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1/17. Subcortical arteriosclerotic encephalopathy (Binswanger's disease). A vascular etiology of dementia.

    A 51-yearold man with moderate intermittent hypertension had a rapidly progressive, profound dementia in the absence of significant localizing neurological signs. Postmortem examination disclosed the vascular alterations and diffuse white matter degeneration which characterize subcortical arteriosclerotic encephalopathy (SAE) or Binswanger's disease. The case underscores the need to consider vascular disease as an etiology of dementia -- even in the absence of focal neurological deficit.
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ranking = 1
keywords = vascular disease
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2/17. Progressive intracranial vascular disease with strokes and seizures in a boy with progeria.

    progeria, a rare genetic disorder, is characterized by severe growth failure, premature aging, and very early atherosclerosis with coronary artery and cerebrovascular disease. There has been no detailed description of progressive cerebrovascular changes in progeria or any attempted neurologic correlation of those changes. A 5-year-old boy developed signs of progeria at 4 months and hypertension at 4 years, treated with atenolol and dipyridamole. Left-sided seizures with a left hemiparesis occurred at 5 years. magnetic resonance imaging (MRI) showed bilateral acute, subacute, and chronic cerebral infarctions. magnetic resonance angiography disclosed severe stenosis of the left internal carotid artery. The child was also found to have an aortic valve vegetation and was anticoagulated. He subsequently developed right-sided seizures, and treatment with gabapentin was started. Later, severe stenosis also of the right internal carotid artery was found. MRI showed new left cerebral infarction. The child's neurologic symptoms almost certainly were caused by cerebral infarctions from progressive atherosclerosis of major intracranial vessels, but clinical-neuroradiologic correlations were imprecise. There were multiple cerebral infarctions of different ages, some asymptomatic, others ipsilateral to the child's neurologic findings. No therapy has halted progression of the child's cerebrovascular disease.
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ranking = 6
keywords = vascular disease
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3/17. Vascular pseudoparkinsonism.

    Vascular pseudoparkinsonism may be confused with idiopathic Parkinson's disease. patients may be unnecessarily treated with anti-parkinsonian drugs while their underlying vascular disease is ignored. We investigated 250 parkinsonian patients seen in our movement disorders Clinic for a possible vascular etiology. After excluding those with a known secondary cause such as drug-induced parkinsonism, progressive supranuclear palsy, multiple system atrophy and hyperparathyroidism, brain computed tomography and/or magnetic resonance imaging were performed on those who showed poor or no response to levodopa. In those with an ischemic lesion demonstrated on neuroimaging, anti-parkinsonian drugs were stopped and the patients were reassessed. Eleven patients (4.4%) had ischemic brain lesions accounting for their parkinsonism. All were initially diagnosed as Parkinson's disease because of the prominence of bradykinesia and rigidity. gait disturbance was also common, but resting tremor was distinctly absent. Three anatomical patterns with different prognosis were identified. Three patients with basal ganglia lacunar infarct recovered spontaneously, three with frontal lobe infarcts remained static and five with periventricular and deep subcortical white matter lesions had progressive deterioration. autopsy in one patient confirmed bilateral frontal lobe watershed infarcts and the absence of brain stem lewy bodies. Parkinsonian patients with poor or no response to levodopa therapy should be investigated for a vascular etiology.
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ranking = 1
keywords = vascular disease
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4/17. Clinical outcome of standard extracranial-intracranial bypass surgery in patients with symptomatic atherosclerotic occlusion of the internal carotid artery.

    OBJECTIVE: In this retrospective study we wanted to determine the role of cerebral revascularization in patients with symptomatic occlusive cerebrovascular disease. Special emphasis was put on subsequent cerebrovascular events, benefit in neurological functioning and bypass patency, as evaluated during the follow-up period. methods: A total of 73 superficial temporal artery to the middle cerebral artery bypasses were performed on 67 patients from 1986-2000. All patients exhibited a symptomatic occlusion of the internal carotid artery verified by angiography. All patients in our group were refractory to medical treatment. 65 patients (69 bypasses) with a mean age of 61 years (range: 38-79 years) were followed up over an average time of 44 months (range: 1.5-150 months). RESULTS: The peri-operative morbidity rate was 3% with no mortality. 55 patients (85%) had no further cerebrovascular events after surgery, and only 7 (11%) patients experienced another cerebrovascular event. 57 (88%) patients showed an improvement of symptoms after surgery and only 1 patient fared worse during the follow-up period due to a stroke he suffered two years after bypass surgery. 90% of all bypasses remained patent during the follow-up. CONCLUSIONS: Although bypass surgery for occlusive cerebrovascular disease is still controversial, our retrospective study suggests both an improvement of symptoms and signs and a risk-reduction for future cerebrovascular events after surgery.
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ranking = 2
keywords = vascular disease
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5/17. Evaluation of EC-IC bypass for patients with atherosclerotic occlusive cerebrovascular disease: clinical and positron emission tomographic studies.

    The authors report clinical evaluations of extracranial-intracranial (EC-IC) bypass surgery for patients presenting with misery perfusion on positron emission tomography (PET) due to atherosclerotic occlusive cerebrovascular disease. Seven patients underwent PET both before and after undergoing EC-IC bypass surgery. Three of the 7 patients had transient ischaemic attacks (TIAs) and 4 had minor strokes. Five of the 7 had unilateral superficial temporal-middle cerebral artery (STA-MCA) anastomosis, one had bilateral STA-MCA anastomoses and one had STA-MCA anastomosis on one side and carotid endarterectomy on the other side. PET showed postoperative improvement in regional cerebral blood flow and metabolism in 5 of the 7 patients. In the remaining 2 patients, one showed no remarkable changes on PET and the other showed exacerbation on PET due to postoperative cerebral infarction. All three patients who had TIAs had no further TIAs after surgery. Two of the 3 patients postoperatively showed improvement in misery perfusion on PET. In the 4 patients who had minor strokes, 2 showed clinical improvements. One of the 2 showed improvement not only in motor function but also in the higher cortical functions such as verbal and performance intelligence quotients. Changes on PET correlated to the clinical improvement in 4 of 7 patients. The EC-IC bypass is therefore effective in patients with haemodynamic hypoperfusion syndromes by improving the state of low perfusion.
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ranking = 5
keywords = vascular disease
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6/17. Are low levels of HDL2-cholesterol a risk factor for atherosclerosis of cerebral vascular disease? Case report.

    A case of a 45 years old man with an atherosclerotic stenosis of right internal carotid and TIA event is reported. The patient showed an increase of total cholesterol and LDL-cholesterol serum levels and, in particular, a very low familiar HDL2-cholesterol serum value. The possibility that this last condition could represent an important co-factor of the extracranial cerebrovascular disease is discussed. The usefulness of a long-term follow-up of all family members, showing the same lipids pattern, is also suggested.
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ranking = 5
keywords = vascular disease
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7/17. radiation-associated atheromatous disease of the cervical carotid artery: report of seven cases and review of the literature.

    The natural history of postirradiation extracranial cerebrovascular disease is uncertain. Previous reported cases spanning 20 years of carotid surgery are difficult to evaluate, because patients may sometimes have unspecified symptoms, physical examinations, postoperative results, and follow-up. Also, the evolution of carotid surgery over the past two decades makes it impossible to compare earlier operative technique with the state-of-the-art technique of today. Our series of 7 patients underwent 9 carotid endarterectomies with an average follow-up period of 46 months. The number of patients is small, and although technically this is a more difficult operation, we feel the results are favorable and may be comparable with endarteerctomy procedures in nonirradiated patients. These patients should be approached as if radiation changes are not a major factor when they are considered for reconstructive arterial surgery.
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ranking = 1
keywords = vascular disease
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8/17. Cerebral blood flow measured by xenon-enhanced computed tomography as a guide to management of patients with cerebrovascular disease.

    CT scanning performed before and sequentially during the inhalation of stable xenon (32%), coupled with end-tidal xenon measurements, has made possible the routine construction of regional cerebral blood flow (rCBF) maps with resolution that approximates that of the CT scanner. The capability of obtaining quantitative flow maps with direct anatomic correlation is now available with a commercial package of hardware and software adapted to the General Electric 9800 scanner. The ability to distinguish between normal and reduced rCBF in specific vascular territories has proved useful in the management of cerebrovascular disease. Specific clinical dilemmas that have been addressed with rCBF information from xenon-enhanced CT scanning include the following: In the patient with asymptomatic occlusive disease, is normal rCBF preserved? Is there adequate collateral flow? Are cerebrovascular symptoms a result of emboli or chronic regional low flow? In the patient with complex multivessel occlusive disease, which revascularization procedure is indicated first? Did operation improve rCBF? Should a further procedure be added? May a diffusely diseased but patent artery, which is the source of emboli, be sacrificed safely without compromising rCBF? On the basis of experience with 155 patients, the management and understanding of cerebrovascular disease has been aided substantially by the incorporation of rCBF mapping by xenon-enhanced CT scan in the evaluation of these patients.
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ranking = 6
keywords = vascular disease
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9/17. Clinical features of subcortical arteriosclerotic encephalopathy (Binswanger disease).

    Subcortical arteriosclerotic encephalopathy, a chronic vascular dementia with hydrocephalus, was characterized pathologically in five patients by severe thickening of small vessels and by diffuse regions of white matter loss with gliosis. Lacunar infarcts were also present. The clinical picture in 11 patients was characterized by: (1) persistent hypertension and systemic vascular disease; (2) acute strokes; (3) subacute accumulation of focal neurologic symptoms and signs over weeks to months; (4) long plateau periods; (5) lengthy clinical course; (6) dementia; (7) prominent motor signs and pseudobulbar palsy and; (8) hydrocephalus. The pathogenesis of subcortical arteriosclerotic encephalopathy is unknown; possible mechanisms include diffuse ischemia and fluid transudation with subsequent gliosis related to subacute hypertensive encephalopathy.
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ranking = 1
keywords = vascular disease
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10/17. Lipohyalinosis and miliary microaneurysms causing cerebral hemorrhage in a patient with moyamoya. A clinicopathological study.

    Moyamoya is an intriguing and controversial syndrome. This patient study serves to align the pathophysiology of intracranial hemorrhage in moyamoya with the cerebral vascular disease seen with hypertension, or aging. The historical evidence linking lipohyalinosis and microaneurysms to cerebral hemorrhage is reviewed, the pathogenesis of this angiopathy is discussed, and explanations considered for its association with the vascular pattern of moyamoya. We propose that hemodynamics, genetics or both are among the primary operant etiologic factors.
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ranking = 1
keywords = vascular disease
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