1/249. Control of primary angiitis of the CNS associated with cerebral amyloid angiopathy by cyclophosphamide alone.Corticosteroids combined with cyclophosphamide are currently recommended for primary angiitis of the CNS. The authors report a 71-year-old man with primary angiitis of the CNS and amyloid angiopathy who responded to cyclophosphamide without steroids, suggesting that corticosteroids may not be needed in all cases of primary angiitis of the CNS.- - - - - - - - - - ranking = 1keywords = cerebral (Clic here for more details about this article) |
2/249. Pathologic findings in a steroid-responsive optic nerve infarct in giant-cell arteritis.OBJECTIVE: To investigate the pathophysiologic mechanism of optic nerve infarction in giant-cell arteritis (GCA). BACKGROUND: Previous pathologic reports of optic nerve infarction in GCA involved patients who were blind at the time of death. The optic nerve infarcts were primarily retrolaminar in localization. Simultaneous short ciliary and ophthalmic artery vasculitis was found in all patients. methods: Clinical neurologic and ophthalmologic examination, temporal artery biopsy, and neuroimaging tests were performed in a patient with an anterior ischemic optic neuropathy secondary to GCA. Pathologic examination of the viscera, eye, and brain were performed at autopsy 1 month later. RESULTS: A prelaminar/retrolaminar infarct was found in this patient. Subsiding vasculitis was limited to the short ciliary arteries, sparing the central retinal, pial, and ophthalmic arteries. CONCLUSIONS: The authors believe that the visual improvement observed in this patient was the result of preserved, anterior optic nerve collateral circulation, as well as the neuroprotective and anti-inflammatory effect of the corticosteroids.- - - - - - - - - - ranking = 0.0013081210351021keywords = brain (Clic here for more details about this article) |
3/249. Neurological manifestations of chronic hepatitis c.hepatitis c virus (HCV) infection is often associated with abnormal immunological responses. We describe four patients with vasculitic neurological signs and symptoms following HCV infection. A 56-year-old woman with HCV infection developed peripheral neuropathy characterized by asymmetric distal painful hypesthesia, dysesthesia and moderate motor weakness of the lower limbs. Serological examinations revealed cryoglobulinemia and low levels of complement c4. A biopsy of the sural nerve revealed vasculitic neuropathy. HCV infection associated immunomediated vasculitis was diagnosed. While steroid therapy was ineffective, treatment with interferon-alpha improved the neuropathy considerably without, however, eliminating HCV infection. A 62-year-old man with HCV infection developed peripheral sensory neuropathy. complement c3 was slightly diminished. Nerve biopsy revealed vasculitic neuropathy. A 71-year-old woman developed chronic symmetric sensomotor polyneuropathy. HCV hepatitis followed blood transfusions. cryoglobulins tested positive, consistent with type II cryoglobulinemia. Complements C3 and C4 were diminished. Inflammatory infiltrates in the sural nerve biopsy specimen led to the diagnosis of chronic vasculitic disorder. A 55-year-old woman with HCV infection developed vasculitis of the skin, connective tissue, visceral organs, and kidney, leading to hemodialysis. Neurologically she developed severe apathy and drowsiness, myoclonic jerks, exaggerated deep tendon reflexes, and positive pyramidal signs. magnetic resonance imaging of the brain showed diffuse increased signal abnormalities involving supra- and infratentorial white matter suggesting cerebral vasculitis. cryoglobulins were positive, complements C3 and C4 slightly diminished (54 mg/dl, 4.3 mg/dl). Supportive therapy resulted in neurological improvement. Treatment with interferon-alpha was discontinued because of agranulocytosis. In patients with peripheral neuropathy or signs of leucencephalopathy, a hepatitis c associated vasculitis should be considered in the differential diagnosis.- - - - - - - - - - ranking = 0.2513081210351keywords = cerebral, brain (Clic here for more details about this article) |
4/249. 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 = 0.5keywords = cerebral (Clic here for more details about this article) |
5/249. Multiple cerebral infarctions from nonbacterial thrombotic endocarditis mimicking cerebral vasculitis.Primary vasculitis of the central nervous system (PVCNS) is an uncommon disorder that can present with a variety of symptoms, making diagnosis and management difficult. We describe a case of cerebral infarction that occurred from nonbacterial thrombotic endocarditis (NBTE) and presented with clinical and radiologic imaging features that suggested PVCNS. The patient was a 58-year-old woman with left hemiparesis, aphasia, and episodic confusion. Magnetic resonance imaging of the brain demonstrated multifocal lesions consistent with infarction involving both cerebral hemispheres, and cerebral angiography showed changes consistent with vasculitis. Although brain biopsy findings were normal, the patient was treated for presumed vasculitis with cyclophosphamide and prednisone. Four months later respiratory failure secondary to polymicrobial pneumonia and adult respiratory distress syndrome developed, and she died. autopsy revealed multiple infarcts in the heart, lungs, right kidney, spleen, and brain. Multiple thrombotic platelet-fibrin vegetations consistent with NBTE were found on all cardiac valves. Examination of the brain revealed no evidence of active or healed vasculitis. cerebral angiography may show findings that suggest vasculitis, but it is not diagnostic, as several other conditions may cause similar changes. Nonbacterial thrombotic endocarditis may cause multiple cerebral infarctions and can be difficult to distinguish from vasculitis, as specific diagnostic tests for PVCNS are lacking.- - - - - - - - - - ranking = 3.0052324841404keywords = cerebral, brain (Clic here for more details about this article) |
6/249. systemic vasculitis and aneurysm formation in the wiskott-aldrich syndrome.A 24 year old male who suffered from the wiskott-aldrich syndrome developed intra-abdominal bleeding on two occasions. Radiological investigations showed aneurysmal dilatation of branches of the hepatic and superior mesenteric arteries. The second abdominal bleed necessitated laparotomy and the bleeding was localised to the kidneys. Right nephrectomy was performed and histological examination showed a necrotising vasculitis, mainly involving medium and small sized renal blood vessels. steroids, immunosuppressive treatment, and control of blood pressure resulted in resolution of the vasculitic process and prevented further haemorrhage. Vasculitis and aneurysm formation are rarely described complications of wiskott-aldrich syndrome and may account for the life threatening haemorrhage which occurs in this condition.- - - - - - - - - - ranking = 0.65144026491742keywords = haemorrhage (Clic here for more details about this article) |
7/249. Systemic mononuclear inflammatory vasculopathy associated with sjogren's syndrome in a patient with primary biliary cirrhosis.We report a 46-year-old woman with primary biliary cirrhosis (PBC) presenting with sjogren's syndrome and systemic mononuclear inflammatory vasculopathy. biopsy specimens of sural nerve showed findings consistent with vasculitic neuropathy. Perivascular inflammatory mononuclear cell infiltration was observed on muscle biopsy specimen. The findings of abdominal computed tomography and brain magnetic resonance imaging were suggestive of vasculitis. Clinical manifestations and radiologic findings were improved after high dose prednisolone therapy.- - - - - - - - - - ranking = 0.0013081210351021keywords = brain (Clic here for more details about this article) |
8/249. systemic vasculitis with bilateral perirenal haemorrhage in chronic myelomonocytic leukaemia.The cases of two patients with chronic myelomonocytic leukaemia associated with periarteritis nodosa-like, antineutrophil cytoplasmic antibody negative, systemic vasculitis, are reported. A 61 year old man was admitted with fever, diffuse myalgia, and abdominal pain. Blood and bone marrow examination showed chronic myelomonocytic leukaemia. Vasculitis of the gall bladder was responsible for acalculous cholecystitis. A massive spontaneous bilateral perirenal haemorrhage occurred. A 73 year old woman with chronic myelomonocytic leukaemia had been followed up for one year when unexplained fever occurred. Two months after the onset of fever, sudden abdominal pain was ascribed to spontaneous bilateral renal haematoma related to bilateral renal arterial aneurysms. Neuromuscular biopsy showed non-necrotising periarteriolar inflammation. To our knowledge, systemic vasculitis has never been reported in chronic myelomonocytic leukaemia. In our two cases a non-random association is suggested because (a) chronic myelomonocytic leukaemia is a rare myelodysplastic syndrome, (b) spontaneous bilateral perirenal haematoma is not a usual feature of periarteritis nodosa.- - - - - - - - - - ranking = 1.6292251228181keywords = haemorrhage, haematoma (Clic here for more details about this article) |
9/249. Giant cell angiitis of the central nervous system with amyloid angiopathy. A case report and review of the literature.We report a new case of giant cell angiitis of the central nervous system associated with cerebral amyloid angiopathy (GA/CAA). A 67-year-old woman was hospitalized with a history of headaches and lapses of consciousness. After improvement with corticosteroidtherpay, treatment was stopped. She relapsed and died 33 days after first admission. Pathological examination showed unusual extension of GA/CAA lesions, in the superficial and deep layer of the cerebral cortex, and in the cerebellum. Simultaneous occurrence of GA and CAA is rare. Histopathologic findings and immunological pathogenesis of the process are discussed: 1) arguments over pre-existence of CAA, responsible for GA; 2) primitive inflammatory process inducing amyloid deposits; 3) GA/CAA may represent an association of histological lesions related to 2 different types of disease: i) neurodegenerative disease with specific lesions (such as presence of diffuse senile plaques and neurofibrillary tangles) inducing inflammatory reaction ii) inflammatory disease, with few or no degenerative lesions, responding to immunotherapy.- - - - - - - - - - ranking = 0.5keywords = cerebral (Clic here for more details about this article) |
10/249. Cerebral thrombosis and vasculitis: an uncommon complication of ulcerative colitis.Cerebral thrombotic disease is a rare and nearly always fatal complication of ulcerative colitis. It is associated with a necrotizing vasculitis. We report a fatal case with a confusing neurologic picture arising from this complication. autopsy revealed necrosis and hemorrhages affecting both cortical grey and white matter. Microscopic examination showed thrombosis of small and medium size vessels associated with hemorrhages and a necrotizing angiitis. Ulcerations, hemorrhages, pseudopolyps, and cryptic abscesses were found in the rectosigmoid region of the colon compatible with active ulcerative colitis. A sudden neurologic deficit in a patient with ulcerative colitis should direct attention to the consideration of a cerebral thrombotic event and the possibility of an associated cerebral vasculitis. diagnosis may be strongly suggested by MRI or arteriography, but it may require confirmation by biopsy of the brain parenchyma and leptomeninges. A hypercoagulable state has been associated with the thrombosis. Anticoagulation has yielded successful results in some patients with cerebral thrombosis but the risk of massive intracranial and gastrointestinal bleeding preclude to establish clear indications. Neurologic improvement has been obtained with the use of steroids and cyclophosphamide.- - - - - - - - - - ranking = 0.7513081210351keywords = cerebral, brain (Clic here for more details about this article) |
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