1/96. Spatz-Lindenberg disease: a rare cause of vascular dementia.BACKGROUND: Isolated cerebral thromboangiitis obliterans (Spatz-Lindenberg disease) is not well recognized as a cause of vascular dementia. CASE DESCRIPTION: A 58-year-old woman presented with dementia and pyramidal signs. neuroimaging showed multiple areas of white matter change. brain biopsy showed intimal thickening of the walls of leptomeningeal and intraparenchymal arteries, almost to complete occlusion, with an intact internal elastic lamina and media and without inflammation or infiltration. The cortex showed only moderate gliosis. CONCLUSIONS: Spatz-Lindenberg disease should be considered in the differential diagnosis of vascular dementia. Additional studies of its pathogenesis are required to determine appropriate treatment.- - - - - - - - - - ranking = 1keywords = angiitis (Clic here for more details about this article) |
2/96. Angiitis of the central nervous system after allogeneic bone marrow transplantation?BACKGROUND AND PURPOSE: There is only limited information about late neurological complications after bone marrow transplantation (BMT). The purpose of this study is to describe a cerebral angiitis-like syndrome after allogeneic BMT. methods: Clinical and diagnostic findings of 5 BMT patients with chronic graft versus host disease and neuropathological data of 1 patient were reported. RESULTS: In the described patients, focal neurological signs and neuropsychological abnormalities occurred years after BMT. MRI revealed periventricular white matter lesions, lacunar or territorial infarctions, leukoencephalopathy, and hemorrhages. Angiitis of the central nervous system was confirmed in 1 patient at autopsy, and an angiitis-like syndrome was suspected in the other patients because of the clinical course and response to treatment. Three patients received cyclophosphamide and steroids (2 improved, 1 died), 1 patient improved after steroids alone, and 1 patient without immunosuppressive therapy deteriorated further. CONCLUSIONS: We propose that an angiitis-like syndrome of the central nervous system can be a neurological manifestation of graft versus host disease, which should be considered a possible cause of cerebral ischemic episodes and pathological MRI scans in BMT patients with graft versus host disease.- - - - - - - - - - ranking = 3keywords = angiitis (Clic here for more details about this article) |
3/96. Cerebral vascular events associated with ulcerative colitis in children.Although peripheral vascular thrombic events are recognized as a serious extra-intestinal complication of inflammatory bowel disease, the occurrence of cerebral vascular events in association with acute exacerbations of this group of diseases is rare. In this article, relevant literature is reviewed and three children, 5, 12, and 13 years of age, who presented with clinical and magnetic resonance imaging evidence of an acute cerebrovascular event in association with an acute exacerbation of their inflammatory bowel disease are described. Except for the presence of anemia, hematologic and coagulation studies were unremarkable, and a search for evidence of a systemic vasculitis proved negative.- - - - - - - - - - ranking = 0.0087934722717095keywords = vasculitis (Clic here for more details about this article) |
4/96. central nervous system complications in two cases of juvenile onset dermatomyositis.central nervous system (CNS) complications are rarely reported in either juvenile or adult onset inflammatory myositides, such as dermatomyositis and polymyositis. We report two children, aged 4 and 10 yr respectively, with a diagnosis of juvenile dermatomyositis, both of whom subsequently developed clinical features of severe CNS involvement, possibly consistent with cerebral vasculopathy. One child died from apparent brainstem involvement; the other developed seizures, pseudoseizures and clinical depression which responded to aggressive immunosuppression. Although the vasculopathy or vasculitis underlying this disorder is known to have a systemic distribution, CNS involvement has rarely been reported and may be under-recognized.- - - - - - - - - - ranking = 0.0087934722717095keywords = vasculitis (Clic here for more details about this article) |
5/96. diffusion MRI findings in isolated intracranial angiitis.This paper reports an 8-year-old girl with proven primary (isolated) angiitis of the central nervous system. On diffusion MRI, multiple scattered lesions were noted in the internal capsulas, thalami, and in the left middle cerebellar pedincle. These were hyperintense on b=1000s/mm(2) (true diffusion) images. The apparent diffusion coefficient (ADC) values of these ranged between 0.40 and 0.52 x 10(-3)mm(2)/s, consistent with acute ischemic infarction (cytotoxic edema). The ADC values of a relatively old lesion in the left occipital region ranged between 1.65 and 1.82 x 10(-3)mm(2)/s, consistent with chronic infarction.- - - - - - - - - - ranking = 5keywords = angiitis (Clic here for more details about this article) |
6/96. Dissociation between attentional set shifting and habit learning: a longitudinal case study.We report a patient (ST) with predominant damage to the right neostriatum, caused by a rare cerebral angiitis. The testing procedure was focused on attentional set shifting (wisconsin Card Sorting Test; WCST) and habit learning (probabilistic classification learning; PCL). ST showed impairments in the WCST, digit span backward, alphabet span, and PCL procedures, whereas he exhibited spared IQ, short-term verbal memory, object recognition, episodic and semantic memory. After 1 month of steroid therapy, there was a significant improvement in the WCST, digit span backward and alphabet span tests, whereas PCL remained severely impaired. The three control patients with damage to the parietal lobe displayed normal learning rates in PCL. These results suggest that separate frontostriatal mechanisms exist for attentional set shifting and habit learning.- - - - - - - - - - ranking = 1keywords = angiitis (Clic here for more details about this article) |
7/96. Intracerebral hemorrhage and postpartum cerebral vasculopathy.INTRODUCTION: Intracerebral hemorrhage (ICH) associated with pregnancy commonly occurs in the postpartum period in the setting of preeclampsia/eclampsia. We describe the clinical course of two patients with ICH due to postpartum cerebral vasculopathy in the absence of toxemia. methods: We reviewed two cases with ICH and postpartum vasculopathy in our hospital (1996-2001) and compared them with seven similar case reports from the literature. RESULTS: Mean age of all patients is 28.7 /-5.6 years (mean /-S.D.). toxemia of pregnancy was absent in all cases. ICHs were cortical in eight and putaminal in one patient. Erythrocyte sedimentation rate was elevated in two. Two cases rehemorrhaged during the same admission. No cerebral infarctions were reported. All patients had diffuse vasculopathy on conventional catheter angiography, with no clinical manifestations or laboratory data supportive of extracerebral or systemic vasculitis. Eight patients were treated with corticosteroids, two with additional cytotoxic agents and one with nimodipine alone. Improvement on follow-up cerebral angiography (catheter or MRA) and transcranial Doppler ultrasonography (TCD) was noted in eight cases. One did not have follow-up cerebral imaging but had an excellent clinical outcome. All cases had good to excellent functional recovery. CONCLUSIONS: Postpartum ICH in the absence of toxemia may be associated with isolated cerebral vasculopathy. The clinical course and functional outcome is good to excellent. This entity appears to be distinct from cerebral vasculitis, which is usually associated with poor outcome.- - - - - - - - - - ranking = 0.017586944543419keywords = vasculitis (Clic here for more details about this article) |
8/96. wegener granulomatosis with aphasia.wegener granulomatosis, a necrotizing granulomatous vasculitis that characteristically involves the respiratory tract and the kidneys, may affect the nervous system. Despite the relative frequency of neurologic manifestations, there has not been a single report of wegener granulomatosis manifesting as a cerebral vascular accident. Our patient had limited wegener granulomatosis with aphasia as the symptom that was observed first. A dramatic recovery occurred after corticosteriod and cytotoxic therapy.- - - - - - - - - - ranking = 0.0087934722717095keywords = vasculitis (Clic here for more details about this article) |
9/96. CNS angiitis in graft vs host disease.Graft-vs-host disease (GVHD) is a potentially treatable cause of progressive neurologic decline after bone marrow transplantation (BMT). The authors present histologic confirmation of CNS granulomatous angiitis in a child with chronic GVHD after BMT. Since cranial MRI showed only nonspecific findings, CNS vasculitis associated with GVHD after BMT may be underdiagnosed.- - - - - - - - - - ranking = 7.8148903086996keywords = granulomatous angiitis, angiitis, vasculitis (Clic here for more details about this article) |
10/96. Cerebral vasculitis in a patient with hereditary complete C4 deficiency and systemic lupus erythematosus.We describe the case of a female patient with hereditary complete C4 deficiency and systemic lupus erythematosus. She had suffered from lupus nephritis in early childhood. At the age of 23 years she developed severe lupus with skin disease and life-threatening cerebral vasculitis. Her cerebral disease was unresponsive to high-dose steroids, intravenous immunoglobulin, fresh frozen plasma and plasma exchange. Improvement was achieved with immunoadsorption in combination with mycophenolate mofetil. The patient made a complete recovery and is maintained in complete remission on mycophenolate and low-dose steroids.- - - - - - - - - - ranking = 0.043967361358547keywords = vasculitis (Clic here for more details about this article) |
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