Association Of Moyamoya Disease Research Articles

Management of a Rare Case of Moyamoya Disease Associated With Left Posterior Cerebral Artery Aneurysm

Background: The association of moyamoya disease and posterior cerebral artery aneurysms is relatively rare and the prognosis is poor. The management is extremely challenging for neurosurgeons. Case Report: We report a case of a 58-year-old man with a history of moyamoya disease who admitted complaining of sudden-onset headaches, vomiting, and seizure and the investigations revealed a subarachnoid hemorrhage, intracerebral hematoma, posterior cerebral artery aneurysms on a moyamoya disease field. The patient was treated surgically by the clipping of the aneurysm, but developed three days later a complication of the MMD and died. Conclusion: The prognosis of this association is very poor, explain the fact that a therapeutic strategy should be done to reduce the mortality and to determine the best management which is surgery versus embolization and revascularization.

Association of moyamoya disease with thyroid autoantibodies and thyroid function: a case−control study and meta‐analysis

Evidence suggests that elevated thyroid function and elevated levels of thyroid autoantibodies are associated with risk of moyamoya disease (MMD). Therefore a meta-analysis of all available evidence was performed, including unpublished data from our own center, in order to assess this association. Major literature databases were reviewed without language restrictions to identify studies examining the association between MMD and thyroid function or thyroid autoantibodies. These data were combined with those from our own prospective study. This study involved consecutively recruited patients with MMD and two groups of age-matched control patients: patients with non-MMD stroke and healthy individuals. The groups were compared in terms of serum triiodothyronine (T3), thyroxine (T4), free T4, free T3, thyroid stimulating hormone, anti-thyroperoxidase antibody and anti-thyroglobulin antibody. Our prospective study involved 28 patients with MMD, 28 age-matched patients with non-MMD stroke and 28 age-matched healthy control participants. The results showed an association between risk of MMD and elevated thyroid autoantibodies [odds ratio (OR) 9.00, 95% confidence interval (CI) 1.03-78.94] but not between risk of MMD and elevated thyroid function (OR 5.87, 95% CI 0.64-53.93). Meta-analysis of our data with findings from the literature further supported the association with elevated thyroid autoantibodies (OR 8.77, 95% CI 4.45-17.29) and indicated an association with elevated thyroid function (OR 9.74, 95% CI 2.18-43.49). Evidence strongly suggests that elevated thyroid autoantibodies and elevated thyroid function are independently associated with MMD. These clinical variables may require regular monitoring in patients with MMD.

An update on the diagnosis and treatment of adult Moyamoya disease taking into consideration controversial issues

Moyamoya disease (MMD) is characterized by a chronic progressive steno-occlusive disease at the distal portion of the internal carotid artery (ICA) with abnormal Moyamoya vessel (MMV) development without associated diseases. Recent advances in radiologic tests have increased the number of MMD patients. Beyond detection improvement by magnetic resonance angiography (MRA) or cerebral angiography, predicting factors for disease severity, future hemorrhage, clinical outcome, post-op complications, and technical advances in assessing cerebral hemodynamics have been increasingly reported. Although treatment of pediatric MMD is well established, controversy remains over the treatment in adult patients. In particular, there are debates over the disease entity of adult MMD, contralateral progression in adult unilateral MMD, treatment strategy for asymptomatic adult MMD, and the association of MMD with thyroid disease. The purpose of this review is to provide an update on the diagnosis and treatment of adult MMD while addressing controversial issues.

Abstract W P174: Association of Moyamoya Disease With Thyroid Autoantibodies and Thyroid Function: A Case-control Study and Meta-analysis

Background and Purpose: Evidence suggests that elevated thyroid function and elevated levels of thyroid autoantibodies are associated with risk of Moyamoya disease (MMD). Therefore we performed a meta-analysis of all available evidence, including unpublished data from our own center, in order to assess this association. Methods: We reviewed the English- and Chinese-language literature in major databases to identify studies examining the association between MMD and thyroid function or thyroid autoantibodies. We combined these data with those from our own prospective study conducted in our hospital. Results: In our center, 28 patients with MMD and 28 age-matched control patients with non-MMD stroke were included. Based on our literature searches, we identified two studies for inclusion in our meta-analysis, to which we added our own prospective study described above. Data from our hospital study indicated an association between risk of MMD and elevated thyroid autoantibodies (OR 9.00, 95% CI 1.03 to 78.94), but not between risk of MMD and elevated thyroid function (OR 5.87, 95% CI 0.64 to 53.93). Meta-analysis of our data with findings from the literature further supported the association with elevated thyroid autoantibodies (OR 8.77, 95% CI 4.45 to 17.29) and also indicated an association with elevated thyroid function (OR 9.74, 95% CI 2.18 to 43.49). Conclusions: Evidence strongly suggests that elevated thyroid autoantibodies and elevated thyroid function are independently associated with MMD. These clinical variables may require regular monitoring in patients with MMD.

Moyamoya Disease with Ebstein Anomaly: an Unusual Co-occurrence

Moyamoya (meaning a hazy puff of smoke) disease is a rare, idiopathic, persistent, occlusive cerebrovascular disease involving bilateral progressive stenosis or occlusion of a terminal portion of the internal carotid artery, or a proximal portion of the anterior cerebral arteries and the middle cerebral arteries. There are irregular perforating vascular networks (moyamoya vessels), seen in the base of the brain, which produce magnetic resonance images of this ‘puff of smoke’ condition. The association of moyamoya disease and congenital heart disease such as Coarctation of aorta, ventricular septal defect and tetralogy of fallot has been previously reported. Here we report a case of 15 yr old female child presented with seizure episode and was diagnosed as having moyamoya disease with ebstein anomaly. To the best of our knowledge this is the first case report of this co-occurrence of moyamoya disease with ebstein anomaly.

Renal agenesis and external iliac artery stenosis in an infant with moyamoya disease

We describe a 14-month-old girl who presented with arterial ischemic stroke due to moyamoya disease, unilateral renal agenesis and external iliac artery stenosis. The association of moyamoya disease with renal agenesis and external iliac artery stenosis has not been described before. This report expands the spectrum of moyamoya disease and suggests that moyamoya disease may have an intrauterine onset.

Moyamoya disease and arteriovenous fistula of the epiaortic vessels

The association of Moyamoya disease (MD) with other cerebrovascular disease has been reported in literature but not the occurrence of MD and arteriovenous fistula of the epiaortic vessels. A 61-year old Italian woman was admitted to our Department because of the sudden onset of Broca's aphasia. At general examination she presented a murmur above the right clavicle. Brain magnetic resonance imaging (MRI) showed an ischemic lesion involving left frontal and temporal lobes. Cerebral digital subtraction angiography (DSA) showed moyamoya vessels and an arteriovenous fistula between vertebral artery and internal giugular vein in right laterocervical region. This is the first report of the association of MD with arteriovenous fistula of the epiaortic vessels.

On the association of moyamoya disease with Down's syndrome

The occurrence of moyamoya disease in a child with Down's syndrome is reported. Ten previously published instances of this association are briefly reviewed, with comments on its possible significance.

Graves' Thyrotoxicosis and Moyamoya Disease

Cerebrovascular accidents due to Moyamoya disease, a disorder characterized by arterial stenosis at the base of the brain accompanied by typical net-like collateral vessels, occurred in two young Japanese women with Graves' disease when they were in thyrotoxicosis. In one patient, a second attack of cerebral infarction occurred with the recurrence of thyrotoxicosis. Association of Moyamoya disease and Graves' thyrotoxicosis is rare and the pathegenetic relationship is discussed.