Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026654 (moyamoya)
 2,046 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The details of stroke in young adults remain unknown in Japan. We performed a multicenter survey to establish a stroke data bank for young adults in Japan. We collected clinical data of 7,245 acute stroke patients admitted to 18 hospitals in Japan. In patients admitted within the first 7 days of stroke, patients aged = < 50, = < 45, and = < 40 accounted for 8.9%, 4.2%, and 2.2%, respectively. Hypertension, diabetes mellitus, hypercholesterolemia, and non-valvular atrial fibrillation were significantly more frequent in the non-young than in the young, but smoking habits and patent foramen ovale were more frequent in the young than in the non-young. Brain infarction was the most predominant stroke subtype in the non-young, but not so in the young (62.6% vs. 36.7%, p < 0.01). Brain hemorrhage (20.8% vs. 32.1%, p < 0.01) and subarachnoid hemorrhage (7.3% vs. 26.1%, p < 0.01) were more frequent in the young. Causes of brain infarction and hemorrhage were often atypical in the young (2.8% vs. 25.1%, p < 0.001 and 4.6% vs. 20.2%, p < 0.0001, respectively). Causes of stroke in the young was often atypical, such as cerebral arterial dissection, Moyamoya disease, antiphospholipid syndrome, arteriovenous malformation, et al. Because causes and underlying risk factors of stroke in young adults were quite different from those in older patients, we need to establish the data bank and to explore optimal measures of the diagnosis and management for young stroke patients.
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PMID:[Stroke in young adults in Japan]. 1644 41

We report a 1-year-old girl who presented moyamoya disease associated with midaortic syndrome. She had been treated for cardiac failure and severe hypertension due to midaortic syndrome until she suffered seizure and repeated cerebral ischemic attack. Cerebral angiography revealed stenosis of the bilateral internal carotid artery at its terminal portion. She was successfully treated with encephaloduroarteriosynangiosis, and ischemic attack ceased postoperatively. This is the first report of moyamoya disease with midaortic syndrome. Although cerebral ischemic attack has been effectively managed by encephaloduroarteriosynangiosis, renovascular hypertension is still difficult to control.
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PMID:Moyamoya disease associated with midaortic syndrome. 1719 Sep 91

We describe an unusual vasculopathy in two sisters of non-consanguineous parents. The first child developed an acute hemiparesis and focal seizures at the age of 6 months during a febrile illness. Magnetic resonance imaging (MRI) of the brain showed bilateral cortical-subcortical infarction not confined to a vascular territory. Subsequently, the child had a persistent stable neurological deficit. Her younger sister had a similar encephalitis-like episode at the age of 4 months, with left-sided cortical-subcortical ischaemic lesions. Two months later she had left-sided focal seizures. MRI showed a right-sided cortical enhancement, magnetic resonance angiography (MRA) was normal. The neurological deficit was stable and she was seizure free. These episodes were initially interpreted as metabolic strokes, but work-up was normal and mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) was excluded. In their teens both sisters were diagnosed with pulmonary and systemic hypertension and, due to the arterial hypertension, myocardial hypertrophy. Renal artery stenosis, pathological pulmonary arteries, and stenosis and rarefication of coronary arteries were found; the aorta and retinal vessels were normal. Repeat cranial MRI and MRA showed multiple collaterals, while the carotid and basilar arteries were extremely narrowed (moyamoya appearance). We suggest the diagnosis is a hereditary systemic vasculopathy of unknown origin.
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PMID:Siblings with infantile cerebral stroke and delayed multivessel involvement--a new hereditary vasculopathy? 1742 10

35-year old patient was admitted to the Department of Neurology, Medical University of Bialystok because of paresis of his left upper limb, progressing over last 10 months and right facial nerve paresis, which started a month ago. During neurological examination he presented with right facial and arm paresis, dysarthric speech, obesity and hypertension. The patient was previously hospitalized in regional hospital, where a lumbar puncture was done revealing normal composition of cerebrospinal fluid. His brain CT revealed bilateral hypodensive areas in frontal and parietal regions of vasogenic character. Doppler ultrasound showed significant slowing of blood flow velocity in both internal carotid arteries. Brain angiography presented with very weak contrast filling of intracranial branches of carotid and vertebral arteries and showed stenosis at the terminal portion of the internal carotid arteries and at the proximal portion of the anterior and middle cerebral arteries. The patient had transthoracic and transesophageal echocardiography, Holter ECG, lab tests (routine lab tests plus coagulation system evaluation with C protein resistance test, anticardiolipne antibodies, antinuclear antibodies, anticytoplasmatic antibodies and thyroid hormones) checked--all tests were within normal range. Based on cerebral angiography and clinical symptoms, after excluding any other reasons of cerebral ischemia, the patient was diagnosed with moyamoya disease and arterial hypertension. The patient was treated pharmacologically with improvement--regression of face assymetry and dysarthria and diminishing of his right arm paresis. The authors of this paper pay attention to moyamoya disease as a rare reason of ischemic strokes in the young in our geographic region. They remind moyamoya disease diagnostic criteria, its etiology and treatment.
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PMID:[Moyamoya disease as a cause of ischemic cerebral stroke in young people]. 1744 85

Cerebral microbleed (CMB) on gradient-echo T2*-weighted MR imaging (T2*-w MR imaging) is associated with microangiopathy. Number of CMBs and new appeared CMBs are markers for stroke recurrence and the performance state. After CMB was reviewed in this manuscript, criterion for CMB is proposed as below. (1) Only microbleed associated with microangiopathies related to primary intracerebral hemorrhage or lacunar infarction is diagnosed as CMB. (2) A low intensity (round or oval shape, <7 mm in diameter) on T2*-w MR imaging defined as a CMB. Exception: (A) Micro-bleedings associated with trauma (cerebral concussion), brain tumor, cavernous angioma, or moyamoya disease are excluded. (B) Calcifications or vascular flow voids were excluded by CT or other MR imagings. Reference: (C) CMB is rarely correlated to a focal neurological sign. (D) CMB is associated with risk factors including hypertension, diabetes mellitus, or high age. CMB is very rare in patients less than 40 years old.
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PMID:[Clinical significance of cerebral microbleed and its diagnostic criteria]. 1766 50

Moyamoya disease is an occlusive intracranial arteriopathy owing to intimal hyperplasia with formation of abnormal cerebrovascular collateral networks; however, the etiology remains unclear. Although this disease is known to be associated with renovascular hypertension, it is extremely rare for it to be associated with stenoses of the coronary arteries. We herein described a case of a 56-year-old female with angina and asymptomatic moyamoya disease. We performed off-pump coronary artery bypass grafting (OPCAB) to avoid cardiopulmonary bypass and the risk of intraoperative hypotension. Conventional coronary artery bypass grafting has a potential risk of brain ischemia in moyamoya patients, but OPCAB may avoid this perioperative cerebral ischemic complication.
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PMID:Off-pump coronary artery bypass grafting in moyamoya disease. 1796 49

Direct surgical intervention to treat ruptured basilar tip aneurysms in patients with moyamoya disease has rarely been attempted, and patients who have undergone such treatment have not fully recovered. We review six cases of surgically treated ruptured basilar tip aneurysm associated with moyamoya disease, including our own case to illustrate aspects of surgical intervention and the difficulties encountered. Patients who underwent surgery after 4 weeks of the onset of symptoms showed impressive results. Of the patients who underwent surgery in the acute stage, two died, including our patient, and one showed excellent recovery. It is emphasized that to achieve satisfactory surgical outcome, the following factors should be considered: (i) delayed operation is preferable, with extracranial-intracranial bypass in selected patients; (ii) careful preservation of moyamoyas and transdural collaterals is mandatory; (iii) intraoperative rupture of the aneurysm should be avoided; and (iv) using a neuroanaesthetic technique of induced hypothermia and hypertension may be preferable.
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PMID:Moyamoya disease associated with basilar tip aneurysm. 1863 70

Intracerebral haemorrhage accompanied with cervical internal carotid artery (ICA) occlusion on the same side without moyamoya-like vessels is rare. A 73-year-old man with left ICA occlusion and no presence of moyamoya disease criteria underwent xenon-enhanced computed tomography with acetazolamide challenge test. The findings showed hypoperfusion and no vasoreactivity in the territory of the left middle cerebral artery. During follow-up he suffered bleeding in the left frontoparietal lobe. Cerebral angiography showed left ICA occlusion and cross flow via the anterior communicating artery without moyamoya vessels. Long-term ischaemia would make perforating or anastomotic arteries vulnerable. These arteries were easily ruptured by hypertension, resulting in intracerebral haemorrhage.
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PMID:Ischaemia-induced vascular vulnerability resulting in intracerebral haemorrhage with ipsilateral internal carotid artery occlusion. 1894 43

We described our management of a patient with moyamoya disease who presented for emergency cesarean section. A 29-year-old primigravida (162 cm, 61 kg) who had been diagnosed as having moyamoya disease at age 24, underwent urgent cesarean section at 35 weeks of gestation. Because she was medicated with aspirin, general anesthesia was selected. Anesthesia was induced with thiamylal and was maintained with sevoflurane in air oxygen mixture and fentanyl before delivery. After delivery, anesthesia was maintained with midazolam and fentanyl. Ventilation and depth of anesthesia were adjusted according to the end-tidal carbon dioxide tension and bispectral index, respectively. Hypertension caused by tracheal intubation was successfully prevented by nicardipine, which was ineffective for intraoperative hypertension. On the other hand, landiolol was effective for treating intraoperative hypertension and tachycardia. The cesarean delivery was uneventful and a healthy 2104 g neonate was delivered with Apgar score of 7 and 9 at 1 and 5 min, respectively. Landiolol was effective for treating intraoperative hypertension and tachycardia. Monitoring of depth of anesthesia, blood pressure, and ventilation would be essential for cesarean section under general anesthesia in patients with moyamoya disease.
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PMID:[General anesthesia for cesarean section in a patient with moyamoya disease]. 1946 10

Moyamoya disease is a cerebrovascular stenotic or occlusive disease predominantly seen in Asian countries. Sometimes there are coexisting renal artery lesions in Moyamoya disease patients. A 32-year-old multipara had a cesarean section at 33 gestational weeks due to preeclampsia. One month later, she developed subarachnoid hemorrhage and angiography demonstrated Moyamoya vessels with renal artery stenosis. After conservative therapy, the patient was discharged without any deficits. Our conclusion is that patients with Moyamoya disease carry a risk of cerebrovascular accident during pregnancy and postpartum. In this case, we did not diagnose Moyamoya disease with renal artery stenosis until the patient developed subarachnoid hemorrhage. It is very important to make a careful differential diagnosis of hypertension during pregnancy and the postpartum period.
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PMID:Postpartum subarachnoid hemorrhage due to Moyamoya disease associated with renal artery stenosis. 1975 43


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