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Moyamoya and smith

Katie Pricola Fehnel, Micah Duggins-Warf, David Zurakowski, Maxwell McKee-Proctor, Rajarshi Majumder, Michael Raber, Xuezhe Han, Edward R Smith
OBJECTIVE The authors report the use of urinary biomarkers as a novel, noninvasive technique to detect juvenile pilocytic astrocytomas (JPAs), capable of distinguishing JPAs from other CNS diseases, including other brain tumors. Preliminary screening of an array of tumors implicated proteases (including matrix metalloproteinases [MMPs]) and their inhibitors (tissue inhibitors of metalloproteinase [TIMPs]) as well as growth factors (including basic fibroblast growth factor [bFGF]) as candidate biomarkers. These data led the authors to hypothesize that tissue inhibitor of metalloproteinase 3 (TIMP3) and bFGF would represent high-probability candidates as JPA-specific biomarkers...
June 17, 2016: Journal of Neurosurgery. Pediatrics
Erin M Hall, Jeffrey Leonard, Jodi L Smith, Kristin P Guilliams, Michael Binkley, Robert J Fallon, Monica L Hulbert
BACKGROUND: Children with sickle cell disease (SCD) and moyamoya may benefit from indirect cerebral revascularization surgery in addition to chronic blood transfusion therapy for infarct prevention. We sought to compare overt and silent infarct recurrence rates in children with SCD undergoing revascularization. METHODS: This was a retrospective cohort study of all children with SCD and moyamoya treated at two children's hospitals. Clinical events and imaging studies were reviewed...
August 2016: Pediatric Blood & Cancer
W Lee Titsworth, R Michael Scott, Edward R Smith
BACKGROUND AND PURPOSE: Comprehensive multicenter data on treatment of pediatric moyamoya in the United States is lacking. We sought to identify national trends in the diagnosis and treatment of this disease. METHODS: A total of 2454 moyamoya admissions from 1997 to 2012 were identified from the Kids Inpatient Database. Demographics, inpatient costs, interventions, and discharge status were analyzed. Admissions with and without surgical revascularization were reviewed separately...
May 2016: Stroke; a Journal of Cerebral Circulation
Edward R Smith
PURPOSE OF REVIEW: Moyamoya and arteriovenous malformations represent, respectively, significant sources of ischemic and hemorrhagic stroke in children after the first year of life. Although rarely encountered in routine pediatric practice, the potential severe morbidity of these entities, coupled with the typical acuity of their presentation, merit ongoing awareness of current relevant diagnostic and therapeutic strategies. RECENT FINDINGS: Mutations in RNF213, ACTA2, and GUCY are implicated in moyamoya...
December 2015: Current Opinion in Pediatrics
Edward R Smith
Moyamoya disease (MMD) is an arteriopathy of the intracranial circulation predominantly affecting the branches of the internal carotid arteries. Heterogeneity in presentation, progression and response to therapy has prompted intense study to improve the diagnosis and prognosis of this disease. Recent progress in the development of moyamoya-related biomarkers has stimulated marked interest in this field. Biomarkers can be defined as biologically derived agents-such as specific molecules or unique patterns on imaging-that can identify the presence of disease or help to predict its course...
June 2015: Journal of Korean Neurosurgical Society
Alfred P See, Alexander E Ropper, Daniel L Underberg, Richard L Robertson, R Michael Scott, Edward R Smith
OBJECT Moyamoya can cause cerebral ischemia and stroke in Down syndrome (DS) patients. In this study, the authors defined a surgically treated population of patients with DS and moyamoya and compared their clinical presentation, response to surgical treatment, and long-term prognosis with those of the general population of patients with moyamoya but without DS. METHODS This study was a retrospective review of a consecutive operative series of moyamoya patients with DS treated at Boston Children's Hospital from 1985 through 2012...
July 2015: Journal of Neurosurgery. Pediatrics
Lissa C Baird, Edward R Smith, Rebecca Ichord, David A Piccoli, Timothy J Bernard, Nancy B Spinner, R Michael Scott, Binita M Kamath
Vasculopathy is well-described in Alagille syndrome (ALGS); however, few data exist regarding neurosurgical interventions. We report 5 children with ALGS with moyamoya who underwent revascularization surgery. Postsurgical complications included 1 stroke and 1 death from thalamic hemorrhage. Global function improved in survivors. Revascularization is reasonably safe in patients with ALGS and may improve neurologic outcomes.
February 2015: Journal of Pediatrics
Bradley A Gross, Scellig S Stone, Edward R Smith
BACKGROUND: Indirect revascularization techniques for posterior circulation moyamoya are infrequently described in the literature given the relative rarity of the disease process, particularly in the best candidates for indirect approaches-children. METHODS: We present a detailed, illustrated video demonstration of the occipital pial synangiosis procedure performed in a 5-year-old child. CONCLUSION: The specific advantages of the pial synangiosis technique-suturing the donor vessel to the pia and a wide arachnoidal opening-can be successfully applied to posterior circulation indirect revascularization...
July 2014: Acta Neurochirurgica
Eric M Jackson, Ning Lin, Sunil Manjila, R Michael Scott, Edward R Smith
Object Patients with moyamoya who are younger than 2 years of age represent a therapeutic challenge because of their frequent neurological instability and concomitant anesthetic risks. The authors report their experience with pial synangiosis revascularization in this population. Methods The authors reviewed the clinical and radiographic records of all patients with moyamoya in a consecutive series of patients under 2 years of age, who underwent cerebral revascularization surgery using pial synangiosis at a single institution...
April 2014: Journal of Neurosurgery. Pediatrics
Ning Lin, Joshua P Aronson, Sunil Manjila, Edward R Smith, R Michael Scott
OBJECT: Surgical treatment of moyamoya disease in the adult population commonly uses direct revascularization, the superficial temporal artery (STA) to middle cerebral artery (MCA) bypass (STA-MCA). Pial synangiosis, a method of indirect revascularization, has been used in adult patients with moyamoya when STA-MCA bypass was not technically feasible. Although the effectiveness of pial synangiosis has been well described in children, only limited reports have examined its role in adult patients with moyamoya disease...
March 2014: Journal of Neurosurgery
Meredith R Golomb, Jodi L Smith
Wound healing is a key component of recovery for children with neurologic conditions undergoing neurosurgical procedures. Understanding factors that can impair wound healing aids in planning long-term clinical care. Children with neurofibromatosis type 1 are at risk for vasculopathies in the brain (including moyamoya vasculopathy) and in other organs, including the heart, lung, and skin. Neurofibromatosis 1 is caused by mutations in the gene for neurofibromin, a protein that plays a role in tissue maintenance and repair as well as tumor suppression...
October 2014: Journal of Child Neurology
Amit Singla, Ning Lin, Allen L Ho, R Michael Scott, Edward R Smith
Surgically created openings such as bur holes can serve as avenues for the development of collateral blood supply to the brain in patients with moyamoya disease. When such collateralization occurs through preexisting shunt catheter sites, the potential exists for perioperative stroke if these vessels are damaged during revision of a ventricular catheter for shunt malfunction. In this paper the authors report on a series of patients with a history of ventriculoperitoneal (VP) shunts who later developed moyamoya disease and were found to have spontaneous transdural collateral vessels at ventricular catheter sites readily visualized on diagnostic angiography...
June 2013: Journal of Neurosurgery. Pediatrics
Subash Lohani, Joseph R Madsen, Ann M Bergin, Edward R Smith
The combination of moyamoya syndrome and symptomatic mesial temporal sclerosis (MTS) has not previously been reported. The authors present the case of a 5-year-old boy with symptomatic MTS who developed progressive moyamoya syndrome. This combination of progressive moyamoya and a structural seizure focus presented a unique clinical problem, with the natural history of MTS predicting a high likelihood of needing resection in the future, which could be challenging following any type of moyamoya-related revascularization surgery...
June 2013: Journal of Neurosurgery. Pediatrics
McKenzie Koss, R Michael Scott, Mira B Irons, Edward R Smith, Nicole J Ullrich
OBJECT: Children with neurofibromatosis Type 1 (NF1) can present with progressive arteriopathy of the branches of the internal carotid artery consistent with moyamoya syndrome. Clinical symptoms, radiographic evidence of ischemia, and the potential for disease progression may necessitate surgical revascularization to minimize the risk of stroke and progressive neurological deficits. This study aims to evaluate the presentation and surgical outcomes of these patients by reviewing clinical, radiographic, and angiographic data...
April 2013: Journal of Neurosurgery. Pediatrics
Edward S Ahn, R Michael Scott, Richard L Robertson, Edward R Smith
OBJECT: Chorea is a movement disorder characterized by brief, irregular, involuntary contractions that appear to flow from 1 muscle to another. There are a limited number of reports in the literature that have linked moyamoya disease and chorea. The authors describe their experience in treating moyamoya disease in patients in whom chorea developed as part of the clinical presentation. METHODS: The authors conducted a retrospective review of a consecutive series of 316 children who underwent pial synangiosis revascularization for moyamoya disease at the Boston Children's Hospital...
March 2013: Journal of Neurosurgery. Pediatrics
Edward R Smith
The arteriopathy of moyamoya is progressive and results in severe disability from cerebral ischemia. Once the diagnosis is confirmed with magnetic resonance imaging (MRI) and catheter angiography, initial measures should consist of administration of low dose aspirin (usually 81 mg daily, barring pre-existing contraindications), maintenance of good hydration and avoidance of hyperventilation (to reduce the risk of reflex cerebral vasoconstriction). Definitive treatment is predicated on surgical revascularization of the affected cerebral hemispheres...
December 2012: Current Treatment Options in Neurology
Edward R Smith, R Michael Scott
Moyamoya is a progressive arteriopathy of unknown origin affecting the branches of the internal carotid artery (ICA). The arteriopathy can present as an isolated medical condition, affecting both sides of the brain ("moyamoya disease") or can be unilateral or found in association with systemic disorders ("moyamoya syndrome"). The ischemia resulting from luminal narrowing predisposes children to transient ischemic attacks and stroke-the primary presentations of affected patients. Although it is rare-affecting 1 in 1 million children in the US-moyamoya is implicated in 6% of all childhood strokes...
April 2012: Journal of Neurosurgery. Pediatrics
Ning Lin, Lissa Baird, McKenzie Koss, Kimberly E Kopecky, Evelyne Gone, Nicole J Ullrich, R Michael Scott, Edward R Smith
OBJECT: Limited data exist to guide management of incidentally discovered pediatric moyamoya. Best exemplified in the setting of unilateral moyamoya, in which the unaffected side is monitored, this phenomenon also occurs in populations undergoing routine surveillance of the cerebral vasculature for other conditions, such as sickle cell disease (SCD) or neurofibromatosis Type 1 (NF1). The authors present their experience with specific syndromic moyamoya populations to better characterize the natural history of radiographic and clinical progression in patients with asymptomatic moyamoya...
December 2011: Neurosurgical Focus
F J Kirkham, P Haywood, P Kashyape, J Borbone, A Lording, K Pryde, M Cox, J Keslake, M Smith, L Cuthbertson, V Murugan, S Mackie, N H Thomas, A Whitney, K M Forrest, A Parker, R Forsyth, C M Kipps
The literature on paediatric acute-onset movement disorders is scattered. In a prospective cohort of 52 children (21 male; age range 2mo-15y), the commonest were chorea, dystonia, tremor, myoclonus, and Parkinsonism in descending order of frequency. In this series of mainly previously well children with cryptogenic acute movement disorders, three groups were recognised: (1) Psychogenic disorders (n = 12), typically >10 years of age, more likely to be female and to have tremor and myoclonus (2) Inflammatory or autoimmune disorders (n = 22), including N-methyl-d-aspartate receptor encephalitis, opsoclonus-myoclonus, Sydenham chorea, systemic lupus erythematosus, acute necrotizing encephalopathy (which may be autosomal dominant), and other encephalitides and (3) Non-inflammatory disorders (n = 18), including drug-induced movement disorder, post-pump chorea, metabolic, e...
September 2011: European Journal of Paediatric Neurology: EJPN
Martina Vendrame, Joseph Kaleyias, Tobias Loddenkemper, Ed Smith, Craig McClain, Mark Rockoff, Sheryl Manganaro, Bonnie McKenzie, Lixia Gao, Michael Scott, Blaise Bourgeois, Sanjeev V Kothare
We describe our experience with intraoperative electroencephalography in moyamoya surgery, a method to monitor for ischemic changes during the procedure and to minimize the risk of intraoperative and perioperative stroke. Case records and intraoperative electroencephalography recordings of all patients (n=220) treated with surgical revascularization for moyamoya (pial synangiosis) performed for 14 years (1994-2008) were reviewed. Electroencephalographic slowing occurred in 100 cases (45.5%), and was persistent in nine cases (9%)...
June 2011: Pediatric Neurology
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