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1/31. 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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ranking = 1
keywords = multiple sclerosis, sclerosis
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2/31. 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 = 0.00065433990124255
keywords = ms
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3/31. 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 = 0.00065433990124255
keywords = ms
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4/31. 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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ranking = 0.0019630197037277
keywords = ms
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5/31. Isolated angiitis of the CNS in children.

    OBJECTIVE: To clarify the clinical features and pathologic manifestations of isolated angiitis of the CNS (IACNS) in children. methods: The authors report two new cases and summarize the literature of childhood IACNS confirmed by pathology. RESULTS: IACNS affecting small vessels (n = 5). neurologic manifestations included headaches, focal seizures, and progressive, behavioral, or multifocal neurologic impairment. MRI showed multifocal, T2-hyperintense, cerebral lesions without mass effect or tumor-like lesions. CSF, erythrocyte sedimentation rate, and cerebral angiograms were often normal.CNS biopsy disclosed a nongranulomatous vasculitis. Children were treated with prednisone alone or combined with cyclophosphamide. One child died. Four children had a favorable outcome. IACNS affecting large and medium arteries (n =5). Three children presented with acute ischemic stroke or TIA. brain CT showed ischemic infarcts. Two children presented with subarachnoid hemorrhage. In this group, CSF, erythrocyte sedimentation rate, and angiograms were often abnormal. No patient received immunosuppressive therapy. Five children died. autopsy showed granulomatous IACNS (n =5). CONCLUSIONS: Clinical and radiologic features correlate with the size of affected vessels. prognosis differs between groups. Potential markers of poor outcome are acute stroke presentation secondary to large and medium-sized artery involvement, granulomatous angiitis, and delayed institution of immunosuppressive therapy.
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ranking = 0.0013086798024851
keywords = ms
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6/31. basilar artery vasculitis secondary to sphenoid sinusitis--case report.

    A 35-year-old male presented with basilar artery vasculitis secondary to sphenoid sinusitis manifesting as rapidly deteriorating symptoms including consciousness disturbance and right hemiparesis. Computed tomography (CT) on admission showed sphenoid sinusitis without intracranial lesion. Emergency angiography demonstrated basilar artery stenosis. The neurological deterioration was considered to be caused by ischemia of the perforating arteries branching from the stenotic portion of the basilar artery. The patient was treated with urokinase infusion through a microcatheter just proximal to the stenosis 3 hours after the onset of the symptoms. His consciousness level and right hemiparesis markedly improved immediately after the procedure. Magnetic resonance (MR) imaging on day 5 revealed that extension of the sphenoid sinusitis into the prepontine cistern had formed an abscess which was attached to the clivus. The basilar artery was embedded in the abscess at the angiographic stenosis. cerebrospinal fluid (CSF) analysis showed white blood cell count of 601/mm3 with 82% neutrophils, 89.2 mg/dl protein, and 31 mg/dl glucose. No causative organism in the CSF could be identified by smear or culture. Early MR imaging and CSF examination are recommended when patients present with both ischemic symptoms involving the basilar artery and opacification of the sphenoid sinus on CT to identify basilar artery vasculitis secondary to sphenoid sinusitis.
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ranking = 0.0019630197037277
keywords = ms
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7/31. Tumor-like multiple sclerosis (MS) lesions: neuropathological clues.

    The neuroradiological evidence of a single, large white matter lesion with mass effect, clinically revealed by signs of endocranial hypertension, is highly suspicious for central nervous system neoplasm. In rare cases, a demyelinating disorder can start with atypical features suggestive of a brain tumor; in these cases a brain biopsy is often carried out. We report our experience regarding cases of multiple sclerosis (MS) with atypical tumor-like presentation. None of our patients underwent biopsy. Serial magnetic resonance imaging performed during steroid treatment, together with other paraclinical data, were sufficient for the final diagnosis of MS. These cases are characterized by a severe clinical course and a rapid clinical deterioration, only partially modified by medical treatments. Atypical severe cases, misdiagnosed as MS, can be indeed due to primary CNS vasculitis.
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ranking = 1.4124441238537
keywords = multiple sclerosis, sclerosis
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8/31. Recurrent stroke as a manifestation of primary angiitis of the central nervous system in a patient infected with human immunodeficiency virus.

    CONTEXT: Cerebral vasculitis in patients infected with human immunodeficiency virus (hiv) is usually related to additional or secondary infectious agents other than neoplastic diseases or hiv itself. OBJECTIVE: To describe a 31-year-old patient infected with hiv who presented with 2 recurrent, acute episodes of neurologic impairment in a 5-month period. DESIGN: Comparison of clinical and histologic data between the present case and previously published cases. SETTING: Community hospital. PATIENT: A 31-year-old, hiv-infected patient with recurrent strokes and chronic lymphocytic meningitis. INTERVENTION: After ruling out cardiac embolisms and coagulation disorders, the presence of central nervous system vasculitis, probably secondary to an infectious process, was suspected based on the clinical examination and cerebrospinal fluid abnormalities. RESULTS: Necropsy findings suggest the diagnosis of primary angiitis of the central nervous system, and the only infectious agent that could be found was hiv. CONCLUSIONS: Histologic studies were compatible with a diagnosis of primary angiitis of the central nervous system, but the pathogenic role of hiv in the genesis of the vasculitic process cannot be elucidated.
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ranking = 0.00065433990124255
keywords = ms
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9/31. Meningoencephalomyelitis with vasculitis due to varicella zoster virus: a case report and review of the literature.

    Varicella zoster virus (VZV) encephalitis is associated with large or small vessel vasculopathy. We report the case of a 67-year-old woman with a history of non-Hodgkin's lymphoma and cancers of the breast and colon, who presented with a zosteriform rash and brown-sequard syndrome. Despite 10 days therapy with intravenous acyclovir, meningoencephalitis developed and the patient died 15 days after onset of neurological symptoms. autopsy showed meningoencephalomyelitis with necrotising vasculitis of leptomeningeal vessels, which is a rare complication of VZV, and we review the literature of the nine similar published cases. polymerase chain reaction of cerebrospinal fluid for VZV was negative 6 days after onset of neurological symptoms, but became positive by day 10. Only one multinucleated giant cell with intranuclear Cowdry type A inclusions was seen within an endothelial cell in a leptomeningeal vessel involved by vasculitis.
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ranking = 0.0013086798024851
keywords = ms
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10/31. Homonymous hemianopia following a triplet pregnancy: post-natal cerebral vasculitis or atypical eclampsia.

    A 46-year-old woman with a triplet pregnancy developed seizures, an oculogyric crisis and a homonymous hemianopia post-natally. Abnormal neuroimaging studies and lumbar puncture suggested possible cerebral vasculitis. Treatment with aciclovir and prednisolone resulted in a slow resolution of symptoms. This case highlights the difficulty in distinguishing eclampsia from rarer neurological causes of peripartum seizures.
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ranking = 0.00065433990124255
keywords = ms
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