1/37. Brain abscesses associated with pulmonary arteriovenous malformations.We present three patients with brain abscesses who also had pulmonary arteriovenous malformations (AVMs). All patients had hereditary haemorrhagic telangiectasia (Osler's disease). It is probable that the lung AVMs contributed to the development of the brain abscesses by allowing bacteria easier access to the systemic circulation through a right to left pulmonary vascular shunt, bypassing the filtering action of the pulmonary capillaries. In addition, one patient required a period of postoperative ventilation using PEEP, which may have exacerbated the shunt through the lung AVM and led to difficulty in weaning her from the ventilator.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/37. brain abscess in patients with hereditary hemorrhagic telangiectasia: case report and literature review.Hereditary hemorrhagic telangiectasia (HHT), or Osler-Weber-Rendu disease, affects multiple organ systems. brain abscess is a potential complication, and this disease carries a high mortality. In the setting of HHT the abscess most likely results from paradoxical septic emboli or bacterial seeding of an ischemic portion of the brain after paradoxical sterile emboli. brain abscess is the diagnosis that must be ruled out in patients with HHT presenting with new onset neurologic symptoms. The clinician can be misled by seemingly benign and nonspecific symptoms, signs, and laboratory test results. Appropriate diagnostic imaging with computed tomography or magnetic resonance imaging of the head is mandatory. We present a case of brain abscess in a patient with HHT presenting to the Emergency Department. The review of the literature deals with the pathophysiology and manifestations of HHT with particular focus on the pathologic and clinical features, and management of cerebral abscess in this setting. Differences between patients with brain abscess with or without HHT are highlighted.- - - - - - - - - - ranking = 1.5keywords = brain (Clic here for more details about this article) |
3/37. Osler-Weber-Rendu syndrome--pathological manifestations and autopsy considerations.An 18-year-old university student with Osler-Weber-Rendu disease collapsed in the bathroom. Attempted resuscitation was unsuccessful. Her past history included recurrent epistaxes, mucosal telangiectasias, intracranial arteriovenous malformations with previous hemorrhage, a single pulmonary arteriovenous malformation, recent onset of grand mal seizures, and depression. There was a positive paternal family history. At autopsy the pulmonary arteriovenous malformation was confirmed. In addition, within the brain there were widespread areas of gliosis with hemosiderin deposition and small vascular malformations. No other significant abnormalities were detected and toxicological screening revealed only moderately elevated levels of carbemazepine. death was attributed to epilepsy associated with glial scarring from previous microhemorrhages. The detection of vascular malformations in the lung or brain at autopsy should prompt careful examination of all tissues for similar lesions. Given the possibility of Osler-Weber-Rendu disease in such cases, fibroblast cultures should be undertaken to enable molecular studies to proceed. The heritable nature of this disorder necessitates accuracy of diagnosis at autopsy; once the diagnosis is confirmed family screening should be recommended.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
4/37. Multiple coronary artery-left ventricular fistulas associated with hereditary hemorrhagic telangiectasia.Coronary artery-left ventricular (LV) fistulas are extremely rare and can cause myocardial ischemia from coronary steal. We describe an elderly woman who presented with unstable angina from multiple and extensive coronary artery-LV fistulas. She also had clinical features suggestive of hereditary hemorrhagic telangiectasia (HHT). association of coronary artery-LV fistulas with HHT has not been reported and can pose a management dilemma in view of the risks of extensive cardiopulmonary surgery and potential complications of myocardial ischemia, stroke, and brain abscess.- - - - - - - - - - ranking = 0.5keywords = brain (Clic here for more details about this article) |
5/37. External beam radiotherapy for severe epistaxis from Osler-Weber-Rendu disease.Hereditary Hemorrhagic Telangiectasia (HHT) is a genetic disorder of blood vessels, which affects about 1 in 10,000 people. The disorder is also sometimes referred to as Osler-Weber-Rendu disease after the doctors who studied HHT. The disorder affects males and females from all racial and ethnic groups, and the most commonly affected organs are the nose, lungs, gastrointestinal tract, and brain--in that order. radiation therapy should be considered for intractable bleeding from the upper airway or alimentary tract.- - - - - - - - - - ranking = 0.5keywords = brain (Clic here for more details about this article) |
6/37. Recurrent brain abscess associated with hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease)--case report.A 53-year-old mentally handicapped female with hereditary hemorrhagic telangiectasia complicated by pulmonary arteriovenous fistula developed a recurrent brain abscess in the left frontal region. She responded well to surgical drainage and antibiotic therapy. Previously, she had two brain abscesses of the left parieto-occipital and right parietal regions removed on separate occasions at 38 and 40 years of age. Although pulmonary arteriovenous fistula was eventually confirmed by angiography at age 41 years, surgical removal of the pulmonary lesion was not indicated due to multiplicity and large vascular shunting. Early recognition of pulmonary arteriovenous fistula in patients with hereditary hemorrhagic telangiectasia is emphasized for diagnosis of brain abscess and prevention of recurrence.- - - - - - - - - - ranking = 3.5keywords = brain (Clic here for more details about this article) |
7/37. Giant spinal perimedullary fistula in hereditary haemorrhagic telangiectasia: diagnosis, endovascular treatment and review of the literature.Hereditary hemorrhagic telangiectasia (HHT, Rendu-Osler-Weber disease) is a vascular disorder with dominant autosomal transmission characterised usually by multiple mucocutaneous and visceral abnormalities. Neurological manifestations due to the primary involvement of spinal cord by vascular malformations are rare. We present a young man with HHT associated with a central nervous system arteriovenous malformation and a giant perimedullary fistula, that was manifested as progressive myelopathy. The diagnosis was made coupling magnetic resonance imaging to selective spinal arteriography. The therapeutic option was endovascular treatment by mechanically detachable coils which resulted in full exclusion of the fistula with full improvement of symptoms. During follow-up a stable clinical and morphological outcome was achieved. Clinical manifestations in HHT, with emphasis on neurological symptoms, are reviewed as well as the therapeutic options to deal with giant perimedullary fistula.- - - - - - - - - - ranking = 0.38738226614558keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/37. central nervous system lesions associated with hereditary hemorrhagic telangiectasia--three case reports.Hereditary hemorrhagic telangiectasia (HHT) is easily overlooked in patients with central nervous system (CNS) lesions. Our clinical experience of three patients with CNS lesions associated with HHT stresses the importance of considering HHT. A 23-year-old male presented with consciousness disturbance and right hemiparesis. Emergency cerebral digital subtraction angiography revealed occlusion of the left middle cerebral artery and the left anterior cerebral artery. Pulmonary arteriography showed three pulmonary arteriovenous malformations (AVMs). A 62-year-old male presented with consciousness disturbance and sensory aphasia. magnetic resonance imaging revealed a ring-enhanced lesion in the left temporal lobe which was removed by left frontotemporal craniotomy. The diagnosis was brain abscess. Chest computed tomography (CT) revealed two pulmonary AVMs. A 32-year-old female presented with progressive mild weakness in her left hand. Initial CT showed subcortical hemorrhage in the right frontal lobe. cerebral angiography revealed no vascular malformations, but chest CT disclosed five pulmonary AVMs. All three patients had a family history of HHT. The possibility of HHT is important to consider in patients with cerebrovascular disease (CVD) or brain abscess to prevent complications, not only in the patients but also their blood relatives. Therefore, the medical and family history of patients with CVD or brain abscess should be investigated and HHT should be considered during the physical examination.- - - - - - - - - - ranking = 2.2521002208129keywords = brain, central nervous system, nervous system (Clic here for more details about this article) |
9/37. Unilateral facial telangiectases suggest type 1 segmental manifestation of Osler-Rendu-Weber syndrome in an 11-year-old boy.An 11-year-old boy revealed multiple telangiectases confined to the left cheek and the left side of the lips. Additionally, unilateral epistaxis was present. Radiological examinations of the brain, lungs and abdomen were normal and bleeding of the gastrointestinal tract was excluded. In this case, we propose a probable segmental type 1 manifestation of the autosomal dominantly inherited Osler-Weber-Rendu syndrome.- - - - - - - - - - ranking = 0.5keywords = brain (Clic here for more details about this article) |
10/37. Isolated pulmonary arteriovenous fistula without Rendu-Osler-Weber disease as a cause of cryptogenic stroke.There has been uncertainty as to whether a right to left shunt through an isolated pulmonary arteriovenous fistula (P-AVF) without Rendu-Osler-Weber (ROW) disease can cause paradoxical brain embolism. A population of 747 acute ischaemic stroke patients was examined to determine the frequency and clinical characteristics of those patients who had an isolated P-AVF. The presence of a P-AVF was determined as follows. On patients with a stroke of undetermined cause, both transoesophageal echocardiography and transcranial Doppler with saline contrast medium was performed to detect a right to left shunt. If a P-AVF was then suspected, selective pulmonary angiography and enhanced chest CT was performed to confirm the presence of the P-AVF. Four patients (0.5%) were diagnosed as having a stroke associated with an isolated P-AVF. All the patients were middle-aged women (mean age 61 years). In all these patients, the P-AVF could not have been suspected on physical findings or chest x ray. The P-AVF was always single and located in the lower lobe. All the patients had asymptomatic deep venous thrombosis, and three patients developed pulmonary embolism. As D-dimer and thrombin-antithrombin complex were elevated in all patients, this indicated an activation of both fibrinolytic and thrombin activity. Our results show that an isolated P-AVF without ROW disease can cause paradoxical brain embolism. Thus, the existence of an isolated P-AVF as a right to left shunt in patients with a stroke of unknown origin should not be overlooked, even if a P-AVF is not suggested by the initial physical findings or chest x ray.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
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