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Moyamoya disease

Robert C Rennert, Kristine Ravina, Ben A Strickland, Joshua Bakhsheshian, Joseph Carey, Jonathan J Russin
BACKGROUND: Surgical innovation is critical for the management of challenging cerebrovascular pathology. Flow-through free flaps are versatile composite grafts that combine viable tissue with a revascularization source. Neurosurgical experience with these flaps is limited. OBJECTIVE: To provide an in-depth technical description of the radial artery fascial (and fasciocutaneous) flow-through free flap (RAFF and RAFCF, respectively) for complex cerebral revascularizations...
June 16, 2018: Operative Neurosurgery (Hagerstown, Md.)
Georgios A Maragkos, Luis C Ascanio, Kohei Chida, Myles D Boone, Christopher S Ogilvy, Ajith J Thomas, Ekkehard M Kasper
BACKGROUND: Moyamoya disease (MMD) management during pregnancy poses a challenge to health care providers, and recommendations are outdated, vague, and controversial. We conducted a systematic review to investigate and present the available evidence. METHOD: We searched five online databases and bibliographies of relevant published original studies to identify case reports, case series, cohort studies, and reviews reporting on patients diagnosed with MMD before, during, or shortly after pregnancy...
June 19, 2018: Acta Neurochirurgica
Jian Wang, Gong Chen, Yongbo Yang, Bing Zhang, Zhongzhi Jia, Peiyuan Gu, Dong Wei, Jing Ji, Weixing Hu, Xihai Zhao
OBJECTIVE: To assess the association between champagne bottle neck sign (CBNS) in carotid artery and intracranial hemorrhage in patients with moyamoya disease (MMD). METHODS: From January 2016 to December 2017, a total of 76 consecutive patients with MMD without definite risk factors associated intracranial hemorrhage who underwent preoperative angiography were included in this retrospective study. CBNS was defined as luminal diameter of internal carotid artery (ICA)/common carotid artery (CCA) ≤ 0...
June 15, 2018: World Neurosurgery
Shuling Shang, Da Zhou, Jingyuan Ya, Sijie Li, Qi Yang, Yuchuan Ding, Xunming Ji, Ran Meng
Moyamoya disease is characterized by progressive stenosis or occlusion of the intracranial portion of the internal carotid artery and their proximal branches, resulting in ischemic or hemorrhagic stroke with high rate of disability and even death. So far, available treatment strategies are quite limited, and novel intervention method is being explored. This review encapsulates current advances of moyamoya disease on the aspects of epidemiology, etiology, clinical features, imaging diagnosis and treatment. In addition, we also bring forward our conjecture, which needs to be testified by future research...
June 18, 2018: Neurosurgical Review
Abdelhafid Houba, Nisrine Laaribi, Mohammed Meziane, Abdelhamid Jaafari, Khalil Abouelalaa, Mustapha Bensghir
BACKGROUND: A stroke in a baby is uncommon, recent studies suggested that their incidence is rising. Moyamoya disease is one of the leading causes of stroke in babies. This condition is mostly described in Japan. In Morocco, moyamoya disease has rarely been reported and a few cases were published. We report a rare Moroccan case of a 23-month-old baby boy who presented with left-sided hemiparesis and was diagnosed as having moyamoya disease. CASE PRESENTATION: A 23-month-old full-term Moroccan baby boy born to a non-consanguineous couple was referred to our hospital with the complaint of sudden onset left-sided hemiparesis...
June 13, 2018: Journal of Medical Case Reports
Satoshi Hori, Daina Kashiwazaki, Shusuke Yamamoto, Güliz Acker, Marcus Czabanka, Naoki Akioka, Naoya Kuwayama, Peter Vajkoczy, Satoshi Kuroda
BACKGROUND: Fragile, dilated moyamoya vessels are the main source of hemorrhagic stroke in moyamoya disease (MMD). However, the prevalence of hemorrhagic stroke largely differs between Asian and western countries, although the underlying pathophysiology has not been clarified. OBJECTIVE: To systematically analyze the difference of collateral angioarchitectures between Japanese and European Caucasians with MMD. METHODS: This study included 71 patients with MMD, including Japanese (n = 41) and European Caucasians (n = 30)...
June 8, 2018: Neurosurgery
Hesamoddin Jahanian, Thomas Christen, Michael E Moseley, Greg Zaharchuk
In resting-state fMRI (rs-fMRI), functional networks are assessed utilizing the temporal correlation between spontaneous Blood Oxygen Level Dependent (BOLD) signal fluctuations of spatially remote brain regions. Recently, several groups have shown that temporal shifts are present in rs-fMRI maps in patients with cerebrovascular disease due to spatial differences in arterial arrival times, and that this can be exploited to map arrival times in the brain. This suggests that rs-fMRI connectivity mapping may be similarly sensitive to such temporal shifts, and that standard rs-fMRI analysis methods may fail to identify functional connectivity networks...
June 11, 2018: Brain Connectivity
Constantin Roder, Eva Bürkle, Florian Heinrich Ebner, Marcos Tatagiba, Ulrike Ernemann, Alfred Buck, Philipp T Meyer, Nadia Khan
BACKGROUND: and Purpose: Moyamoya disease is a stenoocclusive disease of the circle of Willis with growth of pathological collaterals. We studied the indispensability of functional perfusion imaging (H2 15 O-PET with Acetazolamide (AZA) challenge) compared to conventional MRI and angiography for indication of cerebral revascularization of Moyamoya patients. METHODS: We performed a retrospective blinded analysis of individual imaging modalities (MRI, angiogram, PET) and scored each modality for the severity of disease in 21 untreated Moyamoya patients with 78 affected vascular territories...
June 7, 2018: World Neurosurgery
Fatma Laatar, Imen Kacem, Amira Nasri, Mouna Ben Djebara, Amina Gargouri, Riadh Gouider
No abstract text is available yet for this article.
December 2017: La Tunisie Médicale
S Barrit
SAMHD1 is one of seven known genes responsible for Aicardi-Goutières syndrome. It has the particularity to associate to this rare pediatric encephalopathy with autoimmune manifestations, a cerebral vasculopathy type Moyamoya. This condition has only been recently reported, less than fifty times in the literature. Our clinical case is a 11 year old boy from an inbred union whose clinical diagnosis confirmed genetically and followed by a review of current data determined an ad hoc management, presently described...
May 30, 2018: Revue Médicale de Bruxelles
Shinsuke Muraoka, Yoshio Araki, Toshiaki Taoka, Hisashi Kawai, Sho Okamoto, Kenji Uda, Shinji Ota, Shinji Naganawa, Toshihiko Wakabayashi
OBJECT: Moyamoya vasculopathy (MMV) is characterized by progressive stenosis of the intracranial arteries. MMV currently has no curative treatments, and cerebral ischemia and hemorrhage are the major outcomes. Evaluation of the stroke risk of each patient due to the progression of intracranial arterial stenosis is clinically important. METHODS: We prospectively reviewed patients with intracranial arterial stenosis and already-diagnosed MMV. High-resolution magnetic resonance imaging (HR-MRI) using contrast agent is the novel vessel wall imaging (VWI) technique for directly evaluating vascular walls and intracranial artery disease...
June 1, 2018: World Neurosurgery
Hanqiang Jiang, Heng Yang, Wei Ni, Yu Lei, Jiabin Su, Yuxiang Gu, Bin Xu, Ying Mao
OBJECTIVE: Risk factors for rebleeding after revascularization surgery was unclear. We aimed to evaluate long-term outcomes after combined revascularization surgery for adult hemorrhagic moyamoya disease (MMD) and identify risk factors for initial and recurrent hemorrhage. METHODS: 105 adult patients with hemorrhagic MMD from January 2007 to May 2011 were prospectively enrolled in this study. All patients underwent combined revascularization surgery on unilateral hemorrhagic hemispheres and were observed for at least 5 years...
May 30, 2018: World Neurosurgery
Kenya Miyoshi, Kohei Chida, Masakazu Kobayashi, Yoshitaka Kubo, Kenji Yoshida, Kazunori Terasaki, Kuniaki Ogasawara
BACKGROUND: In nonsurgical adult moyamoya disease (MMD) patients with ischemic onset and stable hemodynamics, the cerebral hemodynamic and cognitive course remains unclear. OBJECTIVE: To clarify, through a prospective cohort study, 2-yr clinical, cerebral hemodynamic, and cognitive outcomes of adult patients receiving medication alone for symptomatically ischemic MMD without cerebral misery perfusion. METHODS: Seventy patients without cerebral misery perfusion on the first 15O gas positron emission tomography (PET) were clinically followed up for 2 yr...
May 30, 2018: Neurosurgery
Si Un Lee, Chang Wan Oh, O-Ki Kwon, Jae Seung Bang, Seung Pil Ban, Hyoung Soo Byoun, Tackeun Kim
PURPOSE OF REVIEW: Moyamoya disease (MMD) is being increasingly diagnosed with the development of radiological surveillance technology and increased accessibility to medical care. Accordingly, there have been several recent reports on treatment outcomes in MMD. In this review, we summarize recent advances in surgical treatment and outcomes of adult MMD, while addressing related controversies. RECENT FINDINGS: Recent studies suggest that revascularization surgery leads to significantly more favorable outcomes for stroke prevention, angiographic and hemodynamic changes, and clinical outcomes than does conservative treatment for adult patients with ischemic MMD...
May 28, 2018: Current Treatment Options in Neurology
Eric J Arias, Brent Bruck, Ananth K Vellimana, Charles Eby, Matthew R Reynolds, Morey A Blinder, Gregory J Zipfel
BACKGROUND AND IMPORTANCE: Antiphospholipid syndrome (APS) is an autoimmune disorder associated with a hypercoagulable state and increased risk of intraoperative and postoperative thrombosis. Few neurosurgical studies have examined the management of these patients, though the standard of care in most other disciplines involves the use of anticoagulation therapy. However, this is associated with risks such as hemorrhage, thrombosis due to warfarin withdrawal, and is not compatible with operative intervention...
May 24, 2018: Operative Neurosurgery (Hagerstown, Md.)
Marcello Scala, Pietro Fiaschi, Valeria Capra, Maria Luisa Garrè, Domenico Tortora, Marcello Ravegnani, Marco Pavanello
BACKGROUND: Moyamoya disease (MMD) is a cerebrovascular disorder characterized by the progressive occlusion of the supraclinoid internal carotid artery (ICA), resulting in the formation of an abnormal cerebral vascular network. When MMD occurs in association with an underlying medical condition, including some distinctive genetic disorders, it is named moyamoya syndrome (MMS). The discrimination between MMD and MMS has been validated by recent genetic researches and international reviews...
May 24, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Saya Ozaki, Shiro Ohue, Shinji Iwata, Shinya Fukumoto, Haruhisa Ichikawa, Shinji Onoue, Akihiro Inoue, Yawara Nakamura, Kanehisa Kohno
Postoperative neurological deficits frequently occur in adult moyamoya disease. In this case report, we describe the time course and disease state of a patient with adult moyamoya disease, who experienced a postoperative neurological deficit due to transient hypoperfusion in the pretreated contralateral hemisphere. A 68-year-old female presented with a sudden onset of left hemianopia due to ischemic moyamoya disease. She had severely low cerebral blood flow(CBF)in the right hemisphere and deterioration of cerebrovascular reactivity in the left hemisphere...
May 2018: No Shinkei Geka. Neurological Surgery
Masaki Koh, Takuya Akai, Daina Kashiwazaki, Naoki Akioka, Takahiro Tomita, Shoichi Nagai, Satoshi Kuroda
PURPOSE: Ultrasound-aided fixation of a biodegradable cranial fixation system called SonicWeld Rx<sup>®</sup>, has been widely applied in the fields of craniofacial surgery and plastic surgery. However, there are few reports that denote its use in the field of neurosurgery. This study aimed to evaluate the usefulness of SonicWeld Rx<sup>®</sup> system in pediatric neurosurgery. METHODS: This study included 11 pediatric patients(10 boys, 1 girl), who underwent craniotomy for moyamoya disease, brain tumor, and arachnoid cyst...
May 2018: No Shinkei Geka. Neurological Surgery
David L Penn, Kyle C Wu, Kayla R Presswood, Coleman P Riordan, R Michael Scott, Edward R Smith
Pial synangiosis is a method of indirect surgical revascularization developed at our institution for the treatment of moyamoya disease in pediatric patients. Similar surgical principles are employed in adult cases, often performed because of lack of an adequate donor vessel. Standardized protocols, including preadmission for preoperative intravenous hydration and aspirin administration, as well as intraoperative electroencephalography, are routinely employed to minimize operative risk. Perioperative heparinization is not required...
May 18, 2018: Operative Neurosurgery (Hagerstown, Md.)
Takeshi Funaki, Jun C Takahashi, Susumu Miyamoto
In this article, the authors review the literature related to long-term outcome of pediatric moyamoya disease, focusing on late cerebrovascular events and social outcome of pediatric patients once they reach adulthood. Late-onset de novo hemorrhage is rare but more serious than recurrence of ischemic stroke. Long-term follow-up data on Asian populations suggest that the incidence of de novo hemorrhage might increase at age 20 or later, even more than 10 years after bypass surgery. Social adaptation difficulty, possibly related to cognitive impairment caused by frontal ischemia, continues in 10-20% of patients after they reach adulthood, even if no significant disability is present in daily life...
May 21, 2018: Neurologia Medico-chirurgica
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