11/249. Primary angiitis of CNS : neuropathological study of three autopsied cases with brief review of literature.Primary angiitis of CNS(PACNS) or granulomatous angiitis of CNS is a rare inflammatory disease of small blood vessels mostly confined to the CNS. The clinical and pathological features of 3 autopsied cases are described. Clinically all the three PACNS patients were young males, age ranging from 19 to 31 years. All presented with varied neurological manifestations. There was no evidence of systemic disease in any of the cases. The ESR was normal and CSF analysis showed chronic meningitic pattern. The cerebral angiogram in one case was normal and the CT scan done in another case showed multiple intracerebral haematoma due to vasculitis. Brain biopsy was not done. diagnosis was made at post-mortem examination. histology showed characteristic but variable degree of granulomatous and non-granulomatous angiitis of small vessels. Venulitis with parenchymal haemorrhages was the predominant feature and in one case phlebitis with thrombosis was noted. Since the disease responds to steroids and immunosuppressive therapy, establishing antemortem diagnosis is important. In view of the association of angiitis of CNS with bacteria and viral infections, their role in the evolution of the disease needs to be investigated.- - - - - - - - - - ranking = 1keywords = haemorrhage, cerebral, intracerebral, haematoma (Clic here for more details about this article) |
12/249. MRI in human immunodeficiency virus-associated cerebral vasculitis.Cerebral ischaemia caused by inflammatory vasculopathies has been described as complication of human immunodeficiency virus (hiv) infection. Imaging studies have shown ischaemic lesions and changes of the vascular lumen, but did not allow demonstration of abnormalities within the vessel wall itself. Two hiv-infected men presented with symptoms of a transient ischaemic attack. Initial MRI of the first showed no infarct; in the second two small lacunar lesions were detected. In both cases, multiplanar 3-mm slice contrast-enhanced T1-weighted images showed aneurysmal dilatation, with thickening and contrast enhancement of the wall of the internal carotid and middle cerebral (MCA) arteries. These findings were interpreted as indicating cerebral vasculitis. In the first patient the vasculopathy progressed to carotid artery occlusion, and he developed an infarct in the MCA territory, but then remained neurologically stable. In the second patient varicella zoster virus (VZV) infection was the probable cause of vasculitis. The clinical deficits and vasculitic MRI changes regressed with antiviral and immunosuppressive therapy.- - - - - - - - - - ranking = 1.7651466983084keywords = cerebral (Clic here for more details about this article) |
13/249. New imaging findings in a patient with central nervous system dysfunction after bone marrow transplantation.central nervous system disorders are an important complication of bone marrow transplantation (BMT). We have recently performed cerebral angiography to examine central nervous system dysfunction in a 22-year-old woman with acute lymphoblastic leukaemia who had undergone BMT. Angiography demonstrated multiple stenoses and occlusions in the peripheral branches of the anterior and middle cerebral arteries, a pattern similar to that seen in vasculitis. She was thought to most likely have cytomegalovirus (CMV) vasculitis, but other forms of vasculitis, such as angiitis-like-syndrome-associated graft-versus-host disease could not be excluded. This case suggests that CMV vasculitis may cause central nervous system dysfunction after BMT and that imaging studies may provide useful information about central nervous system disorders in these patients.- - - - - - - - - - ranking = 0.58838223276947keywords = cerebral (Clic here for more details about this article) |
14/249. Microscopic polyarteritis with renal and cerebral involvement.We report a 15-year-old girl who presented with renal failure requiring dialysis and a diagnosis of p-ANCA-associated vasculitis. She subsequently developed cerebral vasculitis during treatment with oral cyclophosphamide and prednisolone. Treatment with intravenous cyclophosphamide and plasma exchange produced a complete recovery.- - - - - - - - - - ranking = 1.4709555819237keywords = cerebral (Clic here for more details about this article) |
15/249. Primary angiitis of the central nervous system and Alzheimer's disease: clinically and pathologically evident in a single patient.Two years after being successfully treated for biopsy confirmed primary angiitis of the central nervous system (PACNS), a 69-year-old woman presented with cognitive decline. In contrast to her first presentation, her altered mental function developed gradually, was not associated with headache or abnormal cerebrospinal fluid analysis, and did not improve with immunosuppression. Reevaluation of her original brain biopsy not only confirmed the presence of PACNS, but also revealed neuritic plaques and neurofibrillary tangles, suggesting a concurrent diagnosis of Alzheimer's disease. Cerebral angiogram did not suggest vasculitis and magnetic resonance imaging showed generalized cerebral atrophy supporting the diagnosis of Alzheimer's. This case illustrates that Alzheimer's dementia and PACNS can coexist in a single patient and that Alzheimer's disease should be considered when a patient with successfully treated PACNS presents with cognitive decline months or years after initial diagnosis.- - - - - - - - - - ranking = 0.29573046673547keywords = cerebral, brain (Clic here for more details about this article) |
16/249. Lymphocytic vasculitis in X-linked lymphoproliferative disease.systemic vasculitis is an uncommon manifestation of X-linked lymphoproliferative disease (XLP), a disorder in which there is a selective immune deficiency to Epstein-Barr virus (EBV). The molecular basis for XLP has recently been ascribed to mutations within SLAM-associated protein (SAP), an SH2 domain-containing protein expressed primarily in T cells. The authors describe a patient who died as a result of chronic systemic vasculitis and fulfilled clinical criteria for the diagnosis of XLP. Sequencing of this patient's SAP gene uncovered a novel point mutation affecting the SH2 domain. The patient presented with virus-associated hemophagocytic syndrome (VAHS) and later had chorioretinitis, bronchiectasis, and hypogammaglobulinemia develop. He further developed mononeuritis and fatal respiratory failure. Evidence of widespread small and medium vessel vasculitis was noted at autopsy with involvement of retinal, cerebral, and coronary arteries as well as the segmental vessels of the kidneys, testes, and pancreas. Immunohistochemical analysis using antibodies to CD20, CD45RO, and CD8 revealed that the vessel wall infiltrates consisted primarily of CD8( ) T cells, implying a cytotoxic T-lymphocyte response to antigen. EBV dna was detected by polymerase chain reaction (PCR) in arterial wall tissue microdissected from infiltrated vessels further suggesting that the CD8( ) T cells were targeting EBV antigens within the endothelium. The authors propose that functional inactivation of the SAP protein can impair the immunologic response to EBV, resulting in systemic vasculitis.- - - - - - - - - - ranking = 0.29419111638474keywords = cerebral (Clic here for more details about this article) |
17/249. Systemic antineutrophil cytoplasmic antibody vasculitis associated with lymphoid neoplasia.Two cases of systemic antineutrophil cytoplasmic antibody (ANCA) vasculitis in the setting of chronic lymphocytic leukaemia and angioimmunoblastic lymphadenopathy type T cell lymphoma are reported. The two patients had fever of unknown origin associated with cutaneous vasculitis and "pulmonary-renal syndrome" with alveolar haemorrhage. Despite anti-infectious treatments, steroids, and chemotherapy, the vasculitis had a fatal paraneoplastic course in several weeks. When infection is excluded in patients with malignancy, atypical features should be promptly investigated for systemic vasculitis, and an ANCA test performed.- - - - - - - - - - ranking = 0.38329587758805keywords = haemorrhage (Clic here for more details about this article) |
18/249. spinal cord involvement in primary angiitis of the central nervous system: a report of two cases.SUMMARY: We report two patients with suspected primary angiitis of the CNS who underwent serial contrast-enhanced MR imaging of the spinal cord. MR abnormalities were multiple and enhancing, and located within the cervical and thoracic cord. Brain MR findings and brain biopsy specimens were positive for primary angiitis of the CNS. On follow-up MR studies, obtained after steroid and immunosuppressive therapy, a significant decrease in the number and size of the enhancing and nonenhancing abnormalities was observed, along with clinical improvement. Numerous small and enhancing abnormalities with a primarily posterior location, seen at the onset of the disease and resolved on follow-up studies, may be considered suggestive of a diagnosis of primary angiitis of the CNS.- - - - - - - - - - ranking = 0.0015393503507322keywords = brain (Clic here for more details about this article) |
19/249. Reversible posterior leucoencephalopathy syndrome in systemic lupus and vasculitis.OBJECTIVES: Reversible posterior leucoencephalopathy syndrome (RPLS) may develop in patients with renal insufficiency, hypertension, and immunosuppression, and is managed by prompt antihypertensive and anticonvulsant treatment. Four patients with renal insufficiency and fluid overload associated with Wegener's granulomatosis (one patient) and systemic lupus erythematosus (SLE) (three patients) are described, whose clinical picture and neuroimaging indicated RPLS. case reports: All patients had headache, seizures, visual abnormalities, and transient motor deficit, and were hypertensive at the onset of the symptoms. Head computed tomography (CT) scan and magnetic resonance imaging showed predominantly posterior signal abnormalities, which were more conspicuous on T(2) weighted spin echo images than on CT scan. All patients had some form of cytotoxic treatment shortly before the syndrome developed, and dramatically responded to blood pressure control and anticonvulsant treatment. In two patients with SLE, dialysis was required for renal insufficiency. DISCUSSION: Follow up neuroimaging studies showed almost complete resolution of signal abnormalities, and suggested that RPLS was associated with cerebral oedema without concomitant infarction. The treatment of hypertension and neurotoxic condition such as uraemia appears of primary importance, while immunosuppressive treatment may cause further damage of the blood-brain barrier.- - - - - - - - - - ranking = 0.29573046673547keywords = cerebral, brain (Clic here for more details about this article) |
20/249. Necrotizing angiitis of the small intestine related to AA-amyloidosis: a novel association.A 71-year-old man with intestinal pseudo-obstruction was found to have a diffusely thickened adynamic small bowel with AA-amyloid in submucosal vessels and muscularis propria, foreign body giant cell reaction to amyloid, and necrotizing angiitis. The mucosa was unremarkable. Immunostains demonstrated numerous CD68 monocyte/macrophages and CD8 T cells associated with the amyloid deposits. The patient had no evidence of systemic vasculitis and no underlying cause for AA-amyloidosis was identified. Necrotizing angiitis coexistent with amyloid angiopathy has been reported in brain and temporal arteries, but not in the gastrointestinal tract and not with AA-amyloid. The inflammatory cell infiltrates in this case are consistent with a foreign-body and/or cell-mediated immunologic reaction to AA-amyloid, although a role for these cells in amyloid formation cannot be excluded.- - - - - - - - - - ranking = 0.0015393503507322keywords = brain (Clic here for more details about this article) |
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