Cases reported "Moyamoya Disease"

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1/132. 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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2/132. moyamoya disease of adult onset brain stem haemorrhage associated with bilateral occlusion of the vertebral arteries--case report.

    An unusual and first case of moyamoya disease of adult onset brain stem haemorrhage associated with occlusion of both vertebral arteries is reported. A 30-year-old man suddenly suffered from dyspnea, dysphagia, and left-sided hemisensory disturbance. Computed tomography and magnetic resonance imaging revealed a fresh haematoma in the left medulla oblongata and various-sized old infarcts in both parietal lobes. Cerebral angiograms disclosed occlusion of the bilateral internal carotid arteries on both sides at their intracranial portion, accompanied with the developed basal moyamoya vessels. The right vertebral artery occluded at its V2-V3 segment, in which the posterior inferior cerebellar artery was opacified via the posterior spinal artery, and the basilar artery was filled from the anterior spinal artery. The left vertebral artery was also occluded at the craniovertebral junction (V4) with collateral flow. Only one case of moyamoya disease associated with bilateral occlusion of the vertebral artery has been reported previously, and a haemorrhage into the medulla oblongata in moyamoya disease has never been described.
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3/132. migraine with aura-like headache associated with moyamoya disease.

    A 49-year-old woman was admitted to our hospital because of severe headache. She had a 10-month history of migraine with aura-like headache that occurred every 7 to 10 days and was preceded by photopsia. Brain CT showed cerebral infarction of the left occipital lobe. Bilateral carotid angiograms showed vascular occlusions in the supraclinoid portion of the bilateral internal carotid arteries with telangiectatic vessels acting as collateral channels to the occluded distal carotid arteries, which were consistent with the diagnosis of moyamoya disease. headache resolved gradually and has never developed again after the infarct of the left occipital lobe. Pathophysiological mechanisms of migraine-like headache were discussed. We conclude that borderline perfusion of occipital lobe cortex could be a trigger for the development of migraine with aura-like headache in susceptible patients. In the case of atypical attack of migraine detailed investigation should be done to detect underlying vascular diseases such as moyamoya disease.
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4/132. Neurofibromatosis, stroke and basilar impression. Case report.

    Neurofibromatosis type 1 (NF1) can virtually affect any organ, presenting most frequently with "cafe au lait" spots and neurofibromas. Vasculopathy is a known complication of NF1, but cerebrovascular disease is rare. We report the case of a 51-year-old man admitted to the hospital with a history of stroke four months before admission. On physical examination, he presented various "cafe au lait" spots and cutaneous neurofibromas. neurologic examination demonstrated right-sided facial paralysis, right-sided hemiplegia, and aphasia. Computed tomography scan of head showed hypodense areas in the basal ganglia and centrum semiovale. Radiographs of cranium and cervical spine showed basilar impression. Angiography revealed complete occlusion of both vertebral and left internal carotid arteries, and partial stenosis of the right internal carotid artery. A large network of collateral vessels was present (moyamoya syndrome). It is an uncommon case of occlusive cerebrovascular disease associated with NF1, since most cases described in the literature are in young people, and tend to spare the posterior cerebral circulation. Basilar impression associated with this case may be considered a pure coincidence, but rare cases of basilar impression and NF1 have been described.
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5/132. A case of adult moyamoya disease showing progressive angiopathy on cerebral angiography.

    In moyamoya disease, progression of carotid occlusive lesion in an adult patient is very rare. We report a case of adult moyamoya disease with acute angiographical stage progression and hemodynamic deterioration. A 56-year-old female complaining of left motor weakness visited our department. On MRI, infarct lesion was found in the right white matter. On cerebral angiography, occlusive lesions in the bilateral internal carotid arterial siphons and moyamoya vessels were found. The right side was stage V and the left side was stage III. On IMP-SPECT, decreased cerebral hemodynamic reserve of the right cerebral hemisphere was found. In this patient, right STA-MCA anastomosis was performed. After operation, she became possible to walk and discharged to home. Five months after operation, good collateral formation and improvement of hemodynamic reserve in the right hemisphere were found. However, on left carotid arteriography, the anterior and middle cerebral arteries were only slightly imaged, and the disease state progressed to stage IV. In addition, decreased blood flow and hemodynamic reserve were appeared in the left hemisphere.
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6/132. Primary antiphospholipid syndrome with moyamoya-like vascular changes.

    We describe a young girl with antiphospholipid syndrome (APS) and moyamoya-like cerebrovascular changes which reversed after anticoagulation. Although there was a risk of hemorrhage from collateral vessels, we speculate that this treatment may have prevented progression of the vascular abnormalities, while resolution of the thrombus resulted in improved cerebrovascular circulation.
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7/132. "Ivy sign" on fluid-attenuated inversion-recovery images in childhood moyamoya disease.

    We report on MR studies of a 15-year-old girl with moyamoya disease in whom diffuse leptomeningeal enhancement ("ivy sign") was revealed by fluid-attenuated inversion-recovery (FLAIR) and contrast-enhanced imaging. We speculate that the mechanism behind this enhancement is caused by a retrograde slow flow of engorged pial vasculature via leptomeningeal anastomosis. Nevertheless, it remains unknown whether the precise source of a high signal on FLAIR images is attributable to pial vessels themselves or congested thickening of the leptomeninges or both.
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8/132. A case of moyamoya-like vessels combined with brain anomaly.

    We report here a rare case of moyamoya-like vessels combined with brain anomaly. MR imaging revealed a small corpus callosum and stenosis of the internal carotid arteries. T2-weighted images revealed multiple hyperintense lesions in the cerebral deep white matter, suggesting ischemic or abnormal myelinated tissues. cerebral angiography showed aplastic carotid siphons and anomalous aneurysmal dilatation of the petrous portion of the internal carotid arteries. The terminal portion of the internal carotid arteries and horizontal portion of the middle cerebral artery were stenotic with moyamoya-like vessels.
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9/132. moyamoya disease mimicking a spontaneous internal carotid artery dissection on Doppler ultrasound.

    moyamoya disease and spontaneous internal carotid artery dissections are rare conditions, but both tend to affect young adults with potentially devastating consequences. A 43-year-old non-Japanese patient presented with neurological symptoms, which, following carotid Doppler ultrasound and angiography, was labelled as being due to a spontaneous internal carotid artery dissection. Repeat imaging at 3 months showed normalisation of the carotid Doppler findings which coincided with the formation of "moyamoya" vessels on the angiogram. This case report illustrates the changes on carotid ultrasound in early moyamoya disease which may mimic the appearances of an internal carotid artery dissection and demonstrates the change of the spectral Doppler waveform that occurs with the formation of new vessels at the base of the brain.
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10/132. Hemorrhagic moyamoya disease during pregnancy.

    BACKGROUND: Intracranial hemorrhage in pregnant patients with moyamoya disease is rare. We review the case of one such patient who presented with pre-eclampsia and a catastrophic intracerebral hemorrhage in order to highlight the associated management difficulties. methods: A case of a pregnant (31 weeks) female brought to the emergency department with hypertension and a progressive decrease in her level of consciousness is presented. She rapidly developed a dilated right pupil and left extensor posturing. A CT scan of her head showed a large putamenal intracerebral hemorrhage. She was intubated, ventilated and given intravenous mannitol and magnesium sulfate. She underwent a simultaneous craniotomy and cesarean section. Post-operatively the patient's ICP and jugular venous saturation were monitored in the intensive care unit. RESULTS: The patient delivered a 1185 g infant who did well. The patient's ICP was well controlled until the tenth post-operative day when she developed malignant brain edema and died. CONCLUSION: This case highlights three important points. First, simultaneous craniotomy and cesarean section can be performed. Second, intraoperative control of bleeding Moyamoya vessels is described. Third, the difficult post-operative management of these cases is highlighted. The literature regarding moyamoya disease and pregnancy is reviewed and some recommendations for the management of this rare but potentially deadly condition are presented.
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