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1/111. Moyamoya syndrome in a patient with congenital human immunodeficiency virus infection.

    A 10-year-old boy with congenital human immunodeficiency virus (hiv) infection developed recurrent episodes of left hemiparesis at age 7 years. The progression of his disease was followed by computed tomography, magnetic resonance imaging, magnetic resonance angiography, and cerebral angiography. The series of images showed progressive stenosis of both carotid arteries at the suprasellar origin with involvement of his anterior and middle cerebral arteries, while prominent collateral vessels developed from his external carotid supply through ophthalmic and middle meningeal arteries. The pattern of cerebrovascular disease is consistent with moyamoya syndrome. We suggest that further studies on the pathophysiology of cerebrovascular disease in patients with hiv could be helpful in understanding the cause of moyamoya disease as well. Also, with the various advances in treatment of hiv, neurovascular complications could be seen more frequently as the long-term survival in these patients improves.
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2/111. radiation-induced cerebrovasculopathy of the distal middle cerebral artery and distal posterior cerebral artery--case report.

    A 15-year-old girl underwent partial removal of a pituitary adenoma followed by local irradiation of the brain with a total of 70 Gy through two lateral opposing ports. Twenty years later, she experienced frequent transient ischemic attacks with left sensory disturbance. cerebral angiography revealed stenoses of the right distal middle cerebral artery (MCA) and the right distal posterior cerebral artery without net-like vessels. There was a severe decrease of vasoreactivity in the right hemisphere. Right superficial temporal artery (STA)-MCA anastomosis was performed. Her neurological deficits were resolved and perfusion reserve capacity had markedly improved 6 months later. We recommend STA-MCA anastomosis in such cases.
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3/111. frontal lobe infarction due to hemodynamic change after surgical revascularization in moyamoya disease--two case reports.

    A 60-year-old female and a 40-year-old male underwent surgical revascularization for moyamoya disease and suffered small infarction in the ipsilateral frontal lobe 3 or 4 days postoperatively. neuroimaging suggested that the bypass flow had caused rapid progression of occlusive changes in the carotid forks, a diminishing of moyamoya vessels, and flow reduction in the anterior cerebral artery ipsilateral to surgery, leading to critical ischemia in the frontal lobe. Surgical revascularization improves the outcome of patients with moyamoya disease, but postoperative management such as hydration is important to avoid ischemic complications due to frontal lobe infarction.
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4/111. Transient ischemic attack in heavy cannabis smokers--how 'safe' is it?

    Drugs are lately considered high-risk factors for cerebrovascular disease. Three male patients (mean age 24.6 years) who were heavy cannabis smokers presented with transient ischemic attacks (TIA) shortly after cannabis abuse. The complete examination of all 3 consisted of: EEG, brain CT scan, brain MRI, cerebral vessel angiography (digital subtraction and magnetic resonance angiography); also a full cerebrospinal fluid, urine and blood analysis (immunological, biochemical and hormonal tests were included). urine was further examined for drug metabolites. An extensive cardiological investigation was carried out. Small vessel leukoencephalopathy was revealed by the brain CT and MRI. EEG recordings of the first patient showed paroxysmal sharp waves with left hemispheric dominance. The other 2 patients had diffuse delta and theta activity in their EEG tracings. The urine analysis was positive for cannabis metabolites. There were no other abnormal findings in the rest of the meticulous and thorough study of all 3 patients, which leads to the conclusion that cannabis was the only risk factor responsible for the observed TIA, contradictory to other studies, which support that cannabis is a 'safe' drug. More research is required in order for this issue to be completely elucidated.
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5/111. 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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6/111. 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.
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7/111. angioplasty for intracranial symptomatic vertebrobasilar ischemia.

    OBJECTIVE: Although anterior circulation disease has both medical and surgical treatment options, management of vertebrobasilar disease has predominantly had only medical options. Some patients remain symptomatic despite medical treatment, and angioplasty has been demonstrated to relieve critical stenoses. However, the elative safety and effectiveness of medical and surgical treatments is not clearly known. This report reviews the clinical characteristics, indications, and procedural risks of intracranial angioplasty in a series of patients with symptomatic posterior circulation ischemia. methods: All patients undergoing angioplasty for critical intracranial vertebral or basilar artery stenosis at the University of california at san francisco Medical Center between June 1986 and July 1999 were included in a retrospective record review. Clinical features and procedural complications were recorded. RESULTS: angioplasty was performed on 25 vessel lesions in 25 patients in whom medical therapy had failed. The patients ranged in age from 50 to 87 years. Of the 25 stenoses, 10 were intracranial vertebral, 9 vertebrobasilar junction, and 6 basilar in location. angioplasty was effective in reducing the degree of stenosis by more than 40% in all 25 vessels. The overall risk of stroke or death was 28%, and the risk of disabling stroke or death was 16%. CONCLUSION: Intracranial posterior circulation angioplasty is effective in the reduction of stenosis and can be performed with relative safety. angioplasty can be considered as a treatment option in patients with recurrent ischemic symptoms despite medical therapy.
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8/111. Shaking limb transient ischemic attacks: unusual presentation of carotid artery occlusive disease: report of two cases.

    OBJECTIVE AND IMPORTANCE: Shaking limb transient ischemic attacks (TIAs) represent a rare clinical syndrome that has been ascribed to focal cerebral ischemia attributable to insufficient brain perfusion, usually resulting from carotid artery occlusive disease. The techniques conventionally used to evaluate this condition are contrast angiography, carotid artery ultrasonography, and magnetic resonance angiography. Treatment consists of internal carotid artery (ICA) endarterectomy or, in the case of complete ICA occlusion, extracranial-intracranial bypass. In this report, two patients with shaking limb TIAs are presented. For one patient, preoperative evaluations included single-photon emission computed tomographic studies with acetazolamide vasodilator challenge; for the second patient, computed tomographic angiography was used to assess vascular anatomic features. CLINICAL PRESENTATION: Two patients with severe carotid artery disease presented with brief, recurrent, shaking limb TIAs. Angiograms obtained for Patient 1 demonstrated complete ICA occlusion in association with severe external carotid artery stenosis, whereas preoperative single-photon emission computed tomographic scans revealed a lack of cerebrovascular reserve in response to acetazolamide challenge. Carotid artery duplex ultrasonography and computed tomographic angiography demonstrated severe stenosis of the ICA for Patient 2. INTERVENTION: Patient 1 underwent a left external carotid artery endarterectomy. Patient 2 underwent a right ICA endarterectomy. CONCLUSION: After surgery, the shaking limb episodes ceased for both patients. Postoperative single-photon emission computed tomographic scans for Patient 1 demonstrated increased cerebral blood flow in response to acetazolamide challenge. These data provide support for the concept that shaking limb TIAs are related to hemodynamic failure and that improvements in cerebral blood flow through conducting vessels can alleviate the condition.
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9/111. Pure or predominantly sensory transient ischemic attacks associated with posterior cerebral artery stenosis.

    Pure or predominantly sensory transient ischemic attacks (ps-TIAs) are uncommon, and underlying vascular abnormalities have rarely been described. The author reports 5 patients with TIAs which were of short duration, stereotypical and purely or predominantly sensory in nature. brain MRI did not reveal any lesions, while angiography demonstrated focal stenoses in the proximal portion of the posterior cerebral artery (PCA). It is concluded that ps-TIAs strongly suggest the presence of PCA disease. Repeated compromise of small vessels supplying the posterior-lateral part of the thalamus seems to be the pathogenic mechanism.
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10/111. perfusion MRI abnormalities in the absence of diffusion changes in a case of moyamoya-like syndrome in neurofibromatosis type 1.

    We report on a 12-year-old boy with neurofibromatosis type 1 who suffered a transient ischemic attack. Angiography revealed occlusion of intracranial arteries, moyamoya vessels and leptomeningeal collaterals. The conventional T2-weighted and the diffusion-weighted MRI images demonstrated no pathology. Dynamic first-pass postgadolinium T2* perfusion-weighted MRI depicted altered hemodynamics in the vascular territory of the left middle cerebral artery, which defined this region as ischemic tissue at risk. The patient suffered a repeat transient ischemic attack 5 days later.
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