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1/53. Multiple major cerebral artery thromboses with profound thrombocytopenia in acute leukaemia.

    A child with acute lymphoblastic leukaemia complicated by prolonged gastrointestinal and skin haemorrhages due to profound thrombocytopenia finally died of thrombotic occlusions of major cerebral arteries due to mucormycosis. biopsy of any suspect lesion is needed urgently before prolonged therapy with amphotericin b is started. So far there have been no cures in childhood.
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2/53. 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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3/53. Occlusion of the cerebral arteries in Recklinghausen's disease.

    Two children, aged 18 months and 6 years, who had Recklinghausen's disease, had occlusion of cerebral arteries. One child had no motor deficit but the other had right hemiparesis and partial occlusion of the left posterior cerebral artery, a fact not found in the literature.
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4/53. Pulmonary barotrauma-induced cerebral arterial gas embolism with spontaneous recovery: commentary on the rationale for therapeutic compression.

    Pulmonary barotrauma-induced cerebral arterial gas embolism (CAGE) continues to complicate compressed gas diving activities. Inadequate lung ventilation secondary to inadvertent breath holding or rapid buoyant ascent can quickly generate a critical state of lung over-pressure. Pulmonary over-pressurization may also occur as a consequence of acute and chronic pulmonary pathologies. Resulting barotrauma frequently causes structural failure within the terminal distal airway. Respiratory gases are then free to embolize the systemic circulation via the pulmonary vasculature and the left heart. The brain is a common target organ. Bubbles that enter the cerebral arteries coalesce to form columns of gas as the vascular network narrows. Small amounts of gas frequently pass directly through the cerebral circulation without occlusion. Larger columns of gas occlude regional brain blood flow, either transiently or permanently, producing a stroke-like clinical picture. In cases of spontaneous redistribution, a period of apparent recovery is frequently followed by relapse. The etiology of relapse appears to be multifactoral, and chiefly the consequence of a failure of reperfusion. Prediction of who will relapse is not possible, and any such relapse is of ominous prognostic significance. It is advisable, therefore, that CAGE patients who undergo spontaneous recovery be promptly recompressed while breathing oxygen. Therapeutic compression will serve to antagonize leukocyte-mediated ischemia-reperfusion injury; limit potential re-embolization of brain blood flow, secondary to further leakage from the original pulmonary lesion or recirculation of gas from the initial occlusive event; protect against embolic injury to other organs; aid in the resolution of component cerebral edema; reduce the likelihood of late brain infarction reported in patients who have undergone spontaneous clinical recovery; and prophylax against decompression sickness in high gas loading dives that precede accelerated ascents and omitted stage decompression.
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5/53. Abnormal intracranial vascular networks ("moyamoya" disease), possibly due to occlusion of bilateral internal carotid arteries--a case report with histometrical analysis.

    An autopsy case of abnormal intracranial vascular networks at the base of the brain corresponding to so-called rete mirabile, associated with occlusion of bilateral internal carotid arteries was reported. This patient was a 62 year-old female who died about two months after sudden onset of subarachnoid hemorrhage. At autopsy, abnormal vascular networks termed as rete mirabile were observed to be collateral blood supplies among the cerebral regions with flow of the anterior, middle, and posterior cerebral arteries, caused by long-standing obstruction of bilateral internal carotid arteries at the syphon level. Morphometrical analysis was done by measuring the length of internal elastic membrane of the internal carotid arteries in cross section, and comparing it with those of controlled persons of the same sex and age without any intracranial disorders and hypertensive histories. The result that no significant difference was observed between the former and the latter values suggested that the unusual cerebro-vascular disorder of this case developed not on the base of congenital anomaly including hypoplasia of internal carotid arteries or arteriovenous malformation but as an acquired lesion established for a long time.
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6/53. Thrombosed aneurysm of the middle cerebral artery with occlusion of the distal parent artery--case report.

    A 54-year-old female presented with subarachnoid hemorrhage due to rupture of a small middle cerebral artery aneurysm, found to be thrombosed at surgery and not visualized on the preoperative angiograms. One major branch of the middle cerebral artery was found to be occluded near the trifurcation. The lumen of the branch proximal to the occlusion had appeared as the aneurysmal opacification on the preoperative angiograms.
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7/53. Local thrombolytic therapy for thromboembolic occlusion of the middle cerebral artery.

    We report on 10 patients with thromboembolic occlusion of the middle cerebral artery (MCA) who underwent local thrombolytic therapy. Six patients developed a MCA occlusion during long-standing interventional neuroradiological procedures, while four had a proven or suspected cardio-embolic stroke. streptokinase or urokinase was applied by a microcatheter placed into the thrombus within six hours of clinical onset. Complete or partial revascularization was achieved in all patients. Recovery was complete in seven and partial in three of the patients. In two patients, minor haemorrhagic transformation of the infarct occurred, which did not lead to neurological deterioration. It is concluded that in a selected group of patients with MCA occlusion, local thrombolytic therapy represents a safe and effective therapy.
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8/53. Progressive occlusive disease of large cerebral arteries and ischemic events in a patient with essential thrombocythemia.

    We report a patient with essential thrombocythemia (ET) who developed progressive occlusive cerebrovascular disease accompanied by ischemic events. A 40-year-old woman presented with an ischemic stroke in the territory of the left middle cerebral artery (MCA). Diagnostic work-up disclosed a moderate stenosis of the left carotid siphon and a mildly increased platelet count. Due to aspirin intolerance warfarin was administered. Twelve months later, ischemic strokes in the left MCA territory recurred. A left internal carotid artery occlusion at the origin was diagnosed. bone marrow biopsy showed an increased number of megakaryocytes. warfarin was replaced by clopidogrel. Cerebral artery obstructions remained unchanged during the next 3 years (six follow-up examinations); no further ischemic events occurred during that period, while mild thrombocytosis persisted. ET may be associated with progressive obstructions of large cerebral arteries; in our case, clopidogrel was effective in preventing recurrence of ischemic events.
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9/53. Benedikt and "plus-minus lid" syndromes arising from posterior cerebral artery branch occlusion.

    A 53-year-old man was admitted with diplopia, right ptosis, left lid retraction, mild left sided weakness and involuntary movements. Neurological examination revealed plus-minus lid and Benedikt's syndromes together. magnetic resonance imaging (MRI) showed two distinct mesencephalic infarctions in territories of paramedian and pedincular perforating arteries. magnetic resonance angiography (MRA) showed severe stenosis of proximal segment of the right posterior cerebral artery (PCA). Rare clinical presentations such as Benedikt and plus-minus syndromes can be seen together and be due to stenosis of the posterior cerebral artery with specific regional infarctions.
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keywords = occlusion
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10/53. Inconspicuous visual field defect in anterior choroidal artery territory infarction.

    We describe a patient with a right anterior choroidal artery territory infarction and an inconspicuous left visual defect. The anterior choroidal artery is a unique artery of the cerebral circulation. The occlusion of this artery can result in dysfunction of motor, sensory, and visual systems with only rare involvement of higher cortical function. Among symptoms reported, visual abnormalities are the most variable and the least common. However, the visual field abnormality may be overlooked and the incidence underestimated since some patients may not be aware of the problem until uncovered by formal visual field testing.
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