Cases reported "Moyamoya Disease"

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1/11. 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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2/11. Intraventricular aneurysms--three case reports.

    Three rare cases of purely intraventricular aneurysms are described, including a unique aneurysm in the fourth ventricle. A 30-year-old female, a 47-year-old male, and an 11-year-old girl presented with disturbance of consciousness due to massive intraventricular hemorrhage. Digital subtraction angiography revealed an idiopathic peripheral aneurysm in the fourth ventricle in the first patient, and aneurysms in the lateral ventricle associated with moyamoya disease in the latter two patients. The former two aneurysms were treated surgically and histologically confirmed to be pseudoaneurysms. The latter aneurysm disappeared spontaneously within 2 months after onset. The aneurysm in the lateral ventricle was resected via a parietal corticotomy with stereotactic insertion of an 8-Fr silicone tube to guide the approach route. This method was very useful because computerized neuronavigation was not available. The aneurysm in the fourth ventricle was resected via a midline suboccipital approach with C-1 laminectomy. Conservative treatment is usually recommended initially for patients with intraventricular aneurysm because spontaneous cure often occurs. We recommend direct surgery if the size of the aneurysm remains unchanged, because the risk of surgery has decreased recently owing to new techniques for neuronavigation.
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3/11. Intracerebral hemorrhage and moyamoya disease in pregnancy.

    PURPOSE: To present a case of moyamoya disease with intracranial hemorrhage complicating pregnancy. CLINICAL FEATURES: A 36-yr-old parturient at 34 wk gestation presented with left hemiparesis, headache, nausea and vomiting. Subsequent deterioration in level of consciousness and the development of a dilated right pupil necessitated immediate intubation. Urgent non-contrast CT scan revealed a large right intracerebral hematoma with transtentorial herniation. The patient underwent simultaneous emergency cesarean section and craniotomy. A postoperative angiogram revealed findings consistent with Moyamoya disease. The neonate survived but the patient developed severe cerebral edema and died eleven days postoperatively. CONCLUSION: adult patients with moyamoya disease often present with intracranial hemorrhage which poses unique anesthetic challenges. We report a case of intracerebral hemorrhage during pregnancy, which is known to be associated with high morbidity and mortality. The anesthetic techniques are reviewed and discussed.
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4/11. moyamoya disease in a child with previous acute necrotizing encephalopathy.

    A previously healthy 24-day-old boy presented with a 2-day history of fever and had a convulsion on the day of admission. MRI showed abnormal signal in the thalami, caudate nuclei and central white matter. Acute necrotising encephalopathy was diagnosed, other causes having been excluded after biochemical and haematological analysis of blood, urine and CSF. He recovered, but with spastic quadriparesis. At the age of 28 months, he suffered sudden deterioration of consciousness and motor weakness of his right limbs. MRI was consistent with an acute cerebrovascular accident. Angiography showed bilateral middle cerebral artery stenosis or frank occlusion with numerous lenticulostriate collateral vessels consistent with moyamoya disease.
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5/11. chorea and Broca aphasia induced by diabetic ketoacidosis in a type 1 diabetic patient diagnosed as moyamoya disease.

    We here report one case of hemichorea and Broca aphasia occurred with diabetic ketoacidosis. A 20-year-old woman with type 1 diabetes mellitus had experienced diabetic ketoacidosis fourth time after the onset of diabetes. At the third ketoacidotic episode, the patient was admitted to our hospital for the first time to show hemichorea of the left extremities. Brain computed tomography (CT) demonstrated a high-density area in the right caudate head and low-density area in the right putamen. magnetic resonance angiography (MRA) demonstrated a stenosis at the root of the bilateral middle and anterior cerebral arteries. The hemichorea disappeared within 3 days. At the fourth ketoacidotic episode, not hemichorea but unconsciousness was there for 2 days even after ketoacidosis disappeared. After the unconscious state, Broca aphasia was demonstrated for 15 days. The cerebral angiography showed a finding compatible to moyamoya disease. These findings support that chorea and Broca aphasia induced by diabetic ketoacidosis was developed in addition to blood vessel abnormalities such as moyamoya disease. We suggest that poorly controlled diabetic patients with hemichorea should undergo cerebral angiography.
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6/11. A ruptured middle cerebral artery aneurysm originating from the site of anastomosis 20 years after extracranial-intracranial bypass for moyamoya disease: case report.

    BACKGROUND: Direct revascularization through a superficial temporal artery-middle cerebral artery (STA-MCA) bypass is often performed to prevent ischemic or hemorrhagic attack in patients with moyamoya disease. This is the first reported case of aneurysm formation and rupture due to an STA-MCA bypass in a patient with moyamoya disease. CASE DESCRIPTION: A 52-year-old man who had undergone bilateral STA-MCA bypass for caudate hemorrhage due to moyamoya disease 20 years previously suffered from sudden-onset unconsciousness. Computed tomography revealed a massive intracerebral hematoma (ICH) in the left frontoparietal region. Angiography showed good patency of the anastomoses and stage IV moyamoya disease. However, no other abnormality was found. Emergency evacuation of the hematoma was performed. The patient's postoperative course was uneventful, but consciousness disturbance of sudden onset occurred 1 month later. Computed tomography showed a hematoma in the lateral ventricle and acute hydrocephalus. Repeat angiography revealed an aneurysm on the left side of the anastomosis. Bilateral ventricle drainage tubes were inserted, and the aneurysm was clipped. A ventriculoperitoneal shunt was later performed. CONCLUSION: In patients with moyamoya disease who have undergone extracranial-intracranial bypass surgery, progressive hemodynamic stress may cause the formation of de novo aneurysms after a postoperative period of several decades. Imaging examinations should therefore be performed periodically for follow-up, and a de novo aneurysm should be suspected in a patient who has an unusual ICH.
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7/11. moyamoya disease presenting as intracerebral hemorrhage during pregnancy: case report and review of the literature.

    Intracranial hemorrhage during pregnancy is rare. The authors present a case of moyamoya disease associated with pregnancy. A 32-year-old woman who was at 32 weeks of gestation experienced sudden severe headache followed by loss of consciousness. Computed tomography revealed an intracerebral hematoma in the left temporal lobe, and left carotid angiograms revealed moyamoya disease. The patient underwent emergency craniotomy, the hematoma was removed, and encephaloduroarteriosynangiosis was performed. The patient showed good recovery and, 2 months later, delivered a child vaginally without any trouble. Most intracranial hemorrhages during pregnancy result from cerebral aneurysms or arteriovenous malformations (AVMs), and few cases due to moyamoya disease have been reported. It is generally believed that subarachnoid hemorrhage due to cerebral aneurysms is likely to occur during the middle trimester of gestation, with the risk increasing progressively toward the third trimester. This may be explained by the fact that the cardiac output increases rather acutely from the first to the middle trimester. There seems to be no significant correlation between the stage of pregnancy and the occurrence of hemorrhage due to AVM. Most authors think that the operative indications for intracranial hemorrhage during pregnancy should be the same as for the nonpregnant state.
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8/11. Subependymal hematoma in "Moyamoya" disease.

    A 27-year-old woman in the ninth month of her pregnancy suddently developed nausea and motor weakness of her right lower extremity while shopping. Subsequently a disturbance of consciousness and right-sided hemiparesis developed. spinal puncture yielded clear CSF but CT scan demonstrated a left subependymal hematoma. Angiographical examination led to a diagnosis of "Moyamoya" disease. The source of the intracranial hemorrhage could not be identified. The hematoma could not have been diagnosed accurately without the CT scan.
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9/11. moyamoya disease in pregnancy: a case report.

    moyamoya disease is a rare condition characterized by narrowing and occlusion of the internal carotid arteries. The disease usually presents as alteration of consciousness caused by intracranial hemorrhage. We describe a case of moyamoya disease presenting as seizures in a pregnant patient. The characteristic angiographic appearances and plan of management are described.
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10/11. An atypical case of adult moyamoya disease with initial onset of brain stem ischemia.

    We present an atypical case of adult moyamoya disease whose clinical onset consisted of ischemic symptoms of the brain stem. She initially presented with left hemisensory disturbance caused by a pontine lesion, followed by a myelopathy of the upper cervical spinal cord. Eight months later, she presented with left hemiplegia and disturbed consciousness. magnetic resonance angiography showed significant narrowing of both horizontal portions of the middle cerebral arteries (M1). Conventional angiography revealed bilateral occlusion of the internal carotid arteries. Her anterior circulation was supplied from the vertebro-basilar system through Moyamoya vessels and leptomeningeal collaterals. The intracranial steal phenomenon was thought to be the reason for the preceding events in the brain stem and upper cervical spinal cord. In addition, transcranial color-coded duplex sonography (TCCS) showed identical findings to conventional angiography with antegrade flow in the proximal M1 and retrograde flow in the distal M1. Thus, TCCS was useful for diagnosing the M1 occlusion in this case of moyamoya disease.
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