Cases reported "Vasculitis"

Filter by keywords:



Filtering documents. Please wait...

11/158. sneddon syndrome with multiple cerebral infarctions 12 years after the onset of livedo vasculitis: a possible involvement of platelet activation.

    sneddon syndrome is characterized by livedo reticularis and multiple cerebral infarctions. Skin and central nervous system symptoms usually have a synchronous onset and at times initial symptoms affect one of them, the other lagging several years behind. We here report a patient with sneddon syndrome who developed multiple cerebral infarctions more than 10 years after the onset of livedo reticularis. While the neurological symptoms were apparent, the patient did not display active skin manifestations. Laboratory findings excluded collagen diseases, antiphospholipid antibody syndrome, and inherited quantitative deficiency of protein c, protein s and antithrombin iii. Abnormal findings included extremely elevated levels of beta-thromboglobulin and platelet factor-4 in the blood, although these acute phase markers of thrombosis were examined several years after the onset of cerebral infarctions. platelet activation may have caused sneddon syndrome in the present case.
- - - - - - - - - -
ranking = 1
keywords = nervous system
(Clic here for more details about this article)

12/158. diffusion MRI findings in isolated intracranial angiitis.

    This paper reports an 8-year-old girl with proven primary (isolated) angiitis of the central nervous system. On diffusion MRI, multiple scattered lesions were noted in the internal capsulas, thalami, and in the left middle cerebellar pedincle. These were hyperintense on b=1000s/mm(2) (true diffusion) images. The apparent diffusion coefficient (ADC) values of these ranged between 0.40 and 0.52 x 10(-3)mm(2)/s, consistent with acute ischemic infarction (cytotoxic edema). The ADC values of a relatively old lesion in the left occipital region ranged between 1.65 and 1.82 x 10(-3)mm(2)/s, consistent with chronic infarction.
- - - - - - - - - -
ranking = 1
keywords = nervous system
(Clic here for more details about this article)

13/158. churg-strauss syndrome presenting as spontaneous subarachnoid haemorrhage.

    churg-strauss syndrome (CSS) is a systemic small-vessel vasculitis characterised by the presence of asthma and eosinophilia. central nervous system involvement (cerebral infarctions or intracerebral haemorrhage) is rare in CSS. Spontaneous subarachnoid hemorrhage (SAH) has been described in other systemic vasculitides. SAH is exceptional in CSS. We present a 47-year-old woman with CSS presenting as a spontaneous SAH with cerebral angiography findings consistent with vasculitis of the basilar artery and without aneurysms or arteriovenous malformations. She received treatment with prednisone and cyclophosphamide, and 2 months later the basilar artery was normal on magnetic resonance angiography.
- - - - - - - - - -
ranking = 1
keywords = nervous system
(Clic here for more details about this article)

14/158. cadasil mimicking primary angiitis of the central nervous system.

    BACKGROUND: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (cadasil) and primary angiitis of the central nervous system (PACNS) share several clinical and radiological features. However, digital subtraction angiogram (DSA) is generally reported as normal in cadasil, whereas lumen irregularities in distal cerebral arteries indicate PACNS. OBJECTIVE: To describe a potential pitfall of DSA interpretation, which led to the tentative diagnosis of PACNS in a cadasil patient. PATIENT AND methods: Single case observation. RESULTS: A 47-year-old man sustained recurrent subcortical infarcts. He had mild hypercholesterolemia and migraine. His family history was unremarkable. The underlying cause of stroke could not be elucidated. Transcranial Doppler sonography revealed decreased intracranial blood flow velocities compatible with cadasil. Lumen irregularities of several peripheral intracranial arteries were seen on DSA, which suggested PACNS. cadasil was confirmed by results from skin biopsy and genetic testing. CONCLUSIONS: First, in patients with cadasil, DSA can show segmental lumen irregularities in distal cerebral arteries suggestive of PACNS. Second, the potential role of transcranial Doppler sonography to distinguish cadasil from PACNS deserves further testing.
- - - - - - - - - -
ranking = 5
keywords = nervous system
(Clic here for more details about this article)

15/158. Molecular genetic diagnosis of a primary central nervous system T cell lymphoma.

    Primary central nervous system T cell lymphomas are rare tumors. Histologically, they may be indistinguishable from other entities like inflammatory processes. In such cases, molecular genetic verification of clonal T cell receptor (TCR) gene rearrangements is an indispensable diagnostic tool. Here we present a case where identification of TCR beta and gamma gene rearrangements by polymerase chain reaction was used to differentiate between a vasculitis and a primary CNS T cell lymphoma, which has profound consequences for therapy management and outcome.
- - - - - - - - - -
ranking = 5
keywords = nervous system
(Clic here for more details about this article)

16/158. Serial CT and MRI findings in a patient with isolated angiitis of the central nervous system associated with cerebral amyloid angiopathy.

    We report serial CT and MRI findings in a biopsy-proven case of cerebral amyloid angiopathy (CAA) with isolated angiitis of the central nervous system (CNS). A 69-year-old man had developed dizziness, dementia, and generalized seizure during the preceding 4 years. An initial examination by brain CT and MRI showed bilateral symmetrical periventricular lesions closely resembling those of Binswanger's disease. Subsequently, the lesions expanded slowly, involving a large area of the right cerebral hemisphere with an obvious mass effect. Since a primary brain tumor was suspected, a brain biopsy was performed, and histopathological examination revealed amyloid beta protein CAA within the meningocortical vessels associated with perivascular monocytic cuffing, indicating the presence of isolated angiitis of the CNS. Multinucleated giant cells containing intracytoplasmic beta protein amyloid around a heavily amyloid-laden cortical vessel were also observed. This is the first case report to show sequential radiographical studies of the leukoencephalopathy associated with CAA and isolated angiitis of the CNS.
- - - - - - - - - -
ranking = 5
keywords = nervous system
(Clic here for more details about this article)

17/158. Treatment of refractory Churg-Strauss-Syndrome (CSS) by TNF-alpha blockade.

    Churg-Strauss-Syndrome (CSS) often takes a mild course and is in many cases treated successfully by glucocorticosteroids (GC) alone. However, there are also several reports demonstrating the necessity of more intensive treatment in life threatening courses with cyclophosphamide and in less severe cases with other immunosuppressive or immunomodulatory drugs like azathioprine, methotrexate or interferon alpha. Relapses of the CSS are detected clinically and serologically and may require cyclophosphamide therapy as well as high-dose GC. We treated 3 cases between 2000 and 2001 that not only experienced a severe relapse (of the heart and the central nervous system) but also proved to be refractory to cyclophosphamide and GC therapy. In the absence of other options we decided to apply TNF-alpha blockers (etanercept in one case and remicade in the two other). This experimental treatment proved to be effective and safe and induced complete remission in one patient and partial remission in the second and at least stopped disease progression in the third. The BVAS 1 markedly improved after additional treatment with TNF-alpha blockers.
- - - - - - - - - -
ranking = 1
keywords = nervous system
(Clic here for more details about this article)

18/158. Linear magnetic resonance enhancement and optic neuropathy in primary angiitis of the central nervous system.

    A 38-year-old woman developed incoherent mentation, tremor, ataxia, and bilateral optic disc edema with mildly depressed visual acuity, nerve fiber bundle defects, and a left afferent pupillary defect. magnetic resonance imaging of the brain disclosed striking linear contrast enhancement radiating from the ventricular borders. Lumbar puncture showed a normal opening pressure with a lymphocytic pleocytosis and elevated protein. On the basis of these findings, the initial diagnosis was viral or post-viral meningoencephalitis and the patient was not treated. During the next 4 weeks, her condition worsened. A brain and meningeal biopsy disclosed findings typical of primary angiitis of the central nervous system. With aggressive treatment, her neurologic status and magnetic resonance imaging normalized and her optic neuropathy improved markedly. Optic neuropathy and linear magnetic resonance imaging enhancement should be recognized as features of primary angiitis of the central nervous system.
- - - - - - - - - -
ranking = 6
keywords = nervous system
(Clic here for more details about this article)

19/158. Kawasaki-like syndromes and other vasculitic syndromes in hiv-infected patients.

    Excluding drug-related hypersensitivity reactions, vasculitic syndromes are not common in hiv-positive patients. review of the existing literature suggests that hiv positive patients may be predisposed to polyarteritis nodosa, microscopic polyarteritis, Kawasaki-like syndromes, acute occlusion syndromes, primary angiitis of the central nervous system and erythema elevatum diutinum. With the exception of erythema elevatum diutinum, these vasculitic syndromes have significant morbidities and mortality if they are not treated. It is therefore important to make these diagnoses and to initiate appropriate therapy in a timely fashion. Because fevers, malaise, weakness, rashes, headaches and neurologic symptoms are common in hiv-positive patients, it is probable that some cases of vasculitis go undiagnosed. In this report, we review vasculitic syndromes seen in hiv-positive patients. We also re-examine a previously published case of a young hiv-positive male who died of an acute myocardial infarction without atherosclerotic disease. Immunohistopathology of the affected arteries suggests that he died of a Kawasaki-like syndrome.
- - - - - - - - - -
ranking = 1
keywords = nervous system
(Clic here for more details about this article)

20/158. Sympathetic ophthalmia associated with ocular and cerebral vasculitis: an angiographic and radiologic study.

    PURPOSE: To describe a case of sympathetic ophthalmia (SO) associated with ocular and cerebral vasculitis. methods: The authors report a 38-year-old woman who presented with bilateral uveitis 7 years after a penetrating trauma to the right eye. Ocular examination included fundus fluorescein and indocyanine green angiography. Medical history disclosed an episode of dysarthria and right-sided weakness. RESULTS: Anterior uveitis was present in the previously injured pseudophakic right eye, which harbored a piece of glass stuck in the lower temporal iris. In the left eye, vitreal inflammation with retinal sheathing and subretinal lesions was predominant. Besides retinal vasculitis, fundus angiography showed choroidal vasculitis. Neurologic studies were compatible with cerebral vasculitis. CONCLUSIONS: In SO, choroidal vasculitis that is invisible clinically can be demonstrated angiographically. Although rare, inflammation can also affect the nervous system.
- - - - - - - - - -
ranking = 1
keywords = nervous system
(Clic here for more details about this article)
<- Previous || Next ->


Leave a message about 'Vasculitis'


We do not evaluate or guarantee the accuracy of any content in this site. Click here for the full disclaimer.