postoperative length of stay 48 hours; thrombosis of the thoracic false lumen (FL), complete (n = 13) and partial (n = 4). The minimum diameter of the true lumen (TL) increase from 18.1 ± 5.4 to 27.9 ± 5.06 mm (p < 0.01). FL reduction from 39.22 ± 18 o 26.6 ± 12.7 mm (p < 0.01). Mean follow up: 18 months; no related graft complications. In the follow up three patients needed to seal distal thoracic re-entry. Conclusions. The clossure of the entry tear usually induces the thrombosis of the thoracic FL. It promotes a remodelling of the aortic wall consistent in the stabilization of the maximum aortic diameter with significant changes of the index TL/FL (reduction of the LF and increase of the TL). The abdominal aorta usually remains unchanged by persistence of paravisceral reentries. The preliminary results suggest that stent-graft repair of the aortic dissection B type is a feasible and effective therapeutic option with excellent mid-term results
Assuntos
Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Humanos , Angioplastia/métodos , Aneurisma da Aorta Torácica/cirurgia , Prótese Vascular , Aorta Torácica/fisiopatologia , Complicações Pós-Operatórias/epidemiologiaRESUMO
Malignant peripheral nerve sheath tumors (MPNST) are rare. We report a case of a MPNST of the proximal sciatic nerve in the thigh, unassociated with pre-existing type 1 neurofibromatosis or history of radiation therapy. A 71-year-old man had a 6 month history of constant, severe, burning pain affecting the sole of the left foot. One month after the onset, the pain radiated to the left calf, posterior aspect of the thigh and buttock, and distal leg weakness followed. Three months prior to admission, the patient developed a large and painful mass in the buttock, that occupied the entire left gluteal region on examination. There was severe weakness of ankle and toe dorsiflexion and plantarflexion, decreased sensation on the lateral and posterior aspect of the left leg as well as on the dorsal and plantar surfaces of the foot, and absent ankle jerk. EMG showed denervation and motor unit loss in the short head of biceps femoris and muscles supplied by tibial and peroneal nerves on the left side. Magnetic resonance imaging revealed a 10-cm enhancing mass of the left sciatic nerve from the upper thigh to the greater sciatic notch. In surgery, a large MPNST with a high Ki67 labeling index (> 60 %) was subtotally removed from the sciatic nerve, and adjuvant radiation therapy was administered. In the ensuing months the tumor invaded the entire pelvic region. A high sciatic malignant tumor can present with a rapidly growing buttock mass and unilateral, neuropathic foot pain imitating the tarsal tunnel syndrome.
Assuntos
Neoplasias de Bainha Neural/diagnóstico , Neoplasias do Sistema Nervoso Periférico/diagnóstico , Nervo Isquiático/patologia , Idoso , Nádegas , Terapia Combinada , Diagnóstico Diferencial , Eletromiografia , Humanos , Imageamento por Ressonância Magnética , Masculino , Invasividade Neoplásica , Neoplasias de Bainha Neural/patologia , Neoplasias de Bainha Neural/radioterapia , Neoplasias de Bainha Neural/cirurgia , Dor/etiologia , Neoplasias do Sistema Nervoso Periférico/patologia , Neoplasias do Sistema Nervoso Periférico/radioterapia , Neoplasias do Sistema Nervoso Periférico/cirurgia , Radioterapia Adjuvante , Nervo Isquiático/cirurgia , Síndrome do Túnel do Tarso/diagnósticoRESUMO
Meningiomas are unusual in childhood, and the intraspinal location is very uncommon. Those arising from lumbar dura matter are the most rare among spinal neoplasms of meningeal origin. We present the case of a 12-years-old girl with a cauda equina meningioma. As in previously reported cases, the initial complain was back pain with radicular irradiation. The girl had few neurological findings, with pyramidal signs of both lower limbs as single neurological impairment. She underwent surgical treatment through an open door laminoplasty and the tumour was completely removed without neurological deficit. After 2-year of follow-up she remains asymptomatic.
Assuntos
Cauda Equina/cirurgia , Meningioma/cirurgia , Neoplasias do Sistema Nervoso Periférico/cirurgia , Dor nas Costas/etiologia , Cauda Equina/patologia , Criança , Feminino , Humanos , Imageamento por Ressonância Magnética , Meningioma/diagnóstico , Meningioma/patologia , Neoplasias do Sistema Nervoso Periférico/diagnóstico , Neoplasias do Sistema Nervoso Periférico/patologiaRESUMO
Los meningiomas son tumores raros en los niños, siendo especialmente inusual su localización raquídea y excepcional su origen en la duramadre de la teca lumbar. Presentamos el caso de una niña de 12 años con un meningioma de cauda equina. En concordancia con la literatura el síntoma inicial fue lumbalgia con irradiación radicular, siendo escasos los hallazgos de la exploración neurológica, que se limitaban a la presencia de signos piramidales en ambas extremidades inferiores. Fue intervenida mediante una laminoplastia en puerta abierta, consiguiéndose la resección completa de la tumoración sin déficit neurológico postoperatorio. La niña permanece asintomática dos años después de la cirugía (AU)
No disponible
Assuntos
Criança , Feminino , Humanos , Meningioma , Dor nas Costas , Cauda Equina , Imageamento por Ressonância Magnética , Neoplasias do Sistema Nervoso PeriféricoRESUMO
BACKGROUND: Cardiac angiosarcoma is a rare tumor that has a predilection for middle-aged males and a marked predominance in the right atrium. The tumor may present abruptly with a fulminant clinical course. Initial presentation with metastatic disease is rare. Only one case has been reported of a patient who presented with cutaneous metastases. METHODS AND RESULTS: We here report the case of a 51-year-old man who initially presented with cutaneous metastases in the absence of cardiac symptoms. The skin biopsy was diagnosed as metastatic undifferentiated sarcoma. The patient died 26 days later with widely disseminated disease. At autopsy a tumor arising in the wall of the left atrium and in the interatrial septum was found. After an immunohistochemical study including CD31 and CD34 stains the diagnosis of cardiac pure epithelioid angiosarcoma was made. To the best of our knowledge this tumor variant has not been documented in the heart until now. CONCLUSION: Although cardiac angiosarcoma is a rare neoplasm, its presence should be suspected in patients with cutaneous metastatic angiosarcoma without an evident source of the tumor, even in absence of cardiac symptoms.
Assuntos
Células Epitelioides/patologia , Neoplasias Cardíacas/diagnóstico , Hemangiossarcoma/diagnóstico , Neoplasias Cutâneas/diagnóstico , Antígenos CD34/análise , Biomarcadores Tumorais/análise , Biópsia , Diagnóstico Diferencial , Evolução Fatal , Átrios do Coração/patologia , Neoplasias Cardíacas/química , Hemangiossarcoma/química , Hemangiossarcoma/secundário , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Metástase Neoplásica , Proteínas de Neoplasias/análise , Molécula-1 de Adesão Celular Endotelial a Plaquetas/análise , Neoplasias Cutâneas/química , Neoplasias Cutâneas/secundárioRESUMO
Neoplasm is an uncommon cause of a parkinsonian syndrome. We report a woman with primary brain B-cell lymphoma presenting as Parkinson's disease. After 1 year of the illness, CT and MRI showed lesions without mass effect in the basal ganglia and corpus callosum. The patient did not respond to levodopa and right cerebellar and brain-stem signs appeared, which prompted further neuroimaging, showing an increase in size of the lesions and a right cerebellar and pontine mass. Stereotactic biopsy of the basal ganglia showed high-grade B-cell lymphoma. Despite the basal ganglia frequently being involved in lymphoma of the brain, presentation with typical or atypical parkinsonism is exceptional.
Assuntos
Neoplasias Encefálicas/complicações , Linfoma de Células B/complicações , Doença de Parkinson/etiologia , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Neoplasias Encefálicas/diagnóstico , Feminino , Humanos , Linfoma de Células B/diagnóstico , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios XRESUMO
OBJECTIVE: To assess if axonal damage in severe acute inflammatory demyelinating polyneuropathy (AIDP) correlates with the appearance of epiperineurium in nerve trunks. BACKGROUND: Increase of endoneurial fluid pressure in nerve trunks possessing epiperineurium may be an important mechanism of axonal damage in AIDP. METHODS: A 79-year-old man had a 2-day history of acroparesthesias and ascending paralysis culminating in quadriplegia, bilateral facial palsy, and mechanical ventilation. Five intravenous immunoglobulin cycles were given without response. He died on day 60. Electrophysiologic studies (days 4, 17, and 50) initially showed normal nerve conduction velocities with further slowing, progressive attenuation of compound muscle action potentials, and profuse denervation. The authors studied the preforaminal anterior and posterior L3 and L5 spinal roots, third and fifth lumbar nerves and their branches, and femoral and sural nerves. RESULTS: Density of myelinated fibers was preserved in L5 ventral and dorsal roots and reduced in sural nerve. Mild de-remyelination was observed in lumbar roots. In both lumbar nerves and their branches, there were extensive de-remyelination and centrofascicular or wedge-shaped areas with marked loss of large myelinated fibers. Axonal degeneration was the predominant lesion in sural nerve. CONCLUSION: The presence of epiperineurium correlates with a drastic change of pathology with superimposed ischemic lesions and distally accentuated axonal loss, suggesting that endoneurial fluid pressure increase could cause axonal damage in AIDP.
Assuntos
Axônios/patologia , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/fisiopatologia , Nervos Periféricos/fisiopatologia , Potenciais de Ação , Idoso , Axônios/ultraestrutura , Carcinoma de Células de Transição/complicações , Carcinoma de Células de Transição/patologia , Eletromiografia , Evolução Fatal , Síndrome de Guillain-Barré/complicações , Síndrome de Guillain-Barré/tratamento farmacológico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Região Lombossacral , Masculino , Condução Nervosa , Medula Espinal/patologia , Raízes Nervosas Espinhais/fisiopatologia , Nervo Sural/fisiopatologia , Neoplasias da Bexiga Urinária/complicações , Neoplasias da Bexiga Urinária/patologiaRESUMO
The clinical presentation and evolution, neuropathological findings, and genotyping of three members of a Spanish family affected with fatal familial insomnia are reported. The mother and two of her offspring developed a rapidly evolving disease with insomnia and behavioural disorders as the initial symptoms and died between 5 and 10 months after the onset of the illness. Frontal brain biopsy in the mother disclosed only non-significant spongiosis, and full neuropathological examination of her offspring showed thalamic and olivary degeneration with isolated focal cortical spongiosis. Genetic examination could only be performed in the contemporary patients and both harboured the prion protein (PrP) 178Asn mutation and homozygous 129 Met/Met genotype.
Assuntos
Doenças Priônicas/genética , Biópsia , Aberrações Cromossômicas/genética , Transtornos Cromossômicos , Feminino , Lobo Frontal/patologia , Genes Dominantes/genética , Genótipo , Humanos , Masculino , Pessoa de Meia-Idade , Testes Neuropsicológicos , Núcleo Olivar/patologia , Linhagem , Mutação Puntual/genética , Doenças Priônicas/diagnóstico , Doenças Priônicas/patologia , Príons/genética , Tálamo/patologiaRESUMO
To explore the mechanisms underlying L-dopa response, we studied, by postmortem autoradiography, selective makers of dopamine presynaptic terminals, [3H]WIN 35428, and dopamine D2 receptors, [3H]nemonapride, in the putamen of four Parkinson's disease (PD) and one striatonigral degeneration (SND) neuropathologically confirmed brains as compared with six matched control brains. Dopamine uptake transporter was dramatically decreased (> 90%) both in PD and SND striatum. Dopamine D2 receptors were preserved in PD, but clearly reduced (> 76%) in the SND putamen. These data confirm that L-dopa response is closely associated with the preservation of striatal dopamine D2 receptors.
Assuntos
Proteínas de Transporte/metabolismo , Glicoproteínas de Membrana , Proteínas de Membrana Transportadoras , Proteínas do Tecido Nervoso , Receptores de Dopamina D2/metabolismo , Degeneração Estriatonigral/metabolismo , Idoso , Autorradiografia , Benzamidas/metabolismo , Cadáver , Cocaína/análogos & derivados , Cocaína/metabolismo , Corpo Estriado/metabolismo , Antagonistas de Dopamina/metabolismo , Proteínas da Membrana Plasmática de Transporte de Dopamina , Inibidores da Captação de Dopamina/metabolismo , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Parkinson/metabolismo , Putamen/metabolismo , Valores de Referência , Degeneração Estriatonigral/patologiaRESUMO
Much clinical and biologic data have been processed in the search for useful objective parameters to predict brain tumor behavior. Seventy cases of astrocytic glioma collected by a single clinical team were studied using a full complement of clinical procedures: follow up (7 years), histologic analysis, DNA content estimation, and cell kinetics by flow cytometry. Proliferating cell nuclear antigen (PCNA) was determined by immunocytochemical-coupling flow cytometry (PFC) and also by counting under light microscopy (PIHC). A statistical evaluation was carried out to establish the usefulness of several parameters for glioma prognosis. The cases were histologically classified as 14 low-grade astrocytomas, 20 anaplastic astrocytomas, and 36 glioblastomas multiforme. The survival curve showed significant differences between histologic groups. Diploid populations were more frequent in low-grade astrocytomas, and aneuploid tumors often had increased S-phase and proliferative fractions. The PCNA-labeled index (PCNA-LI) increased with malignancy and correlated with histologic grading (P = 0.01). The PCNA-LI and age segregated low- from high-grade astrocytomas (including anaplastic astrocytoma and glioblastoma multiforme), but none of the variables considered differentiated anaplastic astrocytoma from glioblastoma multiforme. The Cox regression test displayed significant values for age, histologic diagnosis, and PCNA determinations when considered in tandem. Discriminant analysis obtained a function integrating age and specifically PIHC-LI to help in the prognosis of doubtful cases. The results emphasize the importance of parameters integrating different variables in an attempt to provide an accurate prognosis, the most significant being age, histopathologic diagnosis, and the proliferative fraction determined by PCNA.
Assuntos
Biomarcadores Tumorais/metabolismo , Neoplasias Encefálicas/patologia , Glioma/patologia , Adulto , Estudos de Coortes , DNA/análise , Diagnóstico Diferencial , Feminino , Citometria de Fluxo , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Antígeno Nuclear de Célula em Proliferação/metabolismoRESUMO
Using [3H]sumatriptan as a radioligand, 5-hydroxytryptamine (5-HT)1B receptors were examined in posterior striatum and midbrain post-mortem tissue sections of 12 patients who had died from representative degenerative movement disorders as compared to nine controls. In the control human basal ganglia, the highest densities of [3H]sumatriptan binding were observed in the globus pallidus and substantia nigra. No significant change in the density of [3H]sumatriptan binding sites was found in the striatum and substantia nigra of the six Parkinson's disease brains. In the two brains from patients with progressive supranuclear palsy an increase was found in the densities of [3H]sumatriptan binding sites, most marked in the substantia nigra. In contrast, [3H]sumatriptan labelling was almost absent in the striatonigral degeneration brain and was markedly reduced in the three Huntington's disease brains. This study indicates that the status of 5-HT1B receptors is different in each degenerative movement disorder and suggests that human 5-HT1B receptors are located somatodendritically on GABAergic and peptidergic caudate-putamen neurons which project to the substantia nigra and globus pallidus, where these receptors are presynaptic.
Assuntos
Corpo Estriado/química , Transtornos dos Movimentos/metabolismo , Receptores de Serotonina/análise , Receptores de Serotonina/metabolismo , Substância Negra/química , Idoso , Idoso de 80 Anos ou mais , Corpo Estriado/patologia , Feminino , Humanos , Doença de Huntington/metabolismo , Doença de Huntington/patologia , Masculino , Pessoa de Meia-Idade , Transtornos dos Movimentos/patologia , Degeneração Neural/metabolismo , Degeneração Neural/patologia , Doença de Parkinson/metabolismo , Doença de Parkinson/patologia , Ensaio Radioligante , Receptor 5-HT1B de Serotonina , Agonistas do Receptor de Serotonina/metabolismo , Agonistas do Receptor de Serotonina/farmacologia , Substância Negra/patologia , Sumatriptana/metabolismo , Sumatriptana/farmacologia , Paralisia Supranuclear Progressiva/metabolismo , Paralisia Supranuclear Progressiva/patologia , TrítioRESUMO
The pathological basis of nerve inexcitability in Guillain-Barré syndrome has not been established with certainty. We report the clinicopathological findings in a 67-year-old patient with fulminant Guillain-Barré syndrome who died 18 days after onset. Three serial electrophysiological studies revealed nerve inexcitability. Antibodies to Campylobacter jejuni were present but there was no antiganglioside reactivity. Spinal root sections revealed extensive and almost pure macrophage-associated demyelination with occasional presence of T lymphocytes and neutrophil leukocytes. Conversely, in femoral, median, and sural nerves the outstanding lesion was axonal degeneration, with some denuded axons remaining. Unmyelinated fibers, posterior root ganglia, and dorsal columns were preserved. Endoneurial postcapillary venules showed plump endothelial cells with loss of their tight junctions. We conclude that both primary demyelination and axonal degeneration secondary to inflammation account for nerve inexcitability. Our findings lend support to the hypothesis of increased endoneurial pressure as the cause of wallerian degeneration in nerve trunks.
Assuntos
Nervos Periféricos/fisiopatologia , Polirradiculoneuropatia/fisiopatologia , Potenciais de Ação/fisiologia , Idoso , Axônios/fisiologia , Plexo Braquial/patologia , Plexo Braquial/fisiopatologia , Eletromiografia , Nervo Facial/patologia , Nervo Facial/fisiopatologia , Evolução Fatal , Humanos , Masculino , Nervo Mediano/patologia , Nervo Mediano/fisiopatologia , Neurônios Motores/fisiologia , Neurônios Motores/ultraestrutura , Condução Nervosa/fisiologia , Neurônios Aferentes/fisiologia , Neurônios Aferentes/ultraestrutura , Nervos Periféricos/patologia , Nervo Fibular/patologia , Nervo Fibular/fisiopatologia , Raízes Nervosas Espinhais/patologia , Raízes Nervosas Espinhais/fisiopatologia , Nervo Tibial/patologia , Nervo Tibial/fisiopatologia , Degeneração Walleriana/fisiologiaRESUMO
INTRODUCTION: The peripheral neuropathy is a common complication in patients with osteosclerotic myeloma. The association with hepatosplenomegaly, lymphadenopathy and variable endocrine disturbances and skin changes constitute the POEMS syndrome. The pathogenesis of this syndrome is unclear, but the presence of M protein and elevated levels of interleukin-6 in the serum of patients, suggests an immunologic mechanism. CASE: A 68 year old woman presented with a 2 year history of progressive weakness and paresthesias in both legs. Neurological examination revealed a sensorimotor polyneuropathy, involving predominantly the lower extremities. In addition to neurological findings, the patient had lymphadenopathy and skin hyperpigmentation. Serum protein immunoelectrophoresis revealed a peak of monoclonal IgA lambda paraprotein. Radiographic examination disclosed multiple osteosclerotic lesions. Electrophysiological evaluation showed a severe axonal and demyelinating sensorimotor polyneuropathy. Biopsy of sural nerve revealed demyelination and axonal degeneration. Axillary lymph node biopsy were consistent with giant lymph node hyperplasia. Interleukin-6 serum levels were normal. CONCLUSIONS: The POEMS syndrome or Crow-Fusake syndrome is a rare multisystem disorder often associated with osteosclerotic myeloma. A severe demyelinating sensorimotor polyneuropathy frequently constitutes the chief complaint. These patients have a polyneu-ropathy which resembles idiopathic chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and the electrophysi-ological studies are essential in the diagnosis. The pathogenesis has not been explained. Presumably, the plasma cells secrete an immunoglobulin or another substance that is toxic to peripheral nerves. Interleukin-6 is implicated in the pathogenesis of this disease.
Assuntos
Mieloma Múltiplo/patologia , Osteosclerose/patologia , Síndrome POEMS/diagnóstico , Neoplasias do Sistema Nervoso Periférico/patologia , Coluna Vertebral/patologia , Nervo Sural/patologia , Idoso , Anticorpos Monoclonais , Doenças Desmielinizantes , Ensaio de Imunoadsorção Enzimática , Potenciais Somatossensoriais Evocados , Feminino , Humanos , Interleucina-6/sangue , Mieloma Múltiplo/complicações , Osteosclerose/complicações , Síndrome POEMS/complicações , Neoplasias do Sistema Nervoso Periférico/complicaçõesAssuntos
Ataxia/etiologia , Ventriculografia Cerebral , Síndrome de Creutzfeldt-Jakob/diagnóstico por imagem , Atrofia , Encéfalo/patologia , Cerebelo/patologia , Eletroencefalografia , Evolução Fatal , Feminino , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios XRESUMO
Oligodendrogliomas (n = 26) induced by ethylnitrosourea (ENU) in wistar rats were examined to assess the lectin specificity to oligodendroglial membranes. Two different types of oligodendrogliomas were found in our material: an isomorphous type (n = 12), and a polymorphous type (n = 14). The first one, with two variants according to its size, macro- (n = 9) and microtumors (n = 3), had predominantly a honey-comb pattern with 'clear halos' around the nuclei without anaplasia. The second type, composed mostly by macrotumours, was anaplastic, with high cellular density, necrosis and intratumoral hemorrhages. Peanut agglutinin (PNA) labelled plasma membranes of well-differentiated cellular components of the first group. The tumoral oligodendrocytes lost the property to bind PNA in the second group of tumours, while Concanavalin A (Con A) showed affinity to intracytoplasmic structures of these tumours. PNA is a reliable marker of oligodendroglial plasma membrane of well-differentiated ENU-induced oligodendrogliomas. This experimental model, using PNA and Con A, may have important clinical applications regarding the biological behaviour of this type of neoplasm.
Assuntos
Neoplasias Encefálicas/patologia , Oligodendroglioma/patologia , Animais , Encéfalo , Neoplasias Encefálicas/induzido quimicamente , Neoplasias Encefálicas/metabolismo , Carcinógenos , Concanavalina A/metabolismo , Etilnitrosoureia , Imuno-Histoquímica , Lectinas/metabolismo , Oligodendroglioma/induzido quimicamente , Oligodendroglioma/metabolismo , Aglutinina de Amendoim , Ratos , Ratos WistarRESUMO
Endothelin-1 is a vasoconstrictor peptide released by the vascular endothelium by various chemical and mechanical factors. Cellular rejection is one of the most common complications following orthotopic liver transplantation, endotheliitis being its most specific and consistent histological feature. To assess the role of endothelin in cellular hepatic rejection, we studied 21 cirrhotic patients undergoing elective liver transplantation. These patients were divided in two groups: Group I comprised 10 patients without cellular rejection, and Group II comprised 11 patients with cellular rejection. Endothelin was measured on day 7 after transplantation, on the day of liver biopsy, and after treatment for rejection. We found a significant increase in the plasma endothelin levels in the early postoperative period in the patients with moderate or severe cellular rejection compared with the non-rejection group. It remained significantly elevated until the clinical diagnosis of rejection was made. This value returned to baseline with successful treatment. So, the monitorization of this peptide may be of help in the diagnosis of rejection, its severity, and the evaluation of its resolution.