1/19. 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.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/19. 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.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/19. 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.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
4/19. Postvaricella angiopathy: report of a case with pathologic correlation.Varicella is a common childhood illness, and central nervous system complications occur frequently. Delayed angiopathy has been described, although there are few reports of clinicopathologic correlation. A previously well 4-year-old male is presented. He suffered varicella 2 months before presentation with extensive right middle cerebral artery (MCA) territory infarction. cerebral angiography demonstrated an isolated 89% stenosis of the right proximal MCA. He developed cerebral edema refractory to medical treatment and progressed to transtentorial herniation. Right frontal temporoparietal craniotomies were performed with evacuation of infarcted brain tissue. Pathologic studies revealed small vessel vasculitis with lymphocytic infiltration of the vessel wall. Areas of demyelination were present within the white matter. polymerase chain reaction for varicella was negative on brain tissue. Postvaricella angiopathy, although an uncommon complication, may affect both small and large blood vessels, with catastrophic results.- - - - - - - - - - ranking = 5.1377347944202keywords = blood vessel, vessel (Clic here for more details about this article) |
5/19. 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.- - - - - - - - - - ranking = 3keywords = vessel (Clic here for more details about this article) |
6/19. Granulomatous angiitis of the central nervous system associated with herpes zoster.Granulomatous angiitis of central nervous system (CNS) is a rare inflammatory disease of blood vessels mostly confined to CNS. We describe a case which presented with right sided hemiplegia with aphasia, after herpes zoster ophthalmicus. CT scan and MRI brain showed a large left sided infarct in the left middle cerebral artery (MCA) territory. MRI angiography revealed narrowing and thinning of left internal carotid artery (ICA) and to a lesser extent, left MCA suggestive of granulomatous vasculitis. Herpes zoster is often associated with major CNS involvement and a vascular etiology was previously postulated. Recent pathological reports suggest that cerebral angiitis secondary to herpes virus infection may be more common than realised.- - - - - - - - - - ranking = 3.1377347944202keywords = blood vessel, vessel (Clic here for more details about this article) |
7/19. Follow-up examinations by transcranial Doppler ultrasound in primary angiitis of the central nervous system.BACKGROUND: Primary angiitis of the central nervous system (PACNS) is a rare disease. The definite diagnosis is made upon proof of mononuclear inflammation of the vessel wall on brain biopsy. The diagnosis can also be established on clinical grounds, typical findings on intra-arterial angiography and other investigatory grounds excluding other diseases. Therapy comprises an aggressive immunosuppressive approach. Close monitoring of the patients is mandatory. Transcranial Doppler ultrasound (TCD) has not yet been used to follow up the vasculitic lesions in PACNS. CASE: We report on a 32-year-old female with massive cerebral infarctions secondary to multiple large-vessel stenoses because of probable PACNS. The patient was followed closely by means of TCD. During therapy the cerebral blood flow velocities normalized as displayed by TCD. Clinical improvement followed several days after normalization of cerebral blood flow. CONCLUSIONS: TCD is a valuable noninvasive bedside tool to monitor cerebral blood flow velocities and therapy response in patients with cerebral vasculitis, if large arteries are involved.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
8/19. Varicella-zoster vasculitis presenting with intracranial hemorrhage.Cerebral vasculitis presenting with intracranial hemorrhage is a rare but known entity. We discuss here the case of a 61-year-old woman presenting with subarachnoid hemorrhage. cerebral angiography showed vasculitic changes involving the small and medium-sized vessels. She also had a concomitant herpes zoster rash on her back. A 3-month follow-up angiogram revealed partial resolution of the vasculitic changes.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/19. Leptomeningeal enhancement and venous abnormalities in granulomatous angiitis of the central nervous system.A 68-year-old woman with a relatively acute onset of right homonymous hemianopia, gerstmann syndrome, and global cognitive failure was found to have a lymphocytic pleocytosis and elevated protein on spinal fluid examination and displayed marked meningeal enhancement on magnetic resonance imaging and dilated cortical venules on cerebral angiography. brain and meningeal biopsy disclosed a necrotizing granulomatous inflammation of small and medium-sized subarachnoid vessels. The brain parenchyma was normal. The angiographic presence of venous abnormalities, the lack of observable angiographic arterial involvement, and the lack of parenchymal pathology are distinctly unusual in granulomatous angiitis of the central nervous system. This case, therefore, extends the pathologic and imaging spectrum of this disorder.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/19. Cerebral vasculitis in a patient with HCV-related type II mixed cryoglobulinemia.The peripheral nervous system is often involved in patients with mixed cryoglobulinemia (MC), while there are few reports of central nervous system involvement. We describe a case of HCV-related type II MC with peripheral and central nervous system involvement. A 61-year-old woman, suffering from flaccid tetraparesis, was referred to our department because of an increasing disability. The presence of delirium prompted us to also investigate the central nervous system. MMSE, EEG, EMG, brain CT-scan, color-Doppler of neck vessels, retinal fluorangiography and brain MRI were performed. These investigations suggested a cerebral vasculitis. The finding of very low C4 serum levels, together with high rheumatoid factor serum levels, suggested the search for cryoglobulins. The laboratory findings showed a HCV-related type II (IgMk) MC. A marked improvement of symptoms and of laboratory data was obtained by treatment with methylprednisolone cyclophosphamide.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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