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1/48. Isolated central nervous system vasculitis associated with hepatitis c infection.

    Since its identification in 1989, hepatitis c has been implicated in the pathogenesis of an increasing number of diseases previously believed to be primary or idiopathic. We report 2 rarely seen cases of isolated central nervous system (CNS) vasculitis in patients with hepatitis c infection. Patient 1. A 43-year-old man with 4 day right temporal headache developed a left hemiparesis. Weakness was his only physical finding. Computed tomography (CT) scan demonstrated a large right frontotemporal hemorrhage, and angiography revealed focal dilatations and irregularities of multiple branches of the right middle and anterior cerebral arteries. Cerebral decompression was performed and leptomeningeal biopsies showed granulomatous angiitis. Laboratory results were normal except for elevated liver biochemical tests. Later testing for hepatitis c was positive. His neurological symptoms improved with corticosteroids and cyclophosphamide. Patient 2. A 39 yr old male developed 3 days of left sided weakness, slurred speech and difficulty swallowing fluids. Physical findings were limited to his weakness. magnetic resonance imaging demonstrated a right superior pontine subacute infarct with a small left internal capsule lacunar infarct. Angiography revealed multiple areas of focal narrowing with no areas of abrupt vessel cut off. Cerebral spinal fluid showed 71 PMN, 29 RBC, normal glucose, elevated protein (64 mg/dl), no oligoclonal bands, and low myelin basic protein. Other laboratory analyses were normal including liver biochemical tests. However, hepatitis c serology was positive and mixed cryoglobulins were detected. CNS vasculitis was diagnosed and nearly full recovery was achieved with corticosteroids, cyclophosphamide and warfarin.
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2/48. Circulating antineutrophil autoantibodies in a child with isolated central nervous system vasculitis.

    An 8-year old girl with history of twisted neck and painful swelling on the left side of the neck was found to have malfunction of glossopharyngeal and hypoglossal nerves on the left side. magnetic resonance angiography revealed a giant aneurysm of the internal carotid artery surrounded by a widespread inflammatory tumor. cerebral angiography disclosed a large, false aneurysm with almost complete compression of the internal carotid artery. Circulating antineutrophil cytoplasmic autoantibodies (titer 1:2560) and high levels of antibodies against antiproteinase 3 were detectable. This observation indicates that these autoantibodies may be a diagnostic tool in children in whom an undiagnosed central nervous system inflammatory disease is present.
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ranking = 1.2500138023777
keywords = nervous system, nerve
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3/48. Primary angiitis of the central nervous system as a first presentation in Hodgkin's disease: a case report and review of the literature.

    OBJECTIVE AND IMPORTANCE: Granulomatous angiitis of the central nervous system is a rare cause of neurological deterioration. It is often diagnosed posthumously, and a high index of suspicion is necessary to make the correct diagnosis on a timely basis. CLINICAL PRESENTATION: A 27-year-old woman presented to the emergency room with complaints of worsening headache, nausea, and vomiting for 10 days, which were preceded by swelling of her tongue. At the examination, she had mild ocular tenderness, but no cranial nerve abnormalities. Radiographic examination revealed a right temporal lobe area with edema, and mild contrast enhancement was noted on computed tomography and magnetic resonance imaging. A similar but smaller region was present in the left frontal lobe. INTERVENTION: Stereotactic biopsy of the left temporal lobe revealed granulomatous angiitis. Further workup revealed Hodgkin's disease in the mediastinum. dexamethasone as well as chemotherapy for Hodgkin's disease was initiated. The patient's symptoms resolved, and she returned to work with her disease in remission. CONCLUSION: Previous reports of central nervous system angiitis have shown an association with sjogren's syndrome, herpes zoster infection, human immunodeficiency virus, and Hodgkin's disease. A review of the literature revealed a total of 12 patients with central nervous system angiitis and Hodgkin's disease. As a group, these patients had very poor outcomes. However, of six patients who presented with central nervous system angiitis and concurrent Hodgkin's disease and who underwent aggressive treatment for both conditions, three had a full recovery, two had a partial recovery, and one died.
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ranking = 2.0000138023777
keywords = nervous system, nerve
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4/48. Choroidopathy of systemic lupus erythematosus.

    PURPOSE: To describe the ocular and systemic manifestations associated with systemic lupus erythematosus (SLE) choroidopathy. methods: Three new cases of choroidopathy in patients with active SLE were described. Twenty-five published cases of lupus choroidopathy were summarized. RESULTS: There have been 28 cases of lupus choroidopathy (47 involved eyes) that have been reported in the English literature since 1968, including the three current cases. Only two of the patients were male. The choroidopathy was bilateral in 19 patients (68%). All 28 patients (100%) had active systemic vascular disease at the onset of their choroidopathy; 18 (64%) had nephropathy and 10 (36%) had central nervous system (CNS) lupus vasculitis. All but one of the patients had a known diagnosis of SLE at the onset of choroidopathy. 30 of the 47 involved eyes had presenting visual acuity of 20/40 or better; 14 eyes showed improvement in visual acuity with therapy. 23 patients (82%) had resolution of their choroidopathy when their systemic disease was brought under control. Despite treatment, 4 of the 28 patients (14%) died from complications of SLE. CONCLUSIONS: Although less known than retinopathy, lupus choroidopathy may be more common than generally appreciated. It usually serves as a sensitive indicator of lupus activity. The presence of SLE choroidopathy is generally indicative of coexistent (although sometimes occult) nephropathy, CNS vasculitis, and other SLE visceral lesions. immunomodulation of the systemic disease can lead to improvement and resolution of the systemic vasculitis as well as the choroidopathy.
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5/48. A case of central nervous system vasculitis related to an episode of guillain-barre syndrome.

    The authors report their knowledge about an uncommon case of isolated vasculitis, restricted to the left sylvian artery during an auto-immune guillain-barre syndrome (GBS), sustained by cytomegalovirus (CMV). An acute cardiopulmonary failure requiring a ventilator and vasopressor support manifested, notwithstanding plasma exchanging and immune-modulating therapy. An IgM-enriched formula administration coincided with a rapid amelioration of GBS and vasculitis to a complete recovery the next month after her discharge to a rehabilitation centre.
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6/48. Acute hemiplegia associated with herpes zoster infection in children: report of one case.

    Herpes zoster infection has been rarely reported to cause angiitis of the central nervous system in children. We describe a 4-year, 8-month-old female with acute hemiplegia and central facial palsy 6 weeks after she had had zoster ophthalmicus. The findings of magnetic resonance angiography, the clinical picture, and a preceding history of herpes zoster ophthalmicus suggested zoster vasculitis. Herpes zoster vasculitis is thus another consideration when examining a child with acute hemiplegia and a recent herpes zoster infection.
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7/48. 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.
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ranking = 1.25
keywords = nervous system
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8/48. central nervous system and renal vasculitis associated with primary varicella infection in a child.

    A 7-year-old girl with primary varicella presented with encephalopathy and focal neurologic deficits 10 days after her first skin lesions appeared. She was discovered to have bilateral wedge-shaped renal infarctions, and ischemic lesions in the conus medullaris, cerebral cortex, and deep gray matter consistent with a medium and large vessel arteritis on magnetic resonance imaging. This complication has never before been reported in an immunocompetent child with primary varicella infection, and it represents a rare but serious complication of childhood chickenpox.
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9/48. central nervous system vasculitis as a complication of refractory dermatomyositis.

    We describe a 47-year-old woman with refractory dermatomyositis (DM) who developed progressive cognitive dysfunction. magnetic resonance imaging showed a large cerebral infarction, and the diagnosis of central nervous system (CNS) vasculitis was confirmed by both angiogram and brain biopsy. Her DM and CNS vasculitis responded promptly to the institution of daily cyclophosphamide, and her previously refractory disease entered remission.
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10/48. Primary angiitis of the central nervous system in children: 5 cases.

    We describe 5 children who meet criteria for primary angiitis of the central nervous system (PACNS). All patients presented with headache and/or focal neurologic deficits and exhibited clinical and/or radiographic evidence of disease progression. Two patients had disease progression prior to combined treatment with cyclophosphamide and corticosteroids; one progressed while receiving intravenous cyclophosphamide and stabilized after a change to daily oral dosing; one progressed after discontinuing therapy after less than 12 months and improved after retreatment; and one progressed on steroid therapy alone but was lost to followup. Children who have frequent or severe headaches or focal neurologic deficits should be carefully evaluated and those meeting criteria for PACNS should be treated aggressively.
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keywords = nervous system
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