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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Moyamoya is a rare disease characterized by fibrous dysplasia of the internal carotid and proximal cerebral arteries, which has been described mainly in young Japanese. We present a case of Moyamoya disease with renal artery involvement in a young male patient with an African origin. A 15-year-old boy was referred to our hospital due to uncontrolled blood pressure, headache, somnolence, cognitive deficit and multiple lacunar infarcts in the computed tomography. Cerebral arteriography showed the absence of the normal vascular anatomy at the level of the circle of Willis. The intracranial vessels presented severe stenosis or were occluded and replaced by an extensive network of ectasic collateral vessels. Abdominal ultrasound examination identified asymmetric kidneys, and renal arteriography showed a tight stenosis of the ostium and proximal segment of right main artery, which was only partially relieved by balloon angioplasty. A saphenous bypass from aorta to the right renal artery re-established the renal blood flow. Blood pressure dropped after surgery and was controlled with low doses of diuretic and beta-blocker. After arteriography he presented right hemiplegia, with partial recovering in the following months. In conclusion, we described the first case of Moyamoya disease with concomitant renovascular disease in a young adult of African origin, an association that may be more frequent than usually suspected in clinical practice.
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PMID:Moyamoya disease associated with renovascular disease in a young African-Brazilian patient. 1146 61

A 4-year-old girl was admitted to our department with the complaints of severe headache, nausea, vomiting, and photophobia. She had a 1-year history of migrainelike headache that occurred every 1 to 2 months. Her neurologic examination was normal, but T-weighted axial magnetic resonance imaging (MRI) of the brain showed flow void within the basal ganglia owing to parenchymal vascular collaterals. Magnetic resonance angiography and digital substraction carotid angiography showed both occlusion of the internal carotid artery in the supraclinoid portion and extensive parenchymal vascular collaterals. Because there was no evidence of risk factors for cerebral arterial occlusion and cerebral infarct or hemorrhage, she was diagnosed as having moyamoya disease. In any case of atypical migrainelike headache, a detailed investigation should be kept in mind to detect an underlying vascular disease such as moyamoya.
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PMID:Moyamoya disease presented with migrainelike headache in a 4-year-old girl. 1282 23

We report on a 13-year-old female with systemic lupus erythematosus (SLE) who exhibited symptoms of severe migraine and familial moyamoya disease. Cerebral magnetic resonance angiography (MRA) showed stenosis and occlusion of the bilateral internal carotid arteries associated with the development of collateral circulation (moyamoya vessels). In a child, as in this case, headaches with cerebral infarction associated with moyamoya disease are unusual. Few cases of SLE associated with familial moyamoya disease have been reported, with no previous reports of such cases from Korea. There were no evidences of antiphospholipid syndrome, and activity of SLE or other risk factors for cerebral occlusion were also absent.
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PMID:Systemic lupus erythematosus associated with familial moyamoya disease. 1471 35

Over a 5-year period, we investigated 77 consecutive patients (36 males, 41 females, mean age 40.9 years) referred to our hospital with the diagnosis of CNS vasculitis. Extensive workup including MRI, echocardiography, laboratory tests, angiography ( n=53), and biopsies at appropriate sites ( n=26) was performed based on individual history and symptoms. Prominent symptoms were stroke ( n=61), encephalopathy ( n=14), and headaches ( n=2). Vasculitis was finally diagnosed in 13 patients (17%) including isolated angiitis of the CNS ( n=3), giant cell arteritis ( n=4), and septic arteritis ( n=3). Thirty-two patients (42%) presented noninflammatory vasculopathies including moyamoya ( n=6), Sneddon's syndrome ( n=5), dissection ( n=4), CADASIL ( n=2), and collagen vascular disease ( n=9). Coagulopathy was found in 14 cases (18%) including antiphospholipid syndrome ( n=8) and APC resistance ( n=4). Other causes were cardiogenic embolism ( n=8), multiple sclerosis ( n=5), and migraine stroke ( n=3). Only a minority of patients referred for evaluation of suspected CNS vasculitis actually present with inflammatory vascular disease. Main differential diagnosis includes noninflammatory vasculopathies, coagulopathies, and cardiac disease. Since septic processes may be responsible for the symptoms, "blind" treatment with immunosuppressive agents should be strictly avoided.
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PMID:[Diagnosis and differential cerebral vasculitis diagnosis]. 1477 Feb 79

We report a case presenting with subarachnoid hemorrhage due to a ruptured aneurysm associated with unilateral moyamoya disease. The patient was a 29-year-old woman exhibiting sudden onset headache. Computed tomography revealed subarachnoid hemorrhage around the brain stem. Cerebral angiography showed a saccular aneurysm at the junction of the left P1 portion of the posterior cerebral artery and its perforator. The right internal carotid artery was occluded at the terminal portion, and the right middle cerebral artery territory was perfused anterogradely via abnormal moyamoya vessels in the basal ganglia. The aneurysm was completely embolized with preservation of the perforator. We suggest that as compared to surgical clipping by craniotomy, endovascular therapy is safe and effective for treatment of intracranial aneurysms associated with moyamoya disease.
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PMID:[Endovascular treatment of a ruptured aneurysm associated with unilateral moyamoya disease]. 1503 78

A case with arteriovenous malformation (AVM) associated with moyamoya phenomenon is reported. The 44 year-old female patient was presented with headache and vomiting. Computed tomography (CT) scan showed intraventricular hemorrhage. Cerebral angiography showed right frontal AVM and severe stenosis of right internal carotid artery. The AVM was fed by typical moyamoya vessels, Heubner's artery, and external carotid artery (ECA). We tried to embolize a bleeding point, compartment attached to lateral ventricle, but we could not. So, we embolized feeders from ECA and sent the patient to radiosurgery. We discuss here treatment and etiology of such rare condition.
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PMID:[A case of arteriovenous malformation associated with moyamoya phenomenon]. 1514 5

The authors describe a case of subarachnoid hemorrhage from moyamoya-like vessels associated with an arteriovenous malformation (AVM) in a 44-year-old Hispanic man who presented with severe headache. The AVM was located in the left parietal lobe and the ipsilateral middle cerebral artery was occluded. Although the patient was initially neurologically intact, he began to experience neurological deficits from mild vasospasm, illustrating the sensitivity of the underperfused portion of brain surrounding an AVM. His neurological deficits improved with aggressive hydration and elevated blood pressure. After a 3-week period, the AVM was resected without complication and all of the patient's neurological deficits resolved. The authors review radiographic findings of this unique case.
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PMID:Hemorrhage from moyamoya-like vessels associated with a cerebral arteriovenous malformation. Case report. 1554 Sep 30

After induction of noninvasive diagnostic tools, asymptomatic moyamoya disease is occasionally being diagnosed. However, there is no epidemiological or clinical data concerning this condition because of its rarity. To elucidate clinical features and outcomes in patients with asymptomatic moyamoya disease we conducted a nation-wide questionnaire survey. Questionnaires were sent to 88 neurosurgical institutes in 1994 and we received answers from 66 institutions (75% recovery). Diagnosis of moyamoya disease is based on the guidelines established by the research committee on moyamoya disease of the Ministry of Health and Welfare, Japan. Thirty three asymptomatic moyamoya disease patients were collected (11 male, 22 female) and divided into 2 groups; group 1 defined as patients without any symptoms, and group 2 as patients who did not show any symptoms except headache. 80% of group 1 patients were adults, whereas in group 2, children and adults were equally distributed. Follow-up periods were 3 years and 8 months on average. Among conservatively treated 28 patients, 2 patients died from suspected bleeding (mortality 7%) and 4 patients with TIAs resulted in good outcome. Among surgically treated 5 patients, 1 patient showed poor outcome due to post-operative infarction. Other 26 patients showed excellent outcome. Natural course of asymptomatic moyamoya disease seemed benign, but mortality from bleeding is not negligible. A prospective study of asymptomatic moyamoya disease is necessary to clarify its natural course and appropriate management strategy.
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PMID:[Clinical features and outcomes in patients with asymptomatic moyamoya disease--from the results of nation-wide questionnaire survey]. 1583 May 39

A 73-year-old woman without a history of cerebral ischemia suffered from sudden onset headache. Brain computed tomography (CT) showed intracerebral hemorrhage in the corpus callosum with intraventricular hemorrhage and thin subarachnoid hemorrhage in the basal cistern. Cerebral angiography showed narrowing of the right internal carotid artery just distal to its origin in the neck and abrupt occlusion just after branching of the ophthalmic artery with moyamoya-like vessels. The right A2 segment showed irregular dilatation and stenosis. The right middle cerebral artery was supplied from the basilar artery via the right posterior communicating artery. There was neither aneurysm nor arteriovenous malformation. Thin-slice bone-window CT of the skull base revealed hypoplasia of the right carotid canal. Aplasia of the right internal carotid artery was diagnosed. The origin of the hemorrhage was thought to be the irregularly opacified right A2 segment, which had been subjected to long-standing hemodynamic stress as collateral vessel.
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PMID:[Aplasia of the right internal carotid artery presenting with callosal hemorrhage: case report and review of the literature]. 1595 11

Moyamoya disease is an obstruction of the internal carotids and of the afferent and efferent channels of Willis polygon, which causes a collateral circulation, responsible for the typical angiographic image of a "puff of smoke" (Moyamoya, in Japanese). Its etiology is unknown, and it might be congenital or acquired. The clinical features are cerebral ischemia, recurrent transient ischaemic attacks, sensorimotor paralysis, convulsions and migraine-like headaches. We report a 2 years and 9 months old boy with Down syndrome and Moyamoya disease who presented with focal convulsions.
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PMID:Moyamoya disease and Down syndrome. 1613 77


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