scholarly journals Moyamoya disease in the midwestern United States

1998 ◽  
Vol 5 (5) ◽  
pp. E3 ◽  
Author(s):  
Nicholas M. Wetjen ◽  
P. Charles Garell ◽  
Nicholas V. Stence ◽  
Christopher M. Loftus
Keyword(s):  
United States ◽  
Moyamoya Disease ◽  
Management Practices ◽  
Correct Diagnosis ◽  
Management Strategies ◽  
Superficial Temporal Artery ◽  
Age Distribution ◽  
Temporal Artery ◽  
The United States ◽  
Epidemiological Characteristics

There have been few investigations of moyamoya disease in the United States and no systematic description of the management practices or outcome from this population. The authors reviewed their experience with this disease to gain a better understanding and improve the treatment of patients with moyamoya disease in the United States. Over a 25-year period 30 patients with moyamoya disease have been treated at the University of Iowa. The cases were divided into patients who had classic, probable, and akin moyamoya disease. Results indicated that there was a bimodal age distribution and a female predominance of cases. In estimating the referral pattern of our institution, the authors determined that there were greater numbers of epidemiological characteristics than previously anticipated. Patients were treated either surgically or nonsurgically, and different management strategies were utilized in each of the major groups: superficial temporal artery to middle cerebral artery anastomosis and encephalodurosynangiosis in the surgical group; or antiplatelet, anticoagulation, or nonpharmacological intervention in the nonsurgical group. The authors conclude that there is a higher prevalence and incidence of moyamoya disease in the United States than previously reported and that there are some clinical characteristics of this disease that differ from the cases reported in southeast Asia. These differences may be due to genetic or environmental factors but can also be partly explained by the lower index of suspicion for this disease and, thus, a delay in or complete absence of the correct diagnosis.

scholarly journals Treatment of a cerebral pial arteriovenous fistula in a patient with sickle cell disease-related moyamoya syndrome: case report

2015 ◽  
Vol 16 (2) ◽  
pp. 207-211 ◽  
Author(s):  
Anna Lo Presti ◽  
Alexander G. Weil ◽  
Aria Fallah ◽  
Eric C. Peterson ◽  
Toba N. Niazi ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Moyamoya Disease ◽  
De Novo ◽  
Transarterial Embolization ◽  
Management Strategies ◽  
Superficial Temporal Artery ◽  
Temporal Artery ◽  
Venous Aneurysm ◽  
Cell Disease

Sickle cell disease (SCD) is an autosomal recessive hematological disorder, characterized by sickling of the red blood cells in response to a hypoxic stress and vaso-occlusive crises. It is associated with moyamoya-like changes on cerebral angiographic imaging in 43% of patients. Cerebral aneurysms, arteriovenous malformations, and dural arteriovenous fistulas (AVFs) have been described in association with SCD and moyamoya disease. However, the description of a pial AVF (pAVF) in a patient with SCD and/or moyamoya formation has not yet been reported. The authors present the case of a 15-year-old boy with SCD-associated moyamoya disease harboring a pAVF who developed a de novo venous aneurysm 8 months after undergoing indirect superficial temporal artery-middle cerebral artery (MCA) bypass that was complicated by bilateral ischemia of the MCA territory. The pAVF was successfully treated with transarterial embolization using Onyx. The authors describe the possible pathophysiological mechanisms and management strategies for this rare occurrence.

The efficacy of superficial temporal artery–middle cerebral artery anastomosis in patients with moyamoya disease complaining of severe headache

2012 ◽  
Vol 116 (3) ◽  
pp. 672-679 ◽  
Author(s):  
Yoshikazu Okada ◽  
Takakazu Kawamata ◽  
Akitsugu Kawashima ◽  
Kohji Yamaguchi ◽  
Yuko Ono ◽  
...  
Keyword(s):  
Middle Cerebral Artery ◽  
Moyamoya Disease ◽  
Cerebral Artery ◽  
Superficial Temporal Artery ◽  
Age Distribution ◽  
Young Patients ◽  
Temporal Artery ◽  
Severe Headache ◽  
Cerebrovascular Reactivity ◽  
Complete Relief

Object Some patients with moyamoya disease complain of severe headache, which may be closely related to cerebral ischemia. The efficacy of superficial temporal artery–middle cerebral artery (STA-MCA) anastomosis in these patients was evaluated by clinicoradiological studies. Methods Of 117 consecutive patients with ischemic moyamoya disease, 25 complained mainly of severe headache (headache group) and 92 had no significant headache (nonheadache group). Intensity of headache was evaluated pre- and postoperatively. Furthermore, regional cerebral blood flow (rCBF) and cerebrovascular reactivity (CVR) were assessed pre- and postoperatively. Results The headache group was significantly younger than the nonheadache group. In a group corrected for the age distribution, preoperative rCBF and CVR were similar in headache and nonheadache groups. After STA-MCA anastomosis, 16 patients with headache experienced complete relief from headache, 7 patients showed remarkable improvements and discontinued medications for headache, and the remaining 2 patients had some alleviation of headache but sometimes required medication. In the headache group, the postoperative rCBF was significantly greater than the preoperative value. The postoperative rCBF in this group was also significantly greater than the postoperative rCBF in the nonheadache group. Conclusions These data suggest that severe headache is one of the main symptoms in young patients with moyamoya disease probably related to cerebral circulatory disturbances. An STA-MCA anastomosis is effective in relieving headache in patients with ischemic moyamoya disease manifesting severe headache, probably by improving perfusion pressure and cerebral circulation.

Prolonged/delayed cerebral hyperperfusion in adult patients with moyamoya disease with RNF213 gene polymorphism c.14576G>A (rs112735431) after superficial temporal artery–middle cerebral artery anastomosis

2020 ◽  
pp. 1-8
Author(s):  
Ryosuke Tashiro ◽  
Miki Fujimura ◽  
Masahito Katsuki ◽  
Taketo Nishizawa ◽  
Yasutake Tomata ◽  
...  
Keyword(s):  
Genetic Analysis ◽  
Gene Polymorphism ◽  
Intracerebral Hemorrhage ◽  
Middle Cerebral Artery ◽  
Moyamoya Disease ◽  
Cerebral Artery ◽  
Superficial Temporal Artery ◽  
Temporal Artery ◽  
Adult Patients ◽  
Cerebral Hyperperfusion

OBJECTIVESuperficial temporal artery–middle cerebral artery (STA-MCA) anastomosis is the standard surgical management for moyamoya disease (MMD), whereas cerebral hyperperfusion (CHP) is one of the potential complications of this procedure that can result in delayed intracerebral hemorrhage and/or neurological deterioration. Recent advances in perioperative management in the early postoperative period have significantly reduced the risk of CHP syndrome, but delayed intracerebral hemorrhage and prolonged/delayed CHP are still major clinical issues. The clinical implication of RNF213 gene polymorphism c.14576G>A (rs112735431), a susceptibility variant for MMD, includes early disease onset and a more severe form of MMD, but its significance in perioperative pathology is unknown. Thus, the authors investigated the role of RNF213 polymorphism in perioperative hemodynamics after STA-MCA anastomosis for MMD.METHODSAmong 96 consecutive adult patients with MMD comprising 105 hemispheres who underwent serial quantitative cerebral blood flow (CBF) analysis by N-isopropyl-p-[123I]iodoamphetamine SPECT after STA-MCA anastomosis, 66 patients consented to genetic analysis of RNF213. Patients were routinely maintained under strict blood pressure control during and after surgery. The local CBF values were quantified at the vascular territory supplied by the bypass on postoperative days (PODs) 1 and 7. The authors defined the radiological CHP phenomenon as a local CBF increase of more than 150% compared with the preoperative values, and then they investigated the correlation between RNF213 polymorphism and the development of CHP.RESULTSCHP at POD 1 was observed in 23 hemispheres (23/73 hemispheres [31.5%]), and its incidence was not statistically different between groups (15/41 [36.6%] in RNF213-mutant group vs 8/32 [25.0%] in RNF213–wild type (WT) group; p = 0.321). CHP on POD 7, which is a relatively late period of the CHP phenomenon in MMD, was evident in 9 patients (9/73 hemispheres [12.3%]) after STA-MCA anastomosis. This prolonged/delayed CHP was exclusively observed in the RNF213-mutant group (9/41 [22.0%] in the RNF213-mutant group vs 0/32 [0.0%] in the RNF213-WT group; p = 0.004). Multivariate analysis revealed that RNF213 polymorphism was significantly associated with CBF increase on POD 7 (OR 5.47, 95% CI 1.06–28.35; p = 0.043).CONCLUSIONSProlonged/delayed CHP after revascularization surgery was exclusively found in the RNF213-mutant group. Although the exact mechanism underlying the contribution of RNF213 polymorphism to the prolonged/delayed CBF increase in patients with MMD is unclear, the current study suggests that genetic analysis of RNF213 is useful for predicting the perioperative pathology of patients with MMD.

scholarly journals 55 Malignant primary brain and other central nervous system tumours diagnosed in the Canadian population from 2009 to 2013

2018 ◽  
Vol 45 (S3) ◽  
pp. S16-S17
Author(s):  
EV Walker ◽  
F Davis ◽  
Keyword(s):  
United States ◽  
Central Nervous System ◽  
Nervous System ◽  
Brain Tumour ◽  
Age Distribution ◽  
Paediatric Population ◽  
The United States ◽  
Incidence Rates ◽  
Canadian Population ◽  
Cns Tumours

The Canadian Brain Tumour Registry (CBTR) project was established in 2016 with the aim of enhancing infrastructure for surveillance and clinical research to improve health outcomes for brain tumour patients in Canada. We present a national surveillance report on malignant primary brain and central nervous system (CNS) tumours diagnosed in the Canadian population from 2009-2013. Patients were identified through the Canadian Cancer Registry (CCR); an administrative dataset that includes cancer incidence data from all provinces/territories in Canada. Cancer diagnoses are coded using the ICD-O3 system. Tumour types were classified by site and histology using The Central Brain Tumour Registry of the United States definitions. Incidence rates (IR) and 95% confidence intervals (CI) were calculated per 100,000 person-years and standardized to the 2011 census population age-distribution. Overall, 12,115 malignant brain and CNS tumours were diagnosed in the Canadian population from 2009-2013 (IR:8.43;95%CI:8.28,8.58). Of these, 6,845 were diagnosed in males (IR:9.72;95%CI:9.49,9.95) and 5,270 in females (IR:7.20;95%CI:7.00,7.39). The most common histology overall was glioblastoma (IR:4.06;95%CI:3.95,4.16). Among those aged 0-19 years, 1,130 malignant brain and CNS tumours were diagnosed from 2009-2013 (IR:3.36;95%CI:3.16,3.56). Of these, 625 were diagnosed in males (IR:3.32;95%CI:3.34,3.92) and 505 in females (IR:3.08;95%CI:2.81,3.36). The most common histology among the paediatric population was pilocytic astrocytoma (IR:0.73;95%CI:0.64,0.83). The presentation will include: IRs for other histologies, the geographic distribution of cases and a comparison between Canada and the United States.

Synergistic Interactions Between Direct and Indirect Bypasses in Combined Procedures: The Significance of Indirect Bypasses in Moyamoya Disease

Neurosurgery ◽  
2017 ◽  
Vol 80 (2) ◽  
pp. 201-209 ◽  
Author(s):  
Haruto Uchino ◽  
Jae-Hoon Kim ◽  
Noriyuki Fujima ◽  
Ken Kazumata ◽  
Masaki Ito ◽  
...  
Keyword(s):  
Moyamoya Disease ◽  
Superficial Temporal Artery ◽  
Disease Onset ◽  
Chronic Phase ◽  
Temporal Artery ◽  
Middle Meningeal Artery ◽  
Hemodynamic Parameters ◽  
Combined Procedures ◽  
Multiple Variables ◽  
Meningeal Artery

Abstract BACKGROUND: Whether additional indirect bypasses effectively contribute to revascularization in combined procedures remains unclear in patients with moyamoya disease. OBJECTIVE: To evaluate the longitudinal changes associated with combined procedures while following up pediatric and adult patients long term and to assess whether any other clinical factors or hemodynamic parameters affected these changes to determine an optimal surgical strategy. METHODS: We studied 58 hemispheres in 43 adults and 39 hemispheres in 26 children who underwent combined revascularization for moyamoya disease. To evaluate bypass development, we assessed the sizes of the superficial temporal artery and middle meningeal artery using magnetic resonance angiography. Multivariate analysis determined the effects of multiple variables on bypass development. RESULTS: Indirect bypass (middle meningeal artery) development occurred in 95% and 78% of the pediatric and adult hemispheres, respectively. Of these, dual development of direct and indirect bypasses occurred in 54% of the pediatric hemispheres and in 47% of the adult hemispheres. Reciprocal superficial temporal artery regression occurred in 28% of the hemispheres during the transition from the postoperative acute phase to the chronic phase during indirect bypass development. Good indirect bypass development was associated with adult hemispheres at Suzuki stage 4 or greater (odds ratio, 7.4; 95% confidence interval, 1.4-39.4; P = .02). Disease onset type and preoperative hemodynamic parameters were not considered predictors for the development of surgical revascularization. CONCLUSION: Simultaneous direct and indirect bypass development was most frequently observed, regardless of patient age and hemodynamic status. Applying indirect bypass as an adjunct to direct bypass could maximize revascularization in adults and children.

scholarly journals 1428. Lyme Disease Treatment in the United States: Prescribing Patterns from a Nationwide Commercial Insurance Database, 2016-2018

2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S720-S720
Author(s):  
Amy M Beeson ◽  
Grace E Marx ◽  
Amy M Schwartz ◽  
Alison F Hinckley
Keyword(s):  
United States ◽  
Lyme Disease ◽  
Standard Treatment ◽  
Age Distribution ◽  
Public Health Problem ◽  
The United States ◽  
Adverse Outcomes ◽  
Prescribing Patterns ◽  
Antibiotic Prescribing ◽  
Low Incidence

Abstract Background Lyme disease (LD) is the most common vector-borne disease in the United States and is a significant public health problem. The use of non-standard antibiotic treatment regimens for LD has been associated with adverse effects; however, the overall landscape of treatment has not been described previously. We aimed to describe real-world antibiotic prescribing patterns for LD. Methods We performed a retrospective analysis of the MarketScan commercial claims database of outpatient encounters from 2016-2018 in the United States. We identified all individuals with a visit that included an LD diagnosis code and a prescription within 30 days of the visit for one or more of 12 antibiotics that may be prescribed for LD. We then categorized each individual as having received either standard or non-standard treatment during the two-year period. Standard treatment was defined as treatment with a first, second or third-line antibiotic for LD, for no longer than 30 days, and for no more than two episodes during the study period. Descriptive and multivariable analyses were performed to compare characteristics of people who received standard vs non-standard treatment for LD. Results A total of 84,769 prescriptions met criteria for inclusion, written for 45,926 unique patients. The mean duration of prescriptions was 21.4 days (SD 10.8). Most individuals (84.5%) treated for LD received standard treatment during the study period. Female gender (OR 1.5, p< 0.0001) and age 19-45 (p=0.0003) were significantly associated with being prescribed non-standard LD treatment. Treatment in low-incidence states (OR 2.2 compared to high-incidence states, p< 0.0001) and during non-summer months (OR 2.2, p< 0.0001) was more likely to be non-standard. Age distribution of patients receiving treatment for Lyme disease, by gender and age at first prescription Seasonality of standard versus non-standard treatment of Lyme disease Conclusion In this population of employed, young, and insured patients, young and middle-aged women were at the highest risk of receiving non-standard LD treatment. Treatments prescribed in states with low incidence of LD or during non-summer months were also more likely to be non-standard, a trend which likely reflects misdiagnosis or overtreatment of LD. Future studies are needed to further define prescriber and patient factors associated with non-standard LD treatment and related adverse outcomes. Disclosures All Authors: No reported disclosures

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