Ultrastructural studies of cerebral arteries and collateral vessels in moyamoya disease.

Moyamoya disease is a disease in which certain arteries in the brain are constricted. Blood flow is blocked by the constriction, and also by blood clots (thrombosis). A collateral circulation develops around the blocked vessels to compensate for the blockage, but the collateral vessels are small, weak, and prone to bleeding, aneurysm and thrombosis which may result in TIA, recurrent ischemic or hemorrhagic stroke or seizure. The disease may manifest in pediatric age or young adults. In May 2019 we have diagnosed a young lady with Moyamoya disease who presented with right sided hemiplegia, motor aphasia and dysphagia. She was labeled as hypertensive 6 months prior to this event and used to take anti-hypertensive irregularly and gave past history of occasional headache. Her CT scan and MRI of brain revealed left sided ischemic infarct involving frontotemporoparietal region and cerebral angiogram revealed narrowing of left MCA and non-visualization of distal part. There is extensive fine collaterals (Moyamoya vessels) giving the appearance of puffed smoke. The right ACA and MCA were also narrowed with appearance of early collateral vessels. She was treated with aspirin, PPI, NG feeding, antihypertensive medication, physiotherapy, rehabilitation therapy and other supportive care. His condition gradually improved and discharged on 2.7.19. He was referred to Department of Neurosurgery for cerebral revascularization by STA-MCA (superficial temporal and middle cerebral arteries) bypass surgery after stabilization and MR perfusion study. Journal of Armed Forces Medical College Bangladesh Vol.15 (1) 2019: 110-113

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Moyamoya Disease

Pediatric Neurosurgery ◽

10.1093/med/9780190617073.003.0017 ◽

2019 ◽

pp. 149-156

Author(s):

Edward Smith

Keyword(s):

Moyamoya Disease ◽

Cerebral Arteries ◽

Pressure Control ◽

Vascular Supply ◽

External Carotid Artery ◽

External Carotid ◽

Middle Cerebral Arteries ◽

Detailed Assessment ◽

Collateral Vessels ◽

Carotid Circulation

Moyamoya disease is defined by stenosis of the distal intracranial internal carotid arteries up to and including the bifurcation, with segments of the proximal anterior and middle cerebral arteries, dilated basal collateral vessels, and bilateral findings. Detailed assessment with digital subtraction angiography will define the severity of disease (Suzuki stage) and presence of spontaneous transdural collateral vessels from external carotid artery branches. These collateral vessels must be protected during surgical intervention. The surgical goal is to establish a new vascular supply to the areas of the brain at risk for stroke, utilizing vessels from the external carotid circulation. Blood pressure control and avoidance of hyperventilation are key to minimize perioperative stroke risk. Preoperative hydration, ongoing use of aspirin, and good pain control will also minimize surgical complications.

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Acceleration-selective Arterial Spin-labeling MR Angiography Used to Visualize Distal Cerebral Arteries and Collateral Vessels in Moyamoya Disease

Radiology ◽

10.1148/radiol.2017162279 ◽

2018 ◽

Vol 286 (2) ◽

pp. 611-621 ◽

Cited By ~ 8

Author(s):

Osamu Togao ◽

Akio Hiwatashi ◽

Makoto Obara ◽

Koji Yamashita ◽

Kazufumi Kikuchi ◽

...

Keyword(s):

Moyamoya Disease ◽

Arterial Spin Labeling ◽

Mr Angiography ◽

Cerebral Arteries ◽

Spin Labeling ◽

Collateral Vessels

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Cerebral revascularization using omental transplantation for childhood moyamoya disease

Journal of Neurosurgery ◽

10.3171/jns.1993.79.2.0192 ◽

1993 ◽

Vol 79 (2) ◽

pp. 192-196 ◽

Cited By ~ 81

Author(s):

Jun Karasawa ◽

Hajime Touho ◽

Hideyuki Ohnishi ◽

Susumu Miyamoto ◽

Haruhiko Kikuchi

Keyword(s):

Moyamoya Disease ◽

Cerebral Artery ◽

Anterior Cerebral Artery ◽

Posterior Cerebral Artery ◽

Cerebral Arteries ◽

Neurological Deficits ◽

Content Type ◽

Collateral Vessels ◽

Neurological State ◽

Fine Print

✓ Between January, 1986, and October, 1990, 30 children with moyamoya disease, aged from 2 to 17 years, underwent omental transplantation to either the anterior or the posterior cerebral artery territory. The mean follow-up period was 3.8 years, ranging from 1.6 to 6.4 years. Seventeen patients had symptoms of monoparesis, paraparesis, and/or urinary incontinence and were treated using unilateral or bilateral omental transplantation to the anterior cerebral artery territory. Eleven patients had visual symptoms and were treated with unilateral or bilateral omental transplantation to the posterior cerebral artery territory. Two patients had symptoms associated with both the anterior and the posterior cerebral arteries, and were treated with dual omental transplantations. All 19 patients treated with omental transplantation to the anterior cerebral artery and 11 (84.6%) of the 13 treated with omental transplantation to the posterior cerebral artery showed improvement in their neurological state. Patients with more collateral vessels via the omentum had more rapid and complete improvement in their neurological state. Patients with severe preoperative neurological deficits associated with the posterior cerebral artery had persistence of their symptoms.

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Abstract 2786: Moyamoya Syndrome Associated With Graves' Disease: Report Of 16 Cases

Stroke ◽

10.1161/str.43.suppl_1.a2786 ◽

2012 ◽

Vol 43 (suppl_1) ◽

Author(s):

Jun NI ◽

Li-xin ZHOU ◽

Yan-ping WEI ◽

Lian DUAN ◽

Shan GAO ◽

...

Keyword(s):

Moyamoya Disease ◽

Cerebral Arteries ◽

Laboratory Data ◽

Thyroid Stimulating Hormone ◽

Graves Disease ◽

Thyroid Function Tests ◽

Moyamoya Syndrome ◽

College Hospital ◽

Ischemic Attack ◽

Collateral Vessels

Background and purpose Moyamoya disease is a cerebrovascular disorder characterized by bilateral progressive stenosis and occlusion of terminal portions of internal carotid artery (ICA) accompanying by typical net-like collateral vessels. Patients with both the characteristic moyamoya vasculopathy and the associated conditions are categorized as moyamoya syndrome. These conditions include sickle cell disease, neurofibromatosis type 1, Down’s disease, cranial therapeutic irradiation and other rare diseases. Moyamoya syndrome associated with Graves’ disease has been rarely reported and the underlying coexisting mechanism remains unclear. The aim of this study is to identify the clinical and radiological findings of the patients with simultaneous diagnosis of moyamoya syndrome and Graves’ disease. Possible mechanisms predisposing these individuals to ischemic accidents are discussed. Methods We retrospectively identified 12 patients in Peking Union Medical College Hospital and 4 patients in 307 hospital of PLA between May 2000 and December 2010. All patients were female and mean age (range) was 35.13±12.34 years (11-57 years). The patients were diagnosed with Graves’ disease in endocrinology clinic which meet the full diagnostic criteria and no atherosclerotic factor was found in them. Moyamoya vasculopathy was definitely or probably diagnosed by digital subtract angiography or magnetic resonance angiography. The clinical characteristics and prognosis, laboratory data, vascular radiological characteristics were all collected. Results Stenosis or occlusion of bilateral distal ICA and/or proximal anterior /middle cerebral arteries was found in 13 patients. Three patients had unilateral distal ICA stenosis and abnormal collateral vessels. PCA stenosis was found in 2 patients. Fifteen patients presented with infarction or transient ischemic attack and one with dizziness. Interestingly, thyroid function tests demonstrated predominantly elevated thyroxine and suppressed thyroid stimulating hormone level in 15 patients when cerebrovascular accidents occurred. All patients received antithyroid therapy and two had recurrent ischemic attack after several months of drug withdrawal. Four patients underwent revascularization procedures after normalization of their hormonal conditions in 307 Hospital of PLA and their neurologic status has remained stable during follow-up. Conclusions: Compared with classical moyamoya disease, moyamoya syndrome associated Graves’ disease more commonly presented asymmetric stenosis or occlusion and PCA lesions. Cerebrovascular hemodynamic changes attributable to thyrotoxicosis might be responsible for ischemic attack and further studies are required to verify this hypothesis.

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Effects of Surgical Revascularization on Peripheral Artery Aneurysms in Moyamoya Disease: Report of Three Cases

Neurosurgery ◽

10.1097/00006123-200108000-00039 ◽

2001 ◽

Vol 49 (2) ◽

pp. 463-468 ◽

Cited By ~ 14

Author(s):

Satoshi Kuroda ◽

Kiyohiro Houkin ◽

Hiroyasu Kamiyama ◽

Hiroshi Abe

Keyword(s):

Moyamoya Disease ◽

Superficial Temporal Artery ◽

Temporal Artery ◽

Intracranial Bleeding ◽

Peripheral Artery ◽

Surgical Revascularization ◽

Beneficial Effects ◽

Collateral Arteries ◽

Lenticulostriate Artery ◽

Collateral Vessels

Abstract OBJECTIVE AND IMPORTANCE The beneficial effects of surgical revascularization on rebleeding in moyamoya disease remain unclear. This report is intended to clarify the effects of surgical revascularization on peripheral artery aneurysms, which represent one of the causes of intracranial bleeding in moyamoya disease. CLINICAL PRESENTATION Findings for three female patients who experienced intracranial bleeding are presented. Cerebral angiography revealed that intracranial bleeding resulted from the rupture of peripheral artery aneurysms arising from dilated collateral vessels such as the lenticulostriate artery. INTERVENTION The patients successfully underwent superficial temporal artery-middle cerebral artery anastomosis combined with encephaloduromyoarteriosynangiosis. Angiography demonstrated obliteration of the peripheral artery aneurysms, together with the disappearance or decrease in caliber of the parent collateral arteries, after surgery. None of the patients experienced rebleeding during the follow-up period (up to 52 mo). CONCLUSION The results strongly suggest that surgical revascularization potentially improves cerebral circulation and decreases hemodynamic stress on collateral vessels, obliterating peripheral artery aneurysms.

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Atypical Posterior Reversible Encephalopathy Syndrome in a Postpartum Woman With Moyamoya Disease: A Case Report and Literature Review

Frontiers in Neurology ◽

10.3389/fneur.2021.696056 ◽

2021 ◽

Vol 12 ◽

Author(s):

Ning Zou ◽

Guixiang Guo ◽

Fangchao Wan ◽

Xin Li

Keyword(s):

Glasgow Coma Scale ◽

Moyamoya Disease ◽

Posterior Reversible Encephalopathy Syndrome ◽

Reversible Posterior Leukoencephalopathy Syndrome ◽

Postpartum Woman ◽

Posterior Reversible Encephalopathy ◽

Coma Scale ◽

Posterior Leukoencephalopathy ◽

Collateral Vessels ◽

Reversible Encephalopathy

Background: Moyamoya disease is a rare cerebrovascular occlusive disease, which is characterized by stenosis and gradual occlusion of the internal carotid arteries, causing the progression of characteristic collateral vessels. To date, most studies investigating moyamoya disease have focused on medical implications, and the potential implications for neurocognitive and/or neuropsychiatric functioning were inconclusive.Case Presentation: we present a case of a 26-year-old Chinese postpartum woman who presented to the emergency department with a 19-h history of cognitive decline, vomiting, and convulsions. Blood pressure, heart rate, and respiration rate were 200/120 mmHg, 115 beats/minute, and 30 breaths/minute, respectively, on arrival. The Glasgow Coma Scale, modified RANKIN scale (mRS), and National Institute of Health stroke scale (NIHSS) scores were 3, 5, and 18, respectively. Moyamoya disease was diagnosed using cerebral angiography and digital subtraction angiography. The cognitive functions of orientation, use of language, ability to calculate, and memory significantly improved after 11 days of treatment (Glasgow Coma Scale: 15; mRS: 0; NIHSS: 0).Conclusions:This patient was diagnosed with reversible posterior leukoencephalopathy syndrome related to moyamoya disease. This case highlights that atypical posterior reversible encephalopathy syndrome can occur in patients with moyamoya disease, and should be considered for the differential diagnosis of cerebral infarcts and hemorrhage in a postpartum female.

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Experimental animal models for moyamoya disease and treatment: a pathogenesis-oriented scoping review

Neurosurgical FOCUS ◽

10.3171/2021.6.focus21284 ◽

2021 ◽

Vol 51 (3) ◽

pp. E5

Author(s):

Michael S. Rallo ◽

Omar Akel ◽

Akhilesh Gurram ◽

Hai Sun

Keyword(s):

Animal Models ◽

Experimental Animal ◽

Moyamoya Disease ◽

Scoping Review ◽

Carotid Arteries ◽

Data Extraction ◽

Cerebral Arteries ◽

Susceptibility Gene ◽

Cerebral Hypoperfusion ◽

Experimental Animal Models

OBJECTIVE Moyamoya disease (MMD) is an intracranial steno-occlusive pathology characterized by progressive narrowing of proximal large vessels, including the terminal internal carotid arteries (ICAs), middle cerebral arteries, or anterior cerebral arteries. Named for the “puff of smoke” appearance of the anomalous vascularization visualized on cerebral angiography, MMD lacks a well-defined etiology, although significant insights have been made, including the identification of a susceptibility gene, RNF213, in humans with the disease. A limitation to advancing the understanding and treatment of MMD has been the lack of experimental animal models that authentically reflect the clinical pathogenesis. In an effort to analyze characteristics of currently available models and identify strategies for future model generation, the authors performed a scoping review of experimental animal models that have been used to study MMD. METHODS A systematic search of PubMed, Web of Science, and Scopus was performed to identify articles describing animal models used to study MMD. Additional articles were identified via citation searching. Study selection and data extraction were performed by two independent reviewers based on defined inclusion and exclusion criteria. RESULTS A total of 44 articles were included for full-text review. The methods used to generate these animal models were broadly classified as surgical (n = 25, 56.8%), immunological (n = 7, 15.9%), genetic (n = 6, 13.6%), or a combination (n = 6, 13.6%). Surgical models typically involved permanent ligation of one or both of the common carotid arteries or ICAs to produce chronic cerebral hypoperfusion. Genetic models utilized known MMD or cerebrovascular disease-related genes, such as RNF213 or ACTA2, to induce heritable cerebral vasculopathy. Finally, immunological models attempted to induce vasculitis-type pathology by recapitulating the inflammatory milieu thought to underlie MMD. CONCLUSIONS Models generated for MMD have involved three general approaches: surgical, immunological, and genetic. Although each reflects a key aspect of MMD pathogenesis, the failure of any individual model to recapitulate the development, progression, and consequences of the disease underscores the importance of future work in developing a multietiology model.

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Unilateral sudden hearing loss as the first presenting symptom of moyamoya disease

The Journal of Laryngology & Otology ◽

10.1017/s002221511200309x ◽

2013 ◽

Vol 127 (2) ◽

pp. 196-199 ◽

Cited By ~ 1

Author(s):

L-S Tseng ◽

S-D Luo

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Moyamoya Disease ◽

Carotid Arteries ◽

Internal Carotid ◽

Cerebral Arteries ◽

Sudden Onset ◽

Sudden Sensorineural Hearing Loss ◽

Sensorineural Hearing ◽

Internal Carotid Arteries

AbstractObjective:We describe a rare case of sudden onset of unilateral sensorineural hearing loss occurring as the first symptom of moyamoya disease, which is characterised by progressive stenosis of the intracranial internal carotid arteries and their proximal anterior cerebral arteries and middle cerebral arteries.Method:Case report and review of the world literature regarding moyamoya disease with hearing loss.Results:The reported patient had moyamoya disease that initially presented as sudden, unilateral sensorineural hearing loss. Magnetic resonance imaging showed occlusion of the anterior cerebral, middle cerebral and distal internal carotid arteries bilaterally. The possible mechanism of this patient's sudden sensorineural hearing loss may have been vascular occlusion resulting from thrombotic narrowing or blockage by plaque.Conclusion:The described patient represents the first reported case of sudden onset, unilateral sensorineural hearing loss occurring as the first symptom of moyamoya disease. The possibility of a vascular lesion such as moyamoya disease should be considered in patients with sudden sensorineural hearing loss, especially children, young adults and Asian patients. Due to this disease's poor outcome, early diagnosis and treatment are important to prevent stroke.

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Moyamoya in Hispanics: not only in Japanese

Neurology International ◽

10.4081/ni.2014.5369 ◽

2014 ◽

Vol 6 (2) ◽

Cited By ~ 2

Author(s):

Sarmad Said ◽

Chad J. Cooper ◽

Haider Alkhateeb ◽

Juan M. Galvis ◽

German T. Hernandez ◽

...

Keyword(s):

Cigarette Smoke ◽

Moyamoya Disease ◽

Carotid Arteries ◽

Internal Carotid ◽

Prolonged Survival ◽

Moyamoya Syndrome ◽

Characteristic Appearance ◽

Hispanic Patients ◽

Collateral Vessels ◽

Internal Carotid Arteries

Moyamoya disease was first described in 1957 as <em>hypoplasia of the bilateral internal carotid arteries</em>, the characteristic appearance of the associated network of abnormally dilated collateral vessels on angiography was later likened to something hazy, like a puff of cigarette smoke, which, in Japanese, is <em>moyamoya</em>. This paper describes two cases of moyamoya presentations, including moyamoya disease and moyamoya syndrome. Moyamoya may rarely occur in North American Hispanic patients. The presentation can vary significantly and ranges bwtween fulminant outcome and prolonged survival. Awareness about moyamoya and its different presentations may be beneficial for the patients and can improve the outcome.

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