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1/40. Vasculopathic changes of cadasil can be focal in skin biopsies.

    Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (cadasil) is a newly described cause of vascular dementia. Pathologic examination shows multiple small infarcts in the deep cerebral white matter together with a nonatherosclerotic, nonamyloid angiopathy involving the media of small cerebral arteries. Ultrastructurally, characteristic granular material is present in the basal lamina of vascular smooth muscle cells in cerebral and extracerebral blood vessels. The ultrastructural changes have also been demonstrated in skin biopsies of affected patients; consequently, some investigators have recently recommended skin biopsies for the diagnosis of cadasil. This study describes a 54-year-old male with a family history for strokes who had clinical and radiological features suggestive of cadasil. A skin biopsy was performed to confirm the diagnosis. Initially, the characteristic vasculopathic changes of cadasil were not identified within small blood vessel walls. However, multiple deeper sections in other areas showed electron-dense material associated with vascular smooth muscle cells, characteristic of cadasil. Subsequent genetic testing demonstrated a single nucleotide substitution at position 659 on chromosome 19p13.1 causing an amino-acid change (Cys --> Phe), a finding indicative of cadasil. The involvement of blood vessels within the dermis makes skin biopsy a useful adjunct in the diagnosis of cadasil. However, as illustrated by this case, the findings may be focal, requiring a thorough evaluation of the entire biopsy specimen.
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keywords = blood vessel, vessel
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2/40. Calcification of intracranial vessels in neurocysticercosis.

    We report calcification of intracranial vessels in neurocysticercosis. Calcification was observed in the middle cerebral arteries in two patients, and the circle of willis in two others. The patients with middle cerebral artery calcification underwent CT with inhaled stable xenon and an area of mild hypoperfusion was observed in the ipsilateral cerebral hemisphere.
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ranking = 0.46891552483544
keywords = vessel
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3/40. 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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keywords = vessel
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4/40. A rare sign of intracranial internal carotid artery dissection causing cerebral infarction: a case report and review of the literature.

    We describe a patient with CT-proven ischemic infarction of the left middle cerebral artery (MCA) territory. The emergency CT, on admission, was suggestive of intracranial internal carotid artery dissection by demonstrating gaseous gap defects in the arterial wall and a subsequent thromboembolic process in the MCA. To our knowledge, there is no previous report regarding radiologically detected gas within the vessel wall or lumen secondary to dissection. We discuss this uncommon radiologic sign and its possible pathogenesis with a review of the literature.
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keywords = vessel
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5/40. Impaired dynamic cerebral autoregulation in eclampsia.

    Eclampsia is frequently associated with brain edema, cerebral infarction or hemorrhage. Its underlying cerebrovascular pathophysiology is still poorly understood. We examined cerebral autoregulation by a non-invasive multimodal assessment in a 28-year-old primaparous woman with postpartal eclampsia. Transcranial Doppler sonography showed considerably increased cerebral blood flow velocity (CBFV) of all basal cerebral vessels. magnetic resonance imaging demonstrated multifocal vasogenic brain edema. Using transfer function analysis, a severely decreased phase shift between respiratory-induced 0.1-Hz oscillations of arterial blood pressure and CBFV was observed, indicating substantial disturbance of dynamic cerebral autoregulation (DCA). In contrast, CO(2)-vasomotor reactivity of the right middle cerebral artery was only slightly reduced. We therefore assume that the cerebral arteriolar dysfunction in eclampsia leads primarily to an impairment of the autoregulatory mechanism that is followed by different degrees of arteriolar vasodilation. Because of its probably high sensitivity to hemodynamic disturbances, assessment of DCA might be of great value in early pre-eclampsia for risk prediction of cerebral arteriopathy and eclampsia.
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ranking = 0.093783104967089
keywords = vessel
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6/40. vasoconstriction as the etiology of hypercalcemia-induced seizures.

    PURPOSE: Reversible cerebral vasoconstriction has been hypothesized to be the etiology of seizures due to hypercalcemia, but angiographic studies documenting vasoconstriction have not previously been available. methods: We present a 43-year-old woman who had frequent seizures that later evolved to status epilepticus with marked hypercalcemia at the time of the seizures. RESULTS: magnetic resonance imaging (MRI) of the patient's brain revealed high signal changes in T(2)-weighted imaging, fluorescence-attenuated inversion recovery (FLAIR), and diffusion-weighted imaging (DWI) over the bilateral occipital and thalamic areas. cerebral angiography showed blood vessels narrowing, disappearing altogether over the right posterior cerebral artery (PCA) branch, which is compatible with vasoconstriction. vasoconstriction caused the MRI high signal in the occipital area, which was associated with subsequent periodic lateralized epileptic discharges. The patient's clinical condition improved with management of seizures and hypercalcemia. A second brain MRI 2 weeks later revealed complete resolution of the high-signal lesions. Follow-up cerebral angiography study also showed total recovery of vasoconstriction. CONCLUSIONS: The sequence of events suggests the hypothesis that reversible cerebral vasoconstriction may play a role in hypercalcemia-induced seizures.
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ranking = 0.33333333333333
keywords = blood vessel, vessel
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7/40. Aneurysms located at the proximal anterior cerebral artery and anterior communicating artery associated with middle cerebral artery aplasia: case report.

    BACKGROUND: Aneurysms arising from the proximal anterior cerebral artery (ACA) are quite rare. Here, we report upon such a case of aneurysms located at the proximal ACA and anterior communicating artery associated with middle cerebral artery (MCA) aplasia. CASE DESCRIPTION: A 64-year-old woman complained of severe headache. Brain computed tomography scans demonstrated acute subarachnoid hemorrhage. Angiograms showed 2 aneurysms located at the anterior communicating artery and proximal ACA, but did not show an MCA shadow on the lesion side. Instead, multiple collateral vessels ran toward the sylvian fissure and supplied the MCA territory, together with hypertrophied perforating branches. The operative findings confirmed that the cordlike rudimentary MCA had no internal blood flow. The 2 aneurysms were secured by neck clipping. CONCLUSION: The combined effects of these anomalies on the hemodynamic equilibrium of the arteries and the genesis of the aneurysms are noteworthy.
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ranking = 0.093783104967089
keywords = vessel
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8/40. Treatment of a partially thrombosed giant aneurysm of the vertebral artery by aneurysm trapping and direct vertebral artery-posterior inferior cerebellar artery end-to-end anastomosis: technical case report.

    OBJECTIVE: The purpose of this article is to focus for the first time on the operative management of a direct vertebral artery (VA)-posterior inferior cerebellar artery (pica) end-to-end anastomosis in a partially thrombosed giant VA-pica-complex aneurysm and to underline its usefulness as an additional treatment option. methods: The operative technique of a direct VA-pica end-to-end anatomosis is described in detail. The VA was entering the large aneurysm sack. Distally, the pica originated from the aneurysm sack-VA-complex. The donor and recipient vessel were cut close to the aneurysm. Whereas the VA was cut in a straight manner, the pica was cut at an oblique 45-degree angle to enlarge the vascular end diameter. Vessel ends were flushed with heparinized saline and sutured. The thrombotic material inside the aneurysm sack was removed and the distal VA clipped, leaving the anterior spinal artery and brainstem perforators free. RESULTS: The patient regained consciousness without additional morbidity. magnetic resonance imaging scans revealed a completely decompressed brainstem without infarction. The postoperative angiograms demonstrated a good filling of the anastomosed pica. CONCLUSION: Despite the caliber mistmatch of these two vessels the direct VA-pica end-to-end anastomosis provides an accurate alternative in addition to other anastomoses and bypass techniques, when donor and recipient vessels are suitable and medullary perforators do not have to be disrupted.
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ranking = 0.28134931490127
keywords = vessel
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9/40. Retinal manifestations in fibromuscular dysplasia.

    fibromuscular dysplasia of the arteries (FMD) is a segmental angiopathy which may produce obstruction of the carotid, cerebral, renal, mesenteric, coronary or iliac arteries. Except for lesions related to arterial hypertension, retinal manifestations have not yet been reported. This paper describes the case of a 10-year-old boy with progressive deafness, a history of an unexplained stroke and progressive occlusions of the retinal arterioles in the fundus periphery. This resulted in retinal neovascularization and recurrent retinal and vitreous hemorrhages. Despite repeated photo- and cryocoagulation the eyes progressed to a tractional retinal detachment which was successfully treated by vitrectomy and scleral buckling. The diagnosis of FMD was made on the basis of a histopathological examination of a temporal artery biopsy. The child also presented an asymptomatic but severe aneurysmal dilatation of the aorta and CT scan and MRI showed dilated cerebral arteries. The father of our patient had died at the age of 27 years either from myocardial infarction or rupture of a dissecting aortic aneurysm. He was highly myopic and had lost one eye from retinal detachment. The younger brother of our patient also presents aneurysmal dilatation of the aorta and tortuous cerebral vessels. Ocular examination is still normal. The findings in this family are compatible with an autosomal dominant inheritance with variable expression.
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ranking = 0.093783104967089
keywords = vessel
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10/40. Two pediatric cases with occlusions of the ipsilateral internal carotid and posterior cerebral arteries associated with moyamoya vessels: "unilateral" moyamoya disease.

    Two pediatric cases of unilateral moyamoya disease are reported. The clinical symptoms and the findings of electroencephalography, angiography, and positron emission tomography in these cases were quite the same as those in the cases with bilateral involvement, which is typical of this disease. In both cases angiography demonstrated unilateral occlusion not only of the internal carotid artery but also of the posterior cerebral artery together with so-called Moyamoya vessels on the same side. Although infectious diseases were mentioned in the patients' histories, no obvious basic diseases related to the cerebrovascular occlusion were detected. We consider that these cases with unilateral involvement suggest the presence of unilateral moyamoya disease without progression onto the opposite side.
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ranking = 0.46891552483544
keywords = vessel
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