1/38. 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.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/38. 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.- - - - - - - - - - ranking = 0.5keywords = vascular disease (Clic here for more details about this article) |
3/38. moyamoya disease. Report of three cases in Brazilian patients.moyamoya disease (MMD) is a chronic occlusive cerebrovascular disease of unknown etiology reported mainly in the Japanese. Most cases occur in children. The disease is rare in non-Oriental adults manifesting itself mostly as intracerebral hemorrhages. We describe MMD in 2 non-Oriental young adults and one adolescent that developed cerebral infarctions. The adults were medicated with aspirin and no medication was given to the adolescent. All patients did not deteriorate in a follow-up period from 1 to 4 years. Although rare, MMD is an important cause of stroke in young individuals and may well be underreported: only 18 patients have been reported till 1997 in brazil. Neurologists should include MMD in differential diagnosis of ischemic and hemorrhagic strokes in young adults.- - - - - - - - - - ranking = 0.5keywords = vascular disease (Clic here for more details about this article) |
4/38. 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.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
5/38. myocardial infarction with moyamoya disease and pituitary gigantism in a young female patient.myocardial infarction is very rare in young female patients with systemic vascular disorders. moyamoya disease is a cerebrovascular disease associated with an abnormal vascular network. This report presents a 19-year-old female patient who suffered from chest pain and exertional dyspnea for 2 months prior to admission. She had a history of moyamoya disease and pituitary gigantism since childhood. Her ejection fraction on echocardiogram was 20% and a perfusion defect with partial reversibility in the anterior wall was demonstrated on stress single photon emission computed tomography (SPECT). Diagnostic coronary angiogram revealed critical stenosis in the middle left anterior descending artery, which was treated by coronary stenting. Her subjective symptoms were relieved and the perfusion defect seen on SPECT decreased after coronary intervention.- - - - - - - - - - ranking = 0.5keywords = vascular disease (Clic here for more details about this article) |
6/38. Hypothalamic-pituitary dysfunction associated with moyamoya disease in children.moyamoya disease is a rare cerebral vascular disease that results in narrowing of the vessels of the circle of willis and the formation of a network of collateral vessels at the base of the brain for compensatory perfusion. moyamoya disease has the highest incidence during the first decade of life, and children present most frequently with transient ischemic attacks. We present two cases of moyamoya disease in children with associated hypothalamic-pituitary dysfunction. Both children presented to the endocrinologist for decreased growth velocity. One child had hypothyroidism and both had growth hormone deficiency. A review of the literature reveals a few isolated case reports of hypothalamic-pituitary dysfunction occurring with moyamoya disease and with other states of cerebral vascular insufficiency. We suggest that children with compromise of cerebral vascular perfusion be monitored closely for clinical signs and symptoms of hypothalamic-pituitary dysfunction.- - - - - - - - - - ranking = 0.5keywords = vascular disease (Clic here for more details about this article) |
7/38. Moyamoya syndrome with protein s deficiency.moyamoya disease is a cerebrovascular disease with progressive occlusion of both internal carotid arteries and of their branches and formation of a new vascular network at the base of the brain. Because of the angiographic appearance, it is named as moyamoya. The clinical features are cerebral ischaemia, recurrent transient ischaemic attacks, sensorimotor paralysis, convulsions and migraine-like headaches. A 10-year-old child who acutely developed hemiparesis, weakness and aphasia was found to have moyamoya disease and heterozygous protein S deficiency. This case shows us that during the thromboembolic events the coexistence of protein s deficiency and moyamoya should be investigated.- - - - - - - - - - ranking = 0.5keywords = vascular disease (Clic here for more details about this article) |
8/38. moyamoya disease associated with renovascular disease in a young African-Brazilian patient.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.- - - - - - - - - - ranking = 2.5keywords = vascular disease (Clic here for more details about this article) |
9/38. moyamoya disease in a Hispanic child: a case report.BACKGROUND: moyamoya disease was initially described by Suzuki and Takaku in 1963 as a radiographic phenomenon relating to the tiny collateral vessels characteristic of the disease that resemble a cloud or puff of smoke. The disease is rare and initially it was believed that the disease was confined to the Japanese population. It consists of occlusive vascular disease at the circle of willis with a tendency toward multiple ischemic neurological events and small strokes. In older populations it can often be associated with further vascular degeneration and intracerebral hemorrhage. This paper discusses the diagnosis, treatment, and management of moyamoya disease in a Hispanic child. CASE DESCRIPTION: The case of a Hispanic child who presented with transient ischemic attacks over a period of 1 year is reported. magnetic resonance imaging (MRI) revealed occlusive vascular disease in the posterior circle of willis. Digital subtraction cerebral angiography showed vascular occlusion at the base of the skull with collateral leptomeningeal and posterior circulation contribution in a pattern typical of moyamoya disease. technetium was injected for a SPECT study demonstrating less uptake in the left frontal and left parieto-occipital regions. The patient underwent a left superficial temporal-to-middle cerebral artery anastamosis followed by a right-sided anastamosis in a second operation. The patient tolerated the cerebral revascularization and was symptom-free at 6-month follow-up. cerebral angiography demonstrated improved perfusion in both cerebral hemispheres postoperatively. CONCLUSION: This article reports the occurrence of moyamoya in a Hispanic child. It illustrates the improved perfusion postoperatively as seen on digital subtraction cerebral angiography. Direct revascularization is felt to be difficult in children and alternatives such as encephaloduroarteriosynangiosis have been advocated. Direct revascularization was effective in treating moyamoya disease in this instance. Most of the discussion of moyamoya disease has been focused on the Japanese and far east population. This report confirms the entity as occurring in a Hispanic individual in the united states with no known Japanese ancestry.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
10/38. Are the strokes in moyamoya syndrome associated with down syndrome due to protein c deficiency?Moyamoya syndrome has occasionally been seen in association with down syndrome. We report a child with moyamoya syndrome and down syndrome who was admitted with repeated episodes of strokes; his magnetic resonance imaging and magnetic resonance angiography findings confirmed the presence of occlusive cerebrovascular disease with basal collateral vessels. His protein C levels were significantly decreased during the stroke. Complete clinical recovery was seen during follow-up. This raises the possibility of a link between protein c deficiency and down syndrome with moyamoya syndrome.- - - - - - - - - - ranking = 0.5keywords = vascular disease (Clic here for more details about this article) |
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