scholarly journals Postpartum-Onset Moyamoya Disease: A Rare Cause of Stroke in Unexpected

2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Muhammet Ozer ◽  
Khadija Merchant ◽  
Zulfiya Manning ◽  
Suleyman Yasin Goksu ◽  
Kirti Juneja ◽  
...  

Moyamoya disease (MMD) is a chronic cerebrovascular occlusive disease that is characterized by progressive bilateral stenosis of the terminal portion of the internal carotid artery and its main branches. Cerebrovascular events are the primary presenting symptoms and are related both to stenosis and occlusion of the ICAs and their main branches. Detection of bilateral stenosis by cerebral angiography is considered the gold standard, but computed tomography angiography (CTA) is also an acceptable method of diagnosis. In the current literature, there are no precise data on the incidence of moyamoya disease in Europe and the United States. Also, the pathogenesis of MMD remains obscure, and genetic factors and inflammation are the two most representative mechanisms. Here, we report the case of MMD in a 29-year-old African American female who presented with an ischemic stroke for the second time that manifested after pregnancy. This case is important to increase awareness of the probability of this rare disease in Western countries as well as to call attention to pregnancy’s accelerating effects of MMD. Careful, long-term neurologic and radiologic follow-up is essential in adult patients with MMD to prevent additional stroke events and improve outcomes.

Stroke ◽  
2020 ◽  
Vol 51 (Suppl_1) ◽  
Author(s):  
Satoshi Kuroda ◽  
Naoki Nakayama ◽  
Shusuke Yamamoto ◽  
Daina Kashiwazaki ◽  
Haruto Uchino ◽  
...  

Background and Purpose: Surgical revascularization is now known to improve the outcome in patients with moyamoya disease. However, majority of previous studies reported their short-term (<5 years) outcome. Therefore, this study was aimed to evaluate long-term (5 to 20 years) outcome after STA-MCA anastomosis and ultimate indirect bypass, encephalo-duro-myo- arterio-pericranial synangiosis (EDMAPS). Methods: Cumulative incidence of late morbidity/mortality and disease progression were evaluated among 93 patients who underwent STA-MCA anastomosis and EDMAPS. All of them were prospectively followed up for longer than 5 years post-surgery (mean, 10.5±4.4 years). There were 35 pediatric and 58 adult patients. Clinical diagnosis included TIA or ischemic stroke in 80 patients, hemorrhagic stroke in 10, and asymptomatic in 3. STA-MCA anastomosis and EDMAPS were performed onto their 141 hemispheres. MRI and MRA were performed every 6 or 12 years during follow-up periods. Results: During follow-up periods, 92/93 patients were free from any stroke or death, but one recurred hemorrhagic stroke (0.10% per patient-year). Disease progression occurred in the territory of the contralateral carotid or posterior cerebral artery (PCA) in 19 hemispheres of 15 patients (1.5% per patient-year). The interval between initial surgery and disease progression varied from 0.5 to 15 years. Repeat bypass surgery for anterior and posterior circulations resolved ischemic attacks in all 10 patients. Conclusion: STA-MCA anastomosis and EDMAPS would be the best choice to prevent further cerebrovascular events for longer than 10 years by widely providing surgical collaterals to both the MCA and ACA territories. However, regular follow-up would be essential for longer than 10 years post-surgery to identify the disease progression in the territory of contralateral carotid artery and PCA and prevent late cerebrovascular events.


2012 ◽  
Vol 10 (5) ◽  
pp. 451-456 ◽  
Author(s):  
Maki Mukawa ◽  
Tadashi Nariai ◽  
Yoshiharu Matsushima ◽  
Yoji Tanaka ◽  
Motoki Inaji ◽  
...  

Object Surgical revascularization is considered an effective treatment for juvenile patients with moyamoya disease (MMD). Yet the long-term outcome in surgically treated patients still needs to be clarified. More than 30 years have passed since the authors' department started intensively treating pediatric patients with MMD using indirect anastomosis techniques. In this study the authors surveyed the current status of these patients. Methods Activities of daily living (ADLs) were surveyed and present clinical status was assessed based on the modified Rankin Scale (mRS). Cerebrovascular events subsequent to surgical treatment were also recorded. Results Since 1979, 208 patients younger than 19 years of age with MMD were surgically treated and followed up for > 3 years. Data were available on 172 patients (83%), who had been followed up for a mean of 14.3 years (range 3–32 years). Activity of daily living outcomes were as follows: 138 patients (80.2%) had mRS scores of 0–2, 29 (16.9%) a score of 3, 1 (0.6%) a score of 4, 1 (0.6%) a score of 5, and 3 (1.7%) a score of 6. Cerebrovascular events occurred 8 or more years after surgery in 6 patients (3.4%), that is, 6 hemorrhages and 3 infarctions. The cumulative risk of late-onset stroke at 10, 20, and 30 years after surgical intervention was 0.8%, 6.3%, and 10.0%, respectively. Conclusions This long-term survey demonstrated that most surgically treated pediatric patients with MMD maintain good ADL outcomes. However, a significant number of new cerebrovascular events occurred more than 10 years after the initial surgery. Additional follow-up will help to identify which events may occur during the adult years of patients treated as children.


2020 ◽  
Vol 11 ◽  
pp. 215013272097774
Author(s):  
Stephanie T. Fulleborn ◽  
Paul F. Crawford ◽  
Jeremy T. Jackson ◽  
Christy J.W. Ledford

Introduction Recent evidence reveals that diabetes and prediabetes (preDM) can be reversed to normal glucose regulation (NGR) through significant weight loss, but how physicians clinically identify the principles of partial and complete remission of diabetes is largely unknown. Methods As part of the cross-sectional omnibus survey conducted in March 2019 at a professional annual meeting in the United States, physician participants answered case scenario questions about the diagnosis and documentation of patients with preDM and type 2 diabetes (T2DM). Results Of the registered conference attendees, 387 (72.7%) responded. When presented with the initial case of preDM, 201 physicians (70.8%) selected R73.03 Prediabetes. In a follow-up encounter with improved lab results, 118 physicians (58.7%) indicated that they would not chart any diabetes-related code and 62 (30.8%) would chart preDM again. When presented with the case of T2DM, 256 physicians (90.1%) indicated E11.0–E11.9 Type 2 Diabetes. In the follow-up encounter, only 38 (14.8%) coded a diagnosis reflecting remission from T2DM to prediabetes and 211 (82.4%) charted T2DM. Conclusion Physicians may be reluctant to document diabetes regression as there is little evidence for long-term outcomes and “downgrading” the diagnosis in the medical record may cause screenings to be missed. Documenting this regression in the medical record should communicate the accurate point on the continuum of glucose intolerance with both the patient and the care team.


Stroke ◽  
2013 ◽  
Vol 44 (suppl_1) ◽  
Author(s):  
Satoshi Kuroda ◽  
Naoki Akioka ◽  
Daina Kashiwazaki ◽  
Hideo Hamada ◽  
Naoya Kuwayama ◽  
...  

Introduction —It is well known that surgical revascularization can improve cerebral hemodynamics and prevent further ischemic cerebrovascular events in moyamoya disease. However, a certain subgroup of patients repeats ischemic attacks even after surgery because of insufficient surgery or disease progression during follow-up periods. Hypothesis —Relevant designs and techniques in additional bypass surgery can resolve ischemic cerebrovascular events in patients with moyamoya disease refractory to previous bypass surgery. Methods —This study included totally 7 patients (9 hemispheres) with moyamoya disease refractory to previous bypass surgery. There were 5 children and 2 adults. They underwent previous bypass surgery in Japan and Europe 6 to 240 months before admission. Based on precise clinical and radiological analysis, cerebrovascular events were considered to occur because of insufficient bypass surgery in 5 patients and disease progression in the ipsilateral posterior cerebral artery in 2. Surgical strategies included wide craniotomy to cover the area where cerebral hemodynamics is still impaired and appropriate bypass procedures such as STA-MCA anastomosis, OA-PCA anastomosis, and indirect bypass. Using [123]I-IMP SPECT or [15]O-gas PET, cerebral hemodynamics was precisely examined before and after surgery Results —Postoperative course was uneventful and cerebral hemodynamics significantly improved in all 7 patients. Postoperative cerebral angiography revealed that additional bypass provided collateral blood flow to ischemic area before surgery. Ischemic cerebrovascular events rapidly resolved in 5 patients and gradually decreased in 2. Conclusion —This study strongly suggests adequate surgical design and procedures can resolve ischemic cerebrovascular events in patients with moyamoya disease refractory to previous bypass surgery.


Neurosurgery ◽  
2003 ◽  
Vol 52 (5) ◽  
pp. 1049-1055 ◽  
Author(s):  
Motohiro Morioka ◽  
Jun-ichiro Hamada ◽  
Tatemi Todaka ◽  
Shigetoshi Yano ◽  
Yutaka Kai ◽  
...  

Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
David Fam ◽  
Nomazulu Dlamini ◽  
Cheryl Jaigobin

Background: Moyamoya disease is a progressive occlusive arteriopathy of the terminal ICA and its branches, leading to the formation of unstable collateral vessels. The disease is found worldwide, and is associated with a number of predisposing conditions, termed moyamoya syndrome. Currently there is a paucity of data on the natural history of moyamoya disease in North America, especially the long-term outcome of pediatric moyamoya. Objective: Our objectives were to determine the rate of recurrent TIA, ischemic stroke and intracerebral hemorrhage in patients presenting with pediatric Moyamoya disease and to assess long-term functional outcome. Methods: This study was a retrospective chart review of 52 patients presenting with pediatric moyamoya disease. We included patients aged 18 or older with a diagnosis of moyamoya disease or syndrome. All patients had confirmatory baseline vascular imaging (MRA or CTA +/- cerebral angiography). Baseline demographic variables and annual vascular event recurrence risk were obtained from the records. Modified Rankin Score (MRS) at presentation and last follow-up were determined from clinical records. Results: Of the original cohort, 34 patients were included for analysis. The mean age of the patients was 23.9 years. The mean age at presentation was 9 years (SD=4) with an average follow-up of 11.3 years (SD=5.1) for a total of 383 patient years. There was slight female predominance (1.4:1). Fifty percent of patients (17) presented with ischemic stroke. A total of 26 patients (76%) underwent surgery, with 7 (21%) requiring repeat surgery. The annual vascular recurrent event rate was 5.0% (19/383), which was not significantly different between surgical and non-surgical groups or between different moyamoya syndrome subtypes. Most of the recurrent events were TIA (annual recurrence 3.7%). There was no significant difference between initial MRS, MRS at last follow-up and mean change in MRS between surgical and non-surgical groups. Conclusion: Our study is the largest North American natural history study of pediatric moyamoya. Our observations indicate that pediatric moyamoya in North America have low recurrent vascular event rates and long-term functional outcomes are good, even in conservatively managed patients.


Stroke ◽  
2021 ◽  
Vol 52 (Suppl_1) ◽  
Author(s):  
Juliana Gomez ◽  
Mark Burish ◽  
Sean I Savitz ◽  
Louise D McCullough ◽  
Cecilia Ganduglia Cazaban

Introduction: Migraine is common and often disabling. Recent research has focused on a new class of treatments targeting the pain-signaling molecule calcitonin-gene-related peptide (CGRP). CGRP inhibitors are the first preventive therapies designed specifically for migraine. However, CGRP also induces vasodilation, and plays a role in cerebral blood flow autoregulation. The clinical cerebrovascular effects of long-term blockade of CGRP are not yet known. Methods: We analyzed a retrospective cohort of migraine patients from a large administrative dataset in the United States (OPTUM) that covers patients with commercial insurance and Medicare Advantage, during 2018 and 2019, using ICD-10 codes for all types of migraines. Migraine patients were divided into two groups based on their use of CGRP inhibitors (erenumab, fremanezumab, galcanezumab). We determined the incidence of stroke during 2018 and 2019 among both groups. For the group of migraine patients who used CGRP inhibitors, we focused on the incidence of stroke after these medications were initiated. Results: We identified 646,404 migraine patients; 18, 692 (2.9%) of them had used a CGRP inhibitor. Among the users of CGRP inhibitors, 86% were females, and 58% of patients were in the 41 to 65-year-old range. The average time of follow-up was 197 days after initiation of the medication. A total of 1.3 % of patients who used CGRP inhibitors had an ischemic stroke during the follow-up period versus 2.4% of patients who did not use CGRP inhibitors. The lower rate of stroke in the CGRP antibody group was found in both sexes and all age groups. Conclusions: These preliminary results suggest that CGRP inhibitors are not associated with a higher frequency of stroke in the initial months of use. This could be related to the avoidance of these medications in patients with known cardiovascular risk factors. Further analyses will be completed on vascular risk factor incidence in these populations as well as multiple regression. Prospective, long term studies are needed to confirm these results.


2020 ◽  
pp. 152483992094592 ◽  
Author(s):  
Srikanta Banerjee ◽  
Tim Radak ◽  
Jagdish Khubchandani ◽  
Patrick Dunn

Food insecurity is a significant public health problem in the United States leading to substantial social, economic, and health care–related burdens. While studies continue to estimate the prevalence of food insecurity, the long-term outcomes are not extensively explored. The purpose of this study was to assess the impact of food insecurity on mortality. We analyzed data on adults (≥ 20 years) from the 1999–2010 National Health and Nutrition Examination Survey, with mortality data obtained through 2015. Among the total study participants (n = 25,247), 17.6% reported food insecurity. Food-insecure individuals were more likely to be younger in age, minorities, poorer, with lesser education, obese, smokers, and with diabetes compared to food-secure counterparts. During a 10.2-year follow-up, among the food insecure, 821 individuals died (11%). The hazard ratio (HR) for mortality among the food insecure compared with the food secure, with adjustment for age and gender only, was 1.58; 95% confidence interval [CI: 1.25, 2.01]. The adjusted HRs for all-cause mortality, HR = 1.46, CI [1.23, 1.72], p < .001, and cardiovascular mortality, HR = 1.75, CI [1.19, 2.57], p < .01, were statistically significantly higher among food-insecure individuals, after adjustment for multiple demographic and health risk factors. Individuals who are food-insecure have a significantly higher probability of death from any cause or cardiovascular disease in long-term follow-up. Comprehensive and interdisciplinary approaches to reducing food insecurity–related disparities and health risks should be implemented. Including food insecurity in health risk assessments and addressing food insecurity as a determinant of long-term outcomes may contribute to lower premature death rates.


2020 ◽  
Vol 30 (3) ◽  
pp. 409-412
Author(s):  
Murat Surucu ◽  
İlkay Erdoğan ◽  
Birgül Varan ◽  
Murat Özkan ◽  
N. Kürşad Tokel ◽  
...  

AbstractObjective:Double-chambered right ventricle is characterised by division of the outlet portion of the right ventricle by hypertrophy of the septoparietal trabeculations into two parts. We aim to report our experiences regarding the presenting symptoms of double-chambered right ventricle, long-term prognosis, including the recurrence rate and incidence of arrhythmias after surgery.Methods:We retrospectively investigated 89 consecutive patients who were diagnosed to have double-chambered right ventricle and underwent a surgical intervention from 1995 to 2016. The data obtained by echocardiography, cardiac catheterisation, and surgical findings as well as post-operative follow-up, surgical approaches, post-operative morbidity, mortality, and cardiac events were evaluated.Results:Median age at the time of diagnosis was 2 months and mean age at the time of operation was 5.3 years. Concomitant cardiac anomalies were as follows: perimembranous ventricular septal defect (78 patients), atrial septal defect (9 patients), discrete subaortic membrane (32 patients), right aortic arch (3 patients), aortic valve prolapse and/or mild aortic regurgitation (14 patients), and left superior caval vein (2 patients). The mean follow-up period was 4.86 ± 4.6 years. In these patients, mean systolic pressure gradient in the right ventricle by echocardiography before, immediately, and long-term after surgical intervention was 66.3, 11.8, and 10.4 mmHg, respectively. There were no deaths during the long-term follow-up period. Surgical reinterventions were performed for residual ventricular septal defect (2), residual pulmonary stenosis (1), and severe tricuspid insufficiency (1).Conclusion:The surgical outcomes and prognosis of double-chambered right ventricle are favourable, recurrence and fatal arrhythmias are unlikely in long-term follow-up.


2020 ◽  
Vol 30 (5) ◽  
pp. 685-690
Author(s):  
Tomas Andri Axelsson ◽  
Jonas A Adalsteinsson ◽  
Linda O Arnadottir ◽  
Dadi Helgason ◽  
Hera Johannesdottir ◽  
...  

Abstract OBJECTIVES Our aim was to investigate the outcome of patients with diabetes undergoing coronary artery bypass grafting (CABG) surgery in a whole population with main focus on long-term mortality and complications. METHODS This was a nationwide retrospective analysis of all patients who underwent isolated primary CABG in Iceland between 2001 and 2016. Overall survival together with the composite end point of major adverse cardiac and cerebrovascular events was compared between patients with diabetes and patients without diabetes during a median follow-up of 8.5 years. Multivariable regression analyses were used to evaluate the impact of diabetes on both short- and long-term outcomes. RESULTS Of a total of 2060 patients, 356 (17%) patients had diabetes. Patients with diabetes had a higher body mass index (29.9 vs 27.9 kg/m2) and more often had hypertension (83% vs 62%) and chronic kidney disease (estimated glomerular filtration rate ≤60 ml/min/1.73 m2, 21% vs 14%). Patients with diabetes had an increased risk of operative mortality [odds ratio 2.52, 95% confidence interval (CI) 1.27–4.80] when adjusted for confounders. 5-Year overall survival (85% vs 91%, P &lt; 0.001) and 5-year freedom from major adverse cardiac and cerebrovascular events were also inferior for patients with diabetes (77% vs 82%, P &lt; 0.001). Cox regression analysis adjusting for potential confounders showed that the diagnosis of diabetes significantly predicted all-cause mortality [hazard ratio (HR) 1.87, 95% CI 1.53–2.29] and increased risk of major adverse cardiac and cerebrovascular events (HR 1.47, 95% CI 1.23–1.75). CONCLUSIONS Patients with diabetes have significantly lower survival after CABG, both within 30 days and during long-term follow-up.


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