1/46. Systemic infection with Alaria americana (trematoda).Alaria americana is a trematode, the adult of which is found in mammalian carnivores. The first case of disseminated human infection by the mesocercarial stage of this worm occurred in a 24-year-old man. The infection possibly was acquired by the eating of inadequately cooked frogs, which are intermediate hosts of the worm. The diagnosis was made during life by lung biopsy and confirmed at autopsy. The mesocercariae were present in the stomach wall, lymph nodes, liver, myocardium, pancreas and surrounding adipose tissue, spleen, kidney, lungs, brain and spinal cord. There was no host reaction to the parasites. Granulomas were present in the stomach wall, lymph nodes and liver, but the worms were not identified in them. hypersensitivity vasculitis and a bleeding diathesis due to disseminated intravascular coagulation and a circulating anticoagulant caused his death 8 days after the onset of his illness.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/46. Systemic lupus erythematosus-associated catastrophic antiphospholipid syndrome occurring after typhoid fever: a possible role of Salmonella lipopolysaccharide in the occurrence of diffuse vasculopathy-coagulopathy.We report a case of well-documented typhoid fever in a 30-year-old woman with inactive systemic lupus erythematosus with asymptomatic lupus anticoagulant and high-titer anticardiolipin antibody (aCL). Despite prompt eradication of the salmonella typhi obtained with appropriate antibiotic therapy, multiple organ system dysfunction occurred. The central nervous system was involved, with ischemic infarcts in the occipital lobes. High-dose corticosteroid therapy failed to improve the neurologic manifestations, which responded to repeated plasmapheresis. A sharp fall in aCL and anti-beta2-glycoprotein I antibody titers was recorded before the start of plasmapheresis. At the same time, IgM and IgG antibodies to Salmonella group O:9 lipopolysaccharide became detectable; the IgM antibodies disappeared within 4 months, whereas the IgG antibodies remained detectable during the next 13 months. Despite treatment with high-dose corticosteroids and cyclophosphamide, rapidly progressive glomerulonephritis developed, leading to chronic renal failure. There is convincing evidence of a link between the S. typhi infection and the ensuing catastrophic syndrome in this patient, probably precipitated by bacterial antigens.- - - - - - - - - - ranking = 7.5554866913543keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/46. Disseminated intravascular meconium in a newborn with meconium peritonitis.A 3-day-old premature infant with meconium peritonitis, periventricular leukomalacia, and pulmonary hypertension died with respiratory insufficiency. An autopsy disclosed intravascular squamous cells in the lungs, brain, liver, pancreas, and kidneys. Numerous pulmonary capillaries and arterioles were occluded by squamous cells, accounting for pulmonary hypertension. Brain parenchyma surrounding occluded cerebral vessels showed infarct and gliosis. A mediastinal lymph node filled with squamous cells alluded to the mechanism by which these cells from the peritoneal cavity likely entered the bloodstream--namely, via diaphragmatic pores connecting with lymphatics. Thus, disseminated intravascular meconium rarely may complicate meconium peritonitis and have devastating consequences.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
4/46. Multifocal vasculopathy due to Varicella-Zoster Virus (VZV): serial analysis of VZV dna and intrathecal synthesis of VZV antibody in cerebrospinal fluid.Recognition of multifocal vasculopathy due to varicella-zoster virus (VZV) is often problematic. We describe a human immunodeficiency virus-infected patient who had progressive central nervous system disease for >3 months. Both VZV dna and antibody were detected in cerebrospinal fluid (CSF) specimens; serial polymerase chain reaction analyses confirmed the diagnosis and guided the duration of therapy. Reduced ratios of VZV antibody in serum to that in CSF were also demonstrated.- - - - - - - - - - ranking = 7.5554866913543keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/46. First-trimester features of Fowler syndrome (hydrocephaly-hydranencephaly proliferative vasculopathy).We describe the features of Fowler syndrome (proliferative vasculopathy and hydrocephaly-hydranencephaly) diagnosed in the first trimester. The pregnancy with no significant family history was referred for karyotyping and ultrasound examination after a cystic hygroma was seen at 12 weeks. At 13 weeks, ultrasound examination revealed hydrocephaly-hydranencephaly, fetal akinesia, and arthrogryposis associated with increased nuchal translucency. The parents opted for termination of pregnancy and the diagnosis of Fowler syndrome was confirmed by pathological examination of the fetus. Calcified necrotic lesions and proliferative vasculopathy were observed in the entire central nervous system including the brainstem and spinal cord. Cases previously reported in siblings suggest an autosomal recessive transmission but specific genetic antenatal diagnosis is not yet available. The diagnosis of proliferative vasculopathy and hydrocephaly-hydranencephaly (Fowler syndrome) should be considered whenever hydrocephaly-hydranencephaly associated with a fetal akinetic sequence are encountered at the end of the first trimester. genetic counseling is recommended.- - - - - - - - - - ranking = 8.5554866913543keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
6/46. Unusual intravascular material in the brain. autopsy findings in a patient treated with antihemophilic factor concentrates.Widespread vascular occlusion caused by unusual particulate material in the brain of a patient who was intensively treated with antihemophilic factor (AHF) concentrates prior to death is described. The intravascular particles were seen partially or completely occluding both veins and arteries of small caliber in the brain and also to a much lesser extent in other organs. The resultant small focal infarcts were predominantly distributed in the cerebral white matter. The pathogenesis of this apparently unique vascular occlusive phenomenon and its relationship, if any, to the massive AHF concentrate infusion in this patient remain unknown.- - - - - - - - - - ranking = 6keywords = brain (Clic here for more details about this article) |
7/46. Postpartum angiopathy with reversible posterior leukoencephalopathy.BACKGROUND: Postpartum angiopathy (PPA) is a cerebral vasoconstriction syndrome of uncertain cause that affects large and medium-sized cerebral arteries. Postpartum angiopathy is frequently complicated by ischemic stroke. The reversible posterior leukoencephalopathy syndrome (RPLS) is a distinct clinical-radiological entity characterized by transient vasogenic edema on brain imaging. The pathophysiological features of RPLS are related to small-vessel dysfunction and breakdown of the blood-brain barrier. OBJECTIVES: To report the coexistence of PPA and RPLS in 4 patients and to discuss possible interrelationships between these 2 entities. DESIGN: Four case reports and a review of the literature. RESULTS: Four women developed a clinical-radiological syndrome overlapping PPA and eclampsia shortly after an uncomplicated pregnancy. All had acute severe ("thunderclap") headaches and hypertension. Three developed seizures. All patients had reversible angiographic narrowing of large and medium-sized cerebral arteries. Serial magnetic resonance imaging showed transient nonischemic brain lesions, resembling the lesions described in patients with RPLS. The results of extensive tests for cerebral vasculitis were negative. CONCLUSION: These cases, and the literature, suggest an interrelationship between RPLS and cerebral vasoconstriction syndromes such as PPA.- - - - - - - - - - ranking = 3keywords = brain (Clic here for more details about this article) |
8/46. Ipsilateral beating nystagmus after acoustic schwannoma resection.OBJECTIVE: To highlight the alert value of an ipsilateral beating nystagmus to detect any potential vascular complication in the early postoperative course of acoustic schwannoma (AS) surgery. methods: In a series of 432 cases of AS operated by the same surgeons between January 1991 and December 2000, two cases of vascular complications were detected by an ipsilateral beating nystagmus toward the operated side. RESULTS: One case of arterial infarction giving rise to a partial AICA syndrome and one case of hematoma of the cerebello-pontine angle (CPA) were revealed by an ipsilateral beating nystagmus. It was the first or the only objective neurologic sign encountered in the early postoperative course of AS surgery. CONCLUSIONS: An ipsilateral beating nystagmus after AS resection, even appearing as the only abnormal objective neurologic finding, should always raise suspicion of a brain stem event. Early extubation is recommended for systematic neurologic assessment in order to detect as quickly as possible any potential vascular complication in the early postoperative course of AS.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
9/46. paraplegia and sensory deficit caused by angiotropic large cell lymphoma.We report a case of angiotropic large cell lymphoma (ALCL) with central system involvement in which there was initially an isolated spinal cord stroke with paraplegia. MR imaging of the spinal cord demonstrated increased signal intensity in the center of the cord on T2-weighted images and subsequently a cerebral lesion in the right temporal lobe. The diagnosis of ALCL was established by brain biopsy. An enlarged spinal cord with enhancement after administration of contrast material and increased signal intensity on T2-weighted images, while not specific for ALCL, may be the first imaging findings of that disease.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
10/46. costello syndrome showing moyamoya-like vasculopathy.This report describes a patient with costello syndrome associated with moyamoya-like vasculopathy. His clinical findings were sparse, thin, and light-colored hair, bilateral ptosis, low-set ears, depressed nasal bridge, bulbous nose, short neck, loose pigmented skin with deep palmar and plantar creases, bilateral cryptorchidism, and delays in growth and development. Brain magnetic resonance imaging and cerebral angiography revealed moyamoya-like vasculopathy. A skin biopsy from the extensor surface of the right thigh revealed shortening and rupture of elastic fibers. Electron microscopy indicated reduced depositions of elastin. Formation of a stable elastic fiber system may be impaired in patients with costello syndrome, and brain magnetic resonance imaging and magnetic resonance angiography would be recommended for these patients.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
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