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1/63. multiple sclerosis with extensive lesions left hemiplegia, mental dysfunction and retrobulbar neuritis.

    multiple sclerosis (MS) is usually diagnosed on the basis of the typical clinical course, with remission and exacerbation in multiple parts of the central nervous system (CNS). Recently, magnetic resonance imaging (MRI) has made a large contribution to the diagnosis of patients with MS. But it is difficult to make a definite diagnosis due to clinical variability of the disease and variable MRI findings. We report the case of a 26-year-old woman who developed progressive left hemiplegia, mental dysfunction, and had extensive bilateral cerebral white matter lesions diagnosed by brain MRI. Complete recovery from the left hemiplegia followed the use of corticosteroid, and her brain MRI findings also improved after medication. Early in the clinical course it was difficult to differentiate between multiple sclerosis and granulomatous angiitis of the CNS. After recovery from the first episode of left hemiplegia and mental dysfunction, she developed acute visual disturbance five months after her first admission. Readmission followed and her retrobulbar neuritis was successfully treated by methylpredonisolone pulse therapy. In summary, she experienced two episodes of neurological deficit, had left hemiplegia and mental dysfunction associated with multiple lesions in bilateral cerebral white matter in brain MRI, and the left retrobulbar neuritis with delay of P100 by visual evoked potential study. Due to these two episodes we concluded that she had multiple sclerosis. Though the initial diagnosis was difficult, prompt treatment and close follow-up was important, since multiple sclerosis and granulomatous angiitis of the CNS can both recur.
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keywords = brain
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2/63. 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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ranking = 313.89357316558
keywords = central nervous system, nervous system
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3/63. 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 = 392.36696645698
keywords = central nervous system, nervous system
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4/63. Cerebral vasculitis in a case of Sydenham's chorea.

    A 10-year-old girl with acute-onset hemichorea had multiple areas of abnormal signal seen on magnetic resonance imaging of the brain, associated with middle and anterior cerebral artery vasculitis seen on cerebral angiography. Her serology and clinical course were supportive of the diagnosis of Sydenham's chorea. Other causes of cerebral vasculitis were excluded. follow-up studies revealed resolution of changes seen on magnetic resonance imaging and partial resolution of angiographic abnormalities. This is the first report of abnormal cerebral angiography in Sydenham's chorea.
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keywords = brain
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5/63. 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 = 627.78714633116
keywords = central nervous system, nervous system
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6/63. 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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ranking = 78.473393291395
keywords = central nervous system, nervous system
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7/63. Disseminated mucormycosis caused by absidia corymbifera leading to cerebral vasculitis.

    An 18-year-old woman was admitted to hospital because of subcutaneous hematoma and fever of unknown origin. Acute myeloid leukemia was diagnosed and empirical antimicrobial treatment and induction chemotherapy were started. After initial defervescence, fever relapsed 2 days after the onset of neutropenia. The CT scan of the lung was consistent with an invasive fungal infection. Treatment with amphotericin b was started and antimicrobial treatment was continued with liposomal amphotericin b because of an increase in creatinine later. The fever persisted and the patient suddenly developed progressive neurological symptoms. CT scan of the head suggested cerebral infarction and angiography of the extra- and intracranial arteries showed signs of vasculitis. Six days after the onset of neurological symptoms cerebral death was diagnosed. autopsy revealed non-septate, irregularly branched hyphae in various histologic sections including brain. absidia corymbifera could be isolated from lung tissue confirming the diagnosis of disseminated mucormycosis. In this case, angiographic findings suggested severe cerebral vasculitis which was in fact caused by thromboembolic dissemination of fungal hyphae. This case underlines the fact that cerebral symptoms in febrile neutropenic patients are highly indicative for fungal infections of the brain.
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keywords = brain
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8/63. 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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ranking = 313.89357316558
keywords = central nervous system, nervous system
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9/63. 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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ranking = 78.473393291395
keywords = central nervous system, nervous system
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10/63. SPECT in focal enterovirus encephalitis: evidence for local cerebral vasculitis.

    We report a 4-year-old, left-handed male with focal coxsackievirus A3 encephalitis who presented with seizures and acquired aphasia. electroencephalography exhibited focal spike discharges over the right frontal regions, but cranial magnetic resonance imaging did not reveal any structural abnormalities. However, brain single-photon emission computed tomography performed during the acute phase disclosed focal hypoperfusion in the right frontal lobe, consistent with decreased regional cerebral blood flow in the territory of some branches of the right cerebral anterior artery. Without specific treatment, the patient recovered completely within 1 month, when brain single-photon emission computed tomography images returned to normal and cranial magnetic resonance imaging still demonstrated no abnormalities. The present case suggests the possible role of transient local cerebral vasculitis in the pathogenesis of focal enterovirus encephalitis.
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keywords = brain
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