Moyamoya Vessels Research Articles

Selective and superselective angiography of pediatric moyamoya disease angioarchitecture: the anterior circulation.

The angioarchitecture of the so-called moyamoya vessels in children has not been explicitly analyzed. We aimed to investigate the precise anatomy of the vascular anastomotic networks in patients with childhood moyamoya disease. Six children diagnosed with moyamoya disease for the first time underwent an angiographic investigation with selective and superselective injections. We recorded the arterial branches feeding the moyamoya anastomotic networks, their connections and the recipient vessels. Depending on the level of the steno-occlusive lesion, the feeding vessels included the medial striate arteries, the perforators of the choroidal segment of the carotid, the uncal artery, the medial and lateral branches of the intraventricular segment of the anterior choroidal artery, perforators of the communicating segment, the superior hypophyseal arteries, the prechiasmal branches of the ophthalmic artery, the ethmoidal arteries and the dural branches of the cavernous carotid. Through connections, which are described, the recipient vessels were the lateral striate arteries and the middle cerebral, the medial striate arteries and the anterior cerebral, medullary arteries around the ventricular system, anterior temporal branches of the middle cerebral, orbitofrontal and frontopolar branches of the anterior cerebral, as well as other cortical branches of the anterior and middle cerebral territories. The use of high quality selective and superselective angiography enabled us to clearly demonstrate for the first time aspects of the microangiographic anatomy of the moyamoya anastomotic network previously only vaguely or incompletely described.

Hemiplegic migraine: An atypical presentation of Moyamoya disease.

Sir, Migraine is a common disorder causing attacks of disabling headaches that can be accompanied by an aura in a third of patients. Few patients having motor weakness during the aura qualify for hemiplegic migraine.[1] This can occur as a sporadic or familial disorder; the latter type is characterized by affection of one first or second degree relative, and then known as familial hemiplegic migraine.[2] Moyamoya disease is a progressive cerebrovascular occlusive disease identified by stenosis of distal internal carotid arteries (ICA) and their branches. Dilation of small cerebral vessels in an attempt to compensate for the decreased blood flow in the anterior circulation produces the characteristic “puff of smoke” appearance on angiography.[3] Headache is one of the common presentations of moyamoya disease in children.[4] Pathophysiological mechanisms for headache remain unclear, but presumed to be closely related to the intracranial oligemic state, as reflected by the disappearance of headache after the improvement of cerebral bold flow augmentation surgery, that is, vascular anastomosis.[5] Familial hemiplegic migraine could be a rare presentation of moyamoya disease. We describe a case of moyamoya disease presented as familial hemiplegic migraine. An 11-year-old girl presented to the pain clinic with periodic throbbing pain over right forehead associated with complete hemiparesis of left half of the body since last 2 years. Frequency of headache was 1-2 times/month with intermittent symptom free periods up to 3 months. Headache was not related to any particular time of the day and any precipitating, aggravating or relieving factors were absent. Initially, her complaints began with nausea, circumoral paresthesia and paresthesia of the left half of the body lasting for 20-30 min. Initial aura symptoms were followed by pain and hemiparesis for 4-10 h with spontaneous and complete recovery thereafter. She was treated for migraine without any effect. There were no past histories of head injury, seizures, involuntary movements, developmental delay, central nervous system infections, or other significant medical illness. On examination, she was afebrile, with normal vitals, pupils were equal (7 mm) and reactive to light, cranial nerve examination was normal and disc margins were sharp on fundoscopy. Tone, power, and reflexes were within normal ranges; bilaterally in both the extremities. Patient's elder sister (15-year-old) had similar symptoms but never underwent treatment. Magnetic resonance imaging brain showed bilateral periventricular deep white matter ischemia with mild cortical atrophy. Cerebral angiography revealed the occlusion of supra-clinoid segments of ICA bilaterally with evidences of moyamoya vessels [Figure 1]. Pial to pial collateral circulation from bilateral posterior cerebral artery to anterior cerebral artery and middle cerebral artery noted through pericallosal collateral vessels [Figure 1]. Considering moyamoya disease is the primary pathology responsible for the headache patient was referred for neurosurgical management. Figure 1 (a) Digital subtraction angiography image showing pial collaterals. (b) Choroidal to choroidal pial collaterals (c) Moyamoya vessels This particular case report and the review of the existing literatures suggest that pediatric patients presenting with migraineous or cluster headache refractory to prophylactic and abortive medical therapies should be evaluated further with cerebral angiography. If moyamoya disease is responsible, an early revascularization procedure will alter the prognosis and life expectancy in such patients.

Abstract WP223: Chronic Atherosclerotic Moyamoya Syndrome of the Middle Cerebral Artery: A Hemodynamic Study with CT Perfusion

Background: Moyamoya vessels, an abnormal vascular collateral network at the base of the brain, are associated with moyamoya disease and secondary moyamoya syndrome. Several studies have suggested that ischemic symptoms in patients with moyamoya vessels are most closely related to hemodynamic impairment. However, few studies have evaluated cerebral perfusion state in secondary atherosclerotic moyamoya syndrome. The purpose of our study was to evaluate cerebral hemodynamics measured by acetazolamide CT perfusion (CTP) imaging in patients with moyamoya syndrome associated with symptomatic atherosclerotic occlusion of the unilateral middle cerebral artery (MCA). Methods: Multidetector helical CTP imaging was performed in 14 patients (mean age, 54.4 years), who were clinically diagnosed to have moyamoya syndrome associated with unilateral atherosclerotic steno-occlusion of the proximal MCA. All patient underwent cerebral angiography and CTP imaging. Hemodynamic quantitative parameters of regional cerebral blood flow (rCBF), cerebral blood volume (rCBV), and mean transit time (rMTT) were measured in the anterior cerebral artery (ACA), MCA, and posterior cerebral artery (PCA) territories of both hemispheres separately. The Cerebral vascular reserve (CVR) capacity was measured using acetazolamide. Results: Mean resting rCBF in the ipsilesional ACA and MCA territories were significantly lower than in the contralateral territories (P<0.05). After acetazolamide infusion, the rCBF was significantly increased in the ipsilesional ACA, PCA, and all three contralateral territories (P<0.05) and rCBV was significantly increased in the all ipsilesional and contralateral territories (P<0.05). The CVR capacity changes were not significantly different between ipsilesional and contralateral territories. Conclusions: These findings suggest that the affected MCA territory has decreased perfusion with poor vasomotor reactivity despite abundant collateral circulation through the basal moyamoya vessels in patients with atherosclerotic moyamoya syndrome.

A case of unilateral moyamoya disease suffered from intracerebral hemorrhage due to the rupture of cerebral aneurysm, which appeared seven years later

Background:Whether unilateral moyamoya disease (MMD), confirmed by steno-occlusive lesion at the terminal portion of internal carotid artery with formation of moyamoya vessels unilaterally and normal or equivocal findings contralaterally, is an early form of definite (bilateral) MMD remains controversial. It is well-known that adult patients with MMD tend to suffer from cerebral hemorrhage, occasionally due to the rupture of aneurysm arising from moyamoya vessel.Case Description:A 61-year-old woman was diagnosed as unilateral MMD incidentally and followed by magnetic resonance imaging annually. Seven years after the diagnosis, cerebral aneurysm appeared on the moyamoya vessel. Before further examination, the aneurysm ruptured and resulted in massive cerebral hemorrhage.Conclusion:Even in the unilateral MMD, cerebral hemorrhage may occur due to the rupture of cerebral aneurysm. Careful follow-up is recommended and early treatment is required once cerebral aneurysm is detected.

Moyamoya syndrome occurred in a girl with an inactive systemic lupus erythematosus

We report the case of a 17-year-old Korean girl with systemic lupus erythematosus (SLE) who presented with sudden weakness of the right-sided extremities and dysarthria. Oral prednisolone was being taken to control SLE. Results of clinical and laboratory examinations did not show any evidence of antiphospholipid syndrome or thromboembolic disease nor SLE activity. Cerebral angiography showed stenosis of the left internal carotid artery and right anterior cerebral artery with accompanying collateral circulation (moyamoya vessels). After the patient underwent bypass surgery on the left side, she recovered from the neurological problems and did not experience any additional ischemic attack during the 14-month follow-up period. This case represents an unusual association between moyamoya syndrome and inactive SLE (inactive for a relatively long interval of 2 years) in a young girl.

525 Paediatric Moyamoya Syndrome Associated with Iron Deficiency Anaemia: A Case Report and Review of the Literature

IntroductionMoyamoya syndrome is a rare, progressive disorder characterised by stenosis of the vasculature of the Circle of Willis with formation of a tortuous collateral system. The aetiology may be idiopathic,...

Silent cerebral infarct in child patients with beta thalassaemia intermedia

There are numerous reports that patients with thalassemia are faced with hypercoagulability leading to vascular disorders. One of these complications is known as a silent infarct, defined as a small infarct detected by cerebral imaging but without any neurological symptoms. Since it has a progressive nature, it is of vital importance because it may lead to symptomatic cerebrovascular accidents in the future. Twenty-two children with thalassemia intermedia were enrolled into the study and MRI scans were performed. All demographic data and clinical features of the patients were obtained during the follow-up period. In addition to the patients, 13 healthy controls were included to compare serum anticoagulant levels with those of the thalassemia intermedia patients. Four of the patients were found to have silent cerebral infarcts (SCIs). The lesions involved varying amounts of the deep cerebral white matter and sub-cortical areas. One patient showed 'net line' filling defects within the ambient cistern on MRI images corresponding to moyamoya vessels. Three patients had undergone splenectomy, and three were transfused irregularly and had less than six transfusions per year. More importantly, protein C levels were lower and platelet levels were significantly higher in the patient group compared with controls. We were not able to find any association between SCI and transfusion number or splenectomy. However, of the total patients four thalassemia intermedia patients had SCI in early childhood and this is an unusual finding. In order to verify the findings, further studies must be conducted involving larger numbers of patients.

Moyamoya disease: A case of vanishing cerebral vessels

To provide an overview of moyamoya disease (MMD) including pathophysiology, epidemiology, clinical presentation, diagnosis, treatment, and prognosis. Selected clinical and epidemiological studies, review articles, and diagnostic guidelines for MMD. MMD is a rare cerebrovascular disease characterized by progressive stenosis of the distal internal carotid arteries and their major branches. The dilated and fragile basal collateral circulations display a "puff of smoke" appearance and thus are called moyamoya vessels. Other unique features of MMD include 2:1 female preponderance and its peak incidence in two age groups: early childhood and adults in their mid-40s. The pathophysiology of MMD is unclear and possible causes include genetic linkage, angiogenesis, autoimmune disease, cranial radiation, and infection of the head and neck. Most patients are symptomatic and may present with ischemic or hemorrhagic strokes, seizure, or headache. The diagnosis depends on clinical presentation and radiographic imaging, and disease progression may be halted with direct or indirect cerebral revascularization. It is important to make a correct diagnosis and provide appropriate treatment to reduce the morbidity and mortality associated with MMD. A prompt referral for possible surgical revascularization offers the best chance to reduce additional cerebral injuries and improve clinical outcomes.

Outer diameter measured by 3D CISS MRI and quasi-Moyamoya disease

1. The pathology of Moyamoya disease, especially its pathological vessel walls, compared with those of other cerebrovascular diseases like atherosclerosis. One has to observe not only changes of the inner diameter of the pathological vessels but also that of the outer diameter caused by remodeling of vascular wall originating in the pathological process of a disease. Until now, we have paid attention only to the inner diameter, namely luminal stenosis or occlusion on the basis of angiographical or MRI findings. The 3D-CISS MRI has been revealed to be a good tool for measurement of the outer diameter to observe the latter process for instance changes of vascular wall thickness due to its remodeling. 2. The definition of Moyamoya disease and its differentiation from related diseases. The terminology Quasi-Moyamoya disease ( Moyamoya disease in Japanese) could be found, so far as the author has searched around, initially in a chapter of the book Moyamoya disease edited by Prof. J Suzuki (one of finders of this disease and has coined the name of “Moyamoya”) in 1983 [3]. Watanabe T and Suzuki N classified as “rui” Moyamoya disease which was however out of diagnostic criteria of Moyamoya disease, including: a. Only one side corresponds with the criteria of Moyamoya disease (1) but the other side having normal vasculatures. b. Cases with one side or bilateral middle cerebral artery MCA stenosis at the origin associated with Moyamoya vessels, however, combined with neither internal carotid artery ICA nor anterior cerebral artery ACA stenoses c. Combination with congenital malformation: AVM, primitive trigeminal artery d. Associated with apparent basic disease such as: von Recklinghausen disease, head trauma (mechanical vasoconstriction), fibromuscular dysplasia, tuberculous meningitis, atherosclerosis, post-irradiation, Fanconi’s anemia, sickle cell anemia, nonspecific arteritis, connective tissue anomaly, cerebral angiitis due to leptospirosis

Intracerebral Steal Phenomenon

The "intracerebral steal phenomenon (ISP)" is a paradoxical vasodilatory response that reduces the cerebral blood flow to the impaired perfusion area. This phenomenon has been mainly reported in patients with moyamoya vessels with hyperventilation and with the administration of specific drugs such as acetazolamide. We report a case with contralateral infarction that can be explained by ISP after carotid stenting. This report indicates that ISP should be considered as a potential mechanism for contralateral infarction after carotid stent insertion, particularly in the case of contralateral vessel stenosis.

Long-term follow-up results in 142 adult patients with moyamoya disease according to management modality

To clarify the most beneficial treatment of the management modality based on our experience with adult moyamoya disease (MMD). From 1998 to 2010, clinical results of 142 patients (ischemic, 98; hemorrhagic, 44) with adult MMD were investigated according to management modality. Revascularization surgery (direct, indirect, and combined bypass) was performed in 124 patients. We observed the clinical course of 18 patients who were treated conservatively. Clinical outcome, angiographic features, hemodynamic change, and incidence of recurrent stroke were investigated pre- and postoperatively. In patients with ischemic MMD, direct and combined bypasses were more effective treatments to prevent recurrent ischemic stroke than indirect bypass surgery (P < 0.05). In patients with hemorrhagic MMD, rebleeding was less likely to occur in patients who had undergone bypass surgery. However, no significant difference was observed in the rebleeding rate between patients with and without revascularization surgery (P > 0.05). An angiogram after bypass surgery comparing the extent of revascularization and reduction of moyamoya vessels in patients treated with direct, indirect, and combined bypass showed a significant difference (P < 0.05) in favor of direct and combined bypass. Postoperative angiographic changes and SPECT results demonstrated significant statistical correlation (P < 0.05). Revascularization surgery was effective in further ischemic stroke prevention to a statistically significant extent. Direct and combined bypasses were more effective to prevent recurrent ischemic stroke than indirect bypass. However, there is still no clear evidence that revascularization surgery significantly prevents rebleeding in adult MMD patients. More significant angiographic changes were observed in direct and combined bypasses compared with indirect bypass.

Diagnosis of moyamoya disease using 3-T MRI and MRA: value of cisternal moyamoya vessels

The purpose of this study was to propose new magnetic resonance (MR) criteria of diagnosing moyamoya disease (MMD) from cisternal moyamoya vessels (MMVs) on 3-T magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA) and compare the diagnostic accuracy of the existing MR criteria and the proposed MR criteria. Participants comprised 20 consecutive patients with MMD (4 males, 16 females) diagnosed clinically using conventional angiography and 20 controls (13 male and 7 female arteriosclerosis patients). In these participants, 3-T MRI/MRA was evaluated by the existing MR criteria, which use MMVs in the basal ganglia, and the proposed MR criteria, which use cisternal MMVs, and then these two criteria were statistically compared by McNemar's test. Diagnostic accuracy was 62.5% with the existing MR criteria and 97.5% with the proposed MR criteria. The proposed MR criteria was more sensitive (1.00) than the existing MR criteria (0.45), but less specific (0.95) than the existing MR criteria (1.00). The proposed MR criteria using cisternal MMVs showed significantly higher diagnostic accuracy than the existing MR criteria. We believe that our proposed MR criteria will be beneficial for diagnosing MMD.

Discovery of cerebrovascular moyamoya disease: research during the late 1950s and early 1960s

Carotid angiography employing a percutaneous technique, which was devised by Shimizu in 1937, was popularized among neurosurgeons in Japan soon after World War II. This opened up an opportunity for them to encounter and investigate moyamoya disease, since this disease is more common among Asians. They began to publish their findings on what is currently called moyamoya disease in the Japanese literature during the late 1950s and early 1960s. It was only in the late 1960s, however, that their studies were published in the English literature. The early history of the discovery of this disease is therefore not widely known. In 1957, Takeuchi and Shimizu reported a case of an unknown disease characterized by hypoplasia of the internal carotid arteries. In 1964, Moriyasu and his colleagues published a report of four child cases with occlusion of the internal carotid artery. They also published in the same year a report of five adult cases with occlusion of the internal carotid arteries for an unknown reason, placing special emphasis on an abnormal vascular network located at the base of the brain, which is presently termed moyamoya vessels. In 1968 and 1969, Kudo, Nishimoto, and Takeuchi, as well as Suzuki and Takaku, published their studies in the English literature, which contributed greatly to the recognition of moyamoya disease throughout the world. Takeuchi and Shimizu, and Moriyasu also deserve credit as the researchers who reported the crucial features of moyamoya disease for the first time.

010 Moyamoya and AVM: a management dilemma

HistoryA 46 year right handed lady presented with dysphasia and right hemiparesis. Vascular risk factors included smoking and hypertension.InvestigationsAngiography revealed moyamoya compromising flow through the left terminal ICA and MCA...

Disappearance of aneurysms associated with moyamoya disease after STA–MCA anastomosis with encephaloduro myosynangiosis

Moyamoya disease is a rare cerebrovascular disease characterized by steno-occlusive vasculopathy affecting the terminal internal carotid arteries. Although the effect of direct arterial bypass on the prevention of recurrent haemorrhage or ischemic events in patients with hemorrhagic moyamoya disease has been demonstrated, disappearance of aneurysms associated with moyamoya disease has rarely been reported. In this study, we present two patients with aneurysms associated with moyamoya disease. After superficial temporal artery to middle cerebral artery anastomosis combined with encephaloduro myosynangiosis, the aneurysms on the moyamoya vessels disappeared, which was confirmed by follow-up angiography.

Moyamoya Syndrome Associated With Gamma Knife Surgery for Cerebral Arteriovenous Malformation

A 30-year-old female developed moyamoya syndrome after gamma knife surgery (GKS) for cerebral arteriovenous malformation (AVM), and was treated with bypass surgery. She suffered from flittering scotoma, right transient hemianopsia, and headache for 1 year. Cerebral angiography revealed a Spetzler-Martin grade III AVM located in the left occipital lobe. After staged embolization, GKS was performed with a minimum dose of 20 Gy to the periphery of the nidus at the 50% isodose level of the maximum target dose. Gradual nidus regression was achieved, and the clinical symptoms disappeared completely. However, at 30 months after GKS, the patient suffered transient ischemic attack. Cerebral angiography showed left middle cerebral artery occlusion with moyamoya vessels. The patient underwent direct and indirect bypass surgery. After surgery, the patient was free from ischemic symptoms. Chronic inflammation and long-term changes in expression of cytokines and growth factors after GKS may have triggered this case.

もやもや病，片側型もやもや病，類もやもや病に関する全国調査

Moyamoya disease (MMD) is characterized by progressive occlusion of the internal carotid artery or its terminal branches, associated with formation of extensive collateral vessels (moyamoya vessels) at the base of the brain. Whether unilateral moyamoya disease, confirmed by typical angiographic evidence of moyamoya disease unilaterally and normal or equivocal findings contralaterally, is an early form of definite (bilateral) moyamoya disease remains controversial. Inherited or acquired disorders and conditions may present in conjunction with moyamoya disease. This condition is known as quasi-moyamoya disease (quasi-MMD). We attempted to determine the incidence and total patient number of moyamoya disease, unilateral MMD and quasi-MMD, who were treated during 2005 in Japan. Questionnaires were sent to 2,998 departments, which are listed in resident training programs of neurosurgery, neurology and pediatrics. Totally, 1,183 departments replied, and the response rate was 39.5%. The number of annual first-visit patients of MMD, unilateral MMD and quasi-MMD is 571, 118, and 53, respectively. Thus, the number of annual revisit patients of MMD, unilateral MMD and quasi-MMD is 2,064, 214, and 117 respectively. It is estimated that 6,670.9 MMD patient exists in Japan. The incidence rate of MMD, unilateral MMD and quasi-MMD is 1.13, 0.23 and 0.11/100,000, respectively, and the prevalence is 5.22, 0.66 and 0.34/100,000, respectively. This nationwide study revealed the present epidemic status of MMD, unilateral MMD and quasi-MMD.

Treatment of Moyamoya Syndrome Associated with Systemic Lupus Erythematosus and Hypothyroidism in an Adult by Encephaloduroarteriosynangiosis: A Case Report

A 54-year-old woman presented to our hospital with progressive motor weakness of the right arm. She had a medical history of systemic lupus erythematosus (SLE) and hypothyroidism. Magnetic resonance imaging indicated a watershed infarction of the left hemisphere. Cervical echogram indicated severe stenosis of the internal carotid artery (ICA) without wall thickening. Cerebral angiography indicated left ICA occlusion, development of unilateral moyamoya vessels, and leptomeningeal anastomosis. Encephaloduroarteriosynangiosis (EDAS) was performed after cerebral 99 mTechnetium-ethyl-cysteinate-dimer single-photon emission computed tomography indicated a decreased cerebral blood flow, diminished cerebrovascular perfusion reserve. Motor weakness finally disappeared 6 months after surgery. Moyamoya syndrome is a rare complication of both SLE and hypothyroidism, and the surgical indication remains controversial. By evaluating the decreased cerebral perfusion reserve capacity and the existence of leptomeningeal anastomosis, EDAS could be an efficient method for the treatment of moyamoya syndrome associated with SLE and hypothyroidism.

Intracranial Vasculopathy in a Patient with Systemic Sclerosis: Atherosclerotic or Moyamoya-Like Disease?

Intracranial Vasculopathy in a Patient with Systemic Sclerosis: Atherosclerotic or Moyamoya-Like Disease?

Cerebral infarction following intracranial hemorrhage in pediatric Moyamoya disease - A case report and brief review of literature

Moyamoya disease is a clinical entity characterized by progressive cerebrovascular occlusion with spontaneous development of a collateral vascular network called Moyamoya vessels. This disease mainly manifests as cerebral ischemia. Intracranial bleeding is another major presentation of patients with Moyamoya disease. We report here a 12-year-old male child who presented with severe headache, vomiting and meningismus. Initial neuroimaging study with noncontrast computed tomography scan revealed fresh intraventricular hemorrhage in right-sided lateral ventricle. Magnetic resonance imaging with angiography of brain was done 5 days later when the child developed right-sided hemiparesis, and the diagnosis of Moyamoya disease was confirmed along with lacunar infarction of right posterior peri and paraventricular area and in the left paraventricular area and centrum semiovale. Simultaneous presence of cerebral infarction along with intraventricular hemorrhage in adult with bleeding-type Moyamoya disease is reported in literature, but it is a rare entity in a child.