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1/11. 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.
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ranking = 1
keywords = varicella
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2/11. cerebral infarction in a child. A case report.

    Cerebral infarcts in children are rather rare and in most cases no precise etiology is established. The authors describe a case of cryptogenetic cerebral infarction in a 9-year-old boy. The child presented an acute onset of hemiplegia in the right arm and leg, central facial palsy, dysarthria and steppage. The infarction was proved by Computed tomography (CT) and magnetic resonance imaging (MRI). Laboratory and instrumental studies rule out all known causes of brain infarction. The only possible etiopathogenetic hypothesis was a varicella arteritis which occurred 45 days before the clinical manifestation.
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ranking = 1
keywords = varicella
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3/11. Multifocal varicella-zoster virus vasculopathy without rash.

    A 51-year-old woman with crest syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) developed stepwise progressive focal neurological deficits without zoster rash. Multifocal ischemic infarcts were seen on magnetic resonance imaging, and cerebral angiography revealed focal stenosis of arteries affecting the intracranial circulation. A brain biopsy was nondiagnostic. Virological etiology of the disease was verified by the detection of varicella-zoster virus antibody in cerebrospinal fluid and by reduced serum-cerebrospinal fluid varicella-zoster virus IgG ratios (compared with normally high ratios of total IgG and albumin). Treatment with intravenous acyclovir stabilized but did not significantly improve her neurological deficits.
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ranking = 6
keywords = varicella
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4/11. Post-varicella arteriopathy: benefits of using serial transcranial Doppler examinations.

    A 2(8/12)-year old boy suffered from hemiplegia secondary to a post-varicella arteriopathy. His clinical status improved and after 4 years of follow-up he had no recurrent stroke or transient ischemic attack. Regular improvement of arterial lesions, demonstated by serial transcranial Doppler investigations, excluded a progressive arteriopathy. aspirin therapy was initiated and continued during 2,5 years. Transcranial Doppler is a portable, non-invasive tool that can be easily used on a regular basis to follow-up children with post-varicella arteriopathy.
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ranking = 6
keywords = varicella
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5/11. Varicella with delayed hemiplegia.

    We report 4 children who developed acute hemiplegia 7 weeks to 4 months after varicella infection. In 2 patients, carotid angiography demonstrated segmental narrowing and occlusion of the middle cerebral artery. Their clinical and angiographic features were similar to those associated with contralateral hemiplegia after herpes zoster ophthalmicus, the pathogenesis of which comprises cerebral angiitis due to varicella zoster viral infection. We believe that our patients had the same pathogenesis. In a survey of infectious diseases in our region, the frequency of varicella with delayed hemiparesis was roughly 1:6,500 varicella patients.
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ranking = 4
keywords = varicella
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6/11. Multiple ischemic infarcts in a child with AIDS, varicella zoster infection, and cerebral vasculitis.

    A 4 1/2-year-old girl with acquired immunodeficiency syndrome and prolonged varicella zoster virus skin infection developed multiple ischemic strokes and radiologic and histopathologic evidence of central nervous system vasculitis. Typical features of acquired immunodeficiency syndrome encephalitis were not present and there was no evidence of vasculitis outside the nervous system. central nervous system vasculitis probably resulted from varicella zoster virus infection that persisted because of immunodeficiency. This acquired immunodeficiency syndrome complication has only rarely been described in adults and to our knowledge has not been described in children.
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ranking = 6
keywords = varicella
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7/11. chickenpox with delayed contralateral hemiparesis caused by cerebral angiitis.

    chickenpox and herpes zoster ophthalmicus are caused by the same virus. herpes zoster ophthalmicus can be followed by contralateral hemiparesis, which is thought to be caused by spread of varicella-zoster virus to blood vessels contiguous to the trigeminal nerve and its branches. We report what we believe to be the first case of a patient with chickenpox followed by hemiparesis in whom there was angiographic evidence of an associated vasculitis similar to that found with herpes zoster ophthalmicus.
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ranking = 1
keywords = varicella
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8/11. Granulomatous angiitis of the brain with herpes zoster and varicella encephalitis.

    We studied a case of herpes zoster with varicella encephalitis in which a granulomatous necrotizing vasculitis was present in the optic nerve and brain. The vasculitis was observed even in foci devoid of inflammatory reaction in surrounding tissue, and was therefore interpreted as a primary vasculitis caused by varicella. To our knowledge, this is the first time a granulomatous vasculitis has been described as an integral part of varicella encephalitis, although others have made similar observations in the eye and even in viscera. These findings support the recent suggestion that so-called granulomatous angiitis of the CNS may be caused by the varicella virus.
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ranking = 8
keywords = varicella
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9/11. herpes zoster ophthalmicus and delayed contralateral hemiparesis caused by cerebral angiitis: diagnosis and management approaches.

    Four patients with herpes zoster ophthalmicus and delayed contralateral hemiparesis are described, and their findings are compared with those in patients previously reported in the English language literature. The current patients evidenced multifocal ipsilateral cerebral angiitis by angiography and multifocal infarcts in the distribution of the ipsilateral middle cerebral artery by computed tomographic scanning. cerebrospinal fluid showed mononuclear pleocytosis, positive oligoclonal bands, and an elevated immunoglobulin g index. Two patients were treated with corticosteroids and acyclovir, and 1 with corticosteroids alone, all without apparent response. Necrotizing angiitis ipsilateral to the herpes zoster ophthalmicus was demonstrated postmortem in 1 patient with multifocal cerebral infarction and progressive leukoencephalopathy. Neither herpes varicella zoster immunocytochemical reactivity nor viral inclusions were seen. The leukoencephalopathy associated with herpes varicella zoster either may be caused by cerebral angiitis or, as previously reported, may be a temporally remote manifestation of persistent herpes varicella zoster infection. The cerebral angiitis associated with herpes varicella zoster is histologically similar to granulomatous angiitis, and both may be related to herpes varicella zoster infection of the cerebral vasculature.
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ranking = 5
keywords = varicella
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10/11. Early and late onset manifestations of cerebral vasculitis related to varicella zoster.

    Varicella-zoster associated cerebral vasculitis (VZCV) as a cause of cerebral infarction has hitherto been considered a rare condition. Ischemic stroke in previously healthy children has occurred during recovery from chickenpox or has been attributed to virus reactivation among immunosuppressed patients. The clinical, radiologic and immunologic findings in four children with VZCV will be reported. Clinical manifestations included sudden onset of hemiparesis, motor aphasia and disturbed consciousness in previously healthy children. Only one child had a history of chickenpox six weeks prior to the onset of stroke, whereas a latency period of up to four years between chickenpox and the onset of stroke was found in the other three children. diagnosis of VZCV was confirmed repeatedly by demonstrating intrathecal production of varicella-zoster IgG antibodies in three children or a four-fold increase of varicella-zoster serum IgA-antibodies in one child. Intrathecal production of antibodies against other latent viruses and borreliosis could be excluded. PCR for varicella on CSF, performed in two patients, remained negative. No intrathecal production of varicella-zoster antibodies has been found in a control group of twenty clinically healthy children (age range from 2-18 years) with a previous varicella infection. During follow-up two children recovered completely whereas two children still suffer from serious neurological deficits. Immunological investigations, performed in three children, showed circulating immune-complexes with slightly lowered complement concentrations in two patients. In addition a lowered T-helper/T-suppressor cell ratio of unknown origin was found in three children. These immunological findings will be discussed in the light of the pathophysiology of VZCV.
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ranking = 9
keywords = varicella
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