Read by QxMD icon Read

Moyamoya disease and syndrome

Fuat Arikan, Marta Rubiera, Joaquín Serena, Ana Rodríguez-Hernández, Darío Gándara, Carles Lorenzo-Bosquet, Alejandro Tomasello, Ivette Chocrón, Maximiliano Quintana-Corvalan, Juan Sahuquillo
INTRODUCTION: Cerebral revascularization techniques are an indispensable tool in the current armamentarium of vascular neurosurgeons. We present revascularization surgery experience and results in both moyamoya disease and occlusive cerebral ischaemia. PATIENTS AND METHODS: Patients with ischaemic occlusive disease and moyamoya disease who underwent microsurgical revascularization between October 2014 and September 2017 were analysed. RESULTS: In the study period, 23 patients with occlusive ischaemic disease underwent microsurgical revascularization...
March 14, 2018: Neurocirugía
Ken Kazumata, Haruto Uchino, Kikutaro Tokairin, Masaki Ito, Tohru Shiga, Toshiya Osanai, Masahito Kawabori
BACKGROUND: Cerebral hyperperfusion complicates the postoperative course of patients with moyamoya disease (MMD) following direct revascularization surgery. There is no clear distinction between cerebral hyperperfusion syndrome and benign postoperative increase in regional cerebral blood flow (rCBF). OBJECTIVE: The present study aimed to determine clinically relevant changes in rCBF, anatomical correlations, and factors associated with transient neurological symptoms following revascularization surgery in MMD...
March 9, 2018: World Neurosurgery
Brittany L Ko, John H Unkel
Moyamoya disease (MMD) is a rare cerebrovascular disorder involving progressive constriction of the internal carotid artery and its branches. The disease has a particularly aggressive course in very young patients, and early surgical intervention is often necessary to prevent permanent neurological damage. MMD patients have an increased risk of stroke development, which may be provoked by pain or anxiety. Currently, no reports of pediatric patients with MMD exist in the dental literature. The purpose of this paper was to discuss the dental management of a two-year-old with moyamoya disease who presented with early childhood caries and dental fear, offering recommendations for dental providers with emphasis on stroke prevention, collaboration with the medical team, anesthesia considerations for patients with increased stroke risk, and the challenges to maintain the oral health of a patient undergoing complex medical treatment...
January 1, 2018: Pediatric Dentistry
Kimitoshi Sato, Shun Tsudaka, Takanori Miki, Norikata Kobayashi, Taro Yamashita, Kiyoharu Imataka, Takashi Yoshida, Fuminori Shimizu
The concept of "watershed shift"(WS)has been proposed as a cause of the ischemic complications following a superficial temporal artery-middle cerebral artery(STA-MCA)bypass operation performed for the management of moyamoya disease. Previous reports have observed that only 1.2-5.7% of the patients who underwent a bypass operation for the management of moyamoya disease developed cerebral infarction secondary to the WS phenomenon. To date, the WS phenomenon has not been objectively proven on imaging studies...
February 2018: No Shinkei Geka. Neurological Surgery
Shinsuke Muraoka, Yoshio Araki, Goro Kondo, Michihiro Kurimoto, Yoshiki Shiba, Kenji Uda, Shinji Ota, Sho Okamoto, Toshihiko Wakabayashi
OBJECTIVE: Although revascularization surgery for patients with moyamoya disease can effectively prevent ischemic events and thus improve the long-term clinical outcome, the incidence of postoperative ischemic complications affects patients' quality of life. This study aimed to clarify the risk factors associated with postoperative ischemic complications and to discuss the appropriate perioperative management. METHODS: Fifty-eight revascularization operations were performed in 37 children with moyamoya disease...
February 9, 2018: World Neurosurgery
Shinpei Sato, Daigo Kojima, Yasuyoshi Shimada, Jun Yoshida, Kentaro Fujimato, Shunrou Fujiwara, Masakazu Kobayashi, Yoshitaka Kubo, Kenji Yoshida, Kazunori Terasaki, Shouta Tsutsui, Kenya Miyoshi, Kuniaki Ogasawara
The present study examined whether preoperatively reduced cerebrovascular contractile reactivity to hypocapnia by hyperventilation is associated with development of cerebral hyperperfusion syndrome after arterial bypass surgery for adult patients with cerebral misery perfusion due to ischemic moyamoya disease. Among 65 adult patients with ischemic moyamoya disease, 19 had misery perfusion in the precentral region on preoperative15 O positron emission tomography and underwent arterial bypass surgery for that region...
January 1, 2018: Journal of Cerebral Blood Flow and Metabolism
Payam Sasannejad, Fateme Rezaei, Reza Bidaki, Ehsan Zarepur
Moyamoya disease is a chronic progressive vascular disease of brain characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis with prominent arterial collateral circulation. We introduce here a patient with Moyamoya who was misdiagnosed. She was a 16-yr-old female from north east of Iran who complained left hemiparesis and was diagnosed Moyamoya disease by brain and cervical CT-Angiography. There was still great difficulty in the diagnosis of diffuse white matter lesions. The CT-Angiography showed bilateral internal carotid stenosis with "puff of smoke" collateralization arising from the circle of Willis, therefore Moyamoya disease was raised...
2018: Iranian Journal of Child Neurology
Shuran Huang, Lingyun Gao, Yueqin Chen, Xiang Guo, Deguo Liu, Jiehuan Wang, Zhitao Shi, Zhanguo Sun, Feng Jin, Weijian Chen, Yunjun Yang
OBJECTIVE: Vascular and hemodynamic changes were not consistent in symptomatic and non-symptomatic cerebral hemisphere in patients with symptomatic moyamoya syndrome (MMS). Thus, the purpose of this study is to evaluate the hemodynamic difference between symptomatic and non-symptomatic cerebral hemisphere in patients with symptomatic MMS. METHODS: Patients who were diagnosed with symptomatic MMS were retrospectively collected. All cases underwent CTP examination...
January 27, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Natália Battisti Serafini, Cássio Battisti Serafini, Alanna Santoro Vinhas, Marcio Barbosa Godinho
Neurofibromatosis type 1 is a multisystem genetic disease of autosomal dominant transmission that reveals important cutaneous manifestations such as café-au-lait spots, multiple neurofibromas, and ephelides in skin fold areas, as well as hamartomatous lesions in the eyes, bones, glands, and central nervous system. Moyamoya disease is a rare progressive vaso-occlusive disorder that occurs with important ischemic cerebrovascular events. Despite the rarity of this association in childhood, children diagnosed with neurofibromatosis type 1 and focal neurologic symptoms should be investigated for moyamoya syndrome...
November 2017: Anais Brasileiros de Dermatologia
Candice D Carpenter, Luke L Linscott, James L Leach, Sudhakar Vadivelu, Todd Abruzzo
BACKGROUND: Alagille syndrome is a pediatric multisystem disease with increased prevalence of cerebrovascular disease. The spectrum of cerebrovascular disease in Alagille syndrome includes cerebral aneurysms, moyamoya arteriopathy and dolichoectasia. The prevalence of cerebrovascular disease in Alagille syndrome varies widely in the literature. OBJECTIVE: To determine the prevalence of cerebrovascular disease in our institution's Alagille patient population by employing a full primary review of all available neuroimaging...
January 23, 2018: Pediatric Radiology
Jill M Roberts, Michael E Maniskas, Justin F Fraser, Gregory J Bix
Moyamoya is a cerebrovascular disorder characterized by progressive stenosis of the intracranial internal carotid arteries. There are two forms: Disease and Syndrome, with each characterized by the sub-population it affects. Moyamoya syndrome (MMS) is more prominent in adults in their 20's-40's, and is often associated with autoimmune diseases. Currently, there are no surgical models for inducing moyamoya syndrome, so our aim was to develop a new animal model to study this relatively unknown cerebrovascular disease...
2018: PloS One
Mohammad H Abu Arja, Suzanne E Conley, Violeta Salceda, Fahd Al-Sufiani, Daniel R Boué, Jonathan L Finlay
BACKGROUND: Germ cell tumors (GCT) are the most common central nervous system (CNS) tumors in individuals with Down syndrome. Patients with Down syndrome treated with CNS irradiation are at increased risk of developing cerebrovascular complications such as moyamoya disease. Embryonal carcinoma components are recognized to be more resistant to conventional chemotherapy and radiotherapy and confer a very poor prognosis. CD30 is a member of the tumor necrosis factor-receptor superfamily...
April 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Kylie Cooper, Stephen Brown
BACKGROUND: ACTA2 encodes smooth muscle specific α-actin, a critical component or the contractile complex of vascular smooth muscle. Mutations in ACTA2 are the most common genetic cause of thoracic aortic aneurysm, and are also the cause of other disorders, including Moyamoya disease, coronary artery disease and stroke as well as Multisystemic Smooth Muscle Dysfunction Syndrome. We note that ACTA2 is also expressed in uterine smooth muscle, and this raises the possibility that women harboring ACTA2 mutations might exhibit uterine smooth muscle dysfunction...
December 4, 2017: BMC Medical Genetics
Catherine Y Wang, Stephen L Grupke, Jill Roberts, Jessica Lee, Justin F Fraser
OBJECTIVES: Our study aimed to report both new and previously identified conditions associated with moyamoya syndrome in a Western population and to present our outcomes after surgical treatment with indirect bypass. METHODS: We performed a retrospective chart review of patients evaluated at our institution from June 2011 to June 2015 who were diagnosed with moyamoya. Data collected include patient demographics, presenting manifestations, vessels involved, comorbid conditions, abnormal laboratory values, treatments administered, and clinical outcomes...
November 17, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
Junko Yamanaka, Ikuma Nozaki, Mizue Tanaka, Hideko Uryuu, Noriko Sato, Takeji Matsushita, Hiroyuki Shichino
In the era of Antiretroviral Therapy (ART) in which human immunodeficiency virus type 1 (HIV-1) infection affected children can expect a better prognosis, the importance of careful follow up of pediatric HIV-1 cases for neurological complications has been growing. We present a case of hemorrhagic Moyamoya syndrome in a child with congenital HIV-1 infection. A 10-year-old girl was referred to our hospital for the treatment of Pneumocystis Jirovecii Pneumonia (PCP: Pneumocystis pneumonia). Her HIV-1 control was poor and Moyamoya syndrome was found during the opportunistic infection screening at admission...
November 11, 2017: Journal of Infection and Chemotherapy: Official Journal of the Japan Society of Chemotherapy
(no author information available yet)
No abstract text is available yet for this article.
October 12, 2017: Journal of Neurosurgical Anesthesiology
James Murchison, John M Wilson, Coby Ray, Jessica Ginsberg, Laszlo Nagy
BACKGROUND Cerebral hyperperfusion syndrome is a rare complication of indirect revascularization due to moyamoya disease, but has not been reported previously in the pediatric population. We present a case of an 18-month-old girl with moyamoya disease that was treated with bilateral pial synangiosis and had complications consistent with cerebral hyperperfusion syndrome. This case report discusses the pathophysiological mechanisms involved in cerebral hyperperfusion in moyamoya syndrome. CASE REPORT An 18-month-old female Caucasian presented with seizures and weakness of the left side...
October 9, 2017: American Journal of Case Reports
Julie G Shulman, Samuel Snider, Henri Vaitkevicius, Viken L Babikian, Nirav J Patel
BACKGROUND: Hemodynamic insufficiency is often considered the cause of ischemic stroke in patients with moyamoya syndrome. While high-intensity transient signals (HITS) on transcranial Doppler (TCD) have been reported in this population, the relationship between these signals and ischemic symptoms is not clearly established. Accordingly, current treatment is directed at improving perfusion. CLINICAL PRESENTATION: We present two patients with symptoms of cerebral ischemia and angiographic findings of moyamoya syndrome...
2017: Frontiers in Neurology
Sung-A Chang, Ju Sun Song, Taek Kyu Park, Jeong Hoon Yang, Woo Chan Kwon, So Ree Kim, Sung Mok Kim, Jihoon Cha, Shin Yi Jang, Young Seok Cho, Tae Jung Kim, Oh Young Bang, Jin Young Song, Chang-Seok Ki, Duk-Kyung Kim
BACKGROUND: Peripheral pulmonary arterial stenosis (PPAS) in childhood is frequently associated with other syndromes; however, PPAS in adolescents and adults is rare and its etiology is not well understood. We report the clinical characteristics of adult-onset nonsyndromic PPAS associated with the p.Arg4810Lys variant of the RNF213 gene. METHODS: We recently encountered an index case of severe pulmonary hypertension with multiple PPAS and intra- and extracranial arteriopathy...
February 2018: Chest
Yannick Béjot, José-Luis Barnay, Adrien Chavent, Benoit Daubail, Agnès Jacquin, Apolline Kazemi, Frédéric Ricolfi, Maurice Giroud
INTRODUCTION: Moyamoya syndrome is a rare progressive cerebrovascular occlusive disease for which several associated conditions have been described. CASE REPORT: We report the case of a 76-year-old woman with a history of May-Hegglin anomaly who presented with an isolated unusual diffuse headache. Initial laboratory investigations showed only thrombocytopenia (platelet count 95000/μL). Unenhanced brain computed tomography scan revealed a small subarachnoid hemorrhage in the left frontal lobe...
September 2017: Neurologist
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"