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Internal Medicine (Tokyo, Japan) Dec 2022Ebstein's anomaly is an uncommon congenital disorder affecting the tricuspid valve. We herein report a 38-year-old woman who experienced consciousness and sensory...
Ebstein's anomaly is an uncommon congenital disorder affecting the tricuspid valve. We herein report a 38-year-old woman who experienced consciousness and sensory disturbance during treatment for heart failure caused by Ebstein's anomaly. Urgent magnetic resonance imaging and cerebral angiography demonstrated acute cerebral infarction and internal carotid artery obstruction with the development of collateral arteries. We diagnosed her with multiple cerebral infarctions due to moyamoya disease. Ebstein's anomaly concomitant with moyamoya disease is extremely rare. However, we should consider the possibility of this rare but important concurrence when treating patients with heart failure due to Ebstein's anomaly to avoid excessive diuresis and vasodilation and irreversible brain injury.
Topics: Female; Humans; Adult; Ebstein Anomaly; Moyamoya Disease; Tricuspid Valve; Heart Failure
PubMed: 35569986
DOI: 10.2169/internalmedicine.9516-22 -
Disease Markers 2022For moyamoya disease (MMD) patients who suffered an acute ischemic attack, the infarction patterns on DWI and its association with recurrent adverse cerebrovascular...
For moyamoya disease (MMD) patients who suffered an acute ischemic attack, the infarction patterns on DWI and its association with recurrent adverse cerebrovascular events (ACEs) after bypass surgery remain unknown. 327 patients who suffered an acute ischemic attack and received following revascularization surgery were retrospectively reviewed and were divided into three patterns according to the lesion number and distribution on DWI that obtained within 7 days of onset: no acute infarction (NAI), single acute infarction (SAI), and multiple acute infarctions (MAIs). We used Cox proportional hazard models to estimate hazard ratios (HR) for associations of infarction patterns and the risk of recurrent ACEs and strokes. Over a median follow-up of 41 months (IQR 26-60), there were 61 ACEs and 27 strokes. Compared to the NAI cohort, patients with SAI (HR, 2.92; 95% CI, 1.41-6.05; = 0.004) and MAIs (HR, 4.44; 95% CI, 2.10-9.41; < 0.001) were associated with higher risk of ACEs recurrences. In analysis adjusted for age and surgery modalities, the corresponding HR was 2.90 (95% CI: 1.41-5.98) for SAI and 4.10 (95% CI: 1.95-8.63) for MAIs, and this effect remained persistent on further adjustment for several potential confounders. Similar but less precise association was found in separate analysis that only takes into account stroke recurrences. Thus, different infarction patterns on DWI imply different risks of recurrent ACEs, and more attention should be paid to prevent ACEs in MMD patients with MAIs.
Topics: Humans; Infarction; Moyamoya Disease; Recurrence; Retrospective Studies; Stroke
PubMed: 35392499
DOI: 10.1155/2022/8255018 -
Revue Medicale de Liege Feb 2020Moyamoya disease is a rare cerebral vasculopathy. Disease onset is mainly sudden presenting as an ischemic stroke but also sometimes as a brain hemorraghe. Cerebral...
Moyamoya disease is a rare cerebral vasculopathy. Disease onset is mainly sudden presenting as an ischemic stroke but also sometimes as a brain hemorraghe. Cerebral angiography is the gold standard to confirm the diagnosis. Different therapeutic approaches have been described such as conservative management or endoscopic and surgical approaches. We report the case of a young patient who was diagnosed with a brain hemorraghe following a sudden loss of consciousness.
Topics: Brain; Cerebral Angiography; Humans; Moyamoya Disease; Stroke
PubMed: 32030929
DOI: No ID Found -
Neurologia Medico-chirurgica 2012This review describes the basic concepts of surgical revascularization for moyamoya disease, including direct and indirect bypass surgery. Direct bypass surgery can... (Review)
Review
This review describes the basic concepts of surgical revascularization for moyamoya disease, including direct and indirect bypass surgery. Direct bypass surgery can improve cerebral hemodynamics and reduce further ischemic events immediately after surgery, but may be technically challenging in some pediatric patients. Indirect bypass surgery is simple and has widely been used. However, its beneficial effects can be achieved 3 to 4 months after surgery, and surgical design is quite important to determine the extent of surgical collateral pathways. Combined bypass procedure, especially superficial temporal artery (STA) to middle cerebral artery anastomosis and indirect bypass, encephalo-duro-myo-arterio-pericranial synangiosis, is a safe and effective option to improve the short- and long-term outcome in patients with moyamoya disease. Alternative techniques are also described for specific cases with profound cerebral ischemia in the anterior cerebral artery or posterior cerebral artery territory. Special techniques to safely complete bypass surgery and avoid perioperative complications are presented, including methods to prevent delayed wound healing, to avoid facial nerve palsy after surgery, and to preserve the STA and middle meningeal artery during skin incision and craniotomy. Finally, the importance of careful management of patients is emphasized to reduce the incidence of perioperative complications, including ischemic stroke and hyperperfusion syndrome.
Topics: Cerebral Revascularization; Humans; Middle Cerebral Artery; Moyamoya Disease; Radiography; Temporal Arteries
PubMed: 22688064
DOI: 10.2176/nmc.52.287 -
Stroke and Vascular Neurology Dec 2021Moyamoya disease (MMD) is an increasingly recognised cause of stroke, mainly described in East Asia. China is the largest nation in Asia, but few studies reported the...
BACKGROUND AND OBJECTIVE
Moyamoya disease (MMD) is an increasingly recognised cause of stroke, mainly described in East Asia. China is the largest nation in Asia, but few studies reported the epidemiology of MMD, especially at a national level. We aimed to estimate the incidence and prevalence of MMD in China.
METHODS
We performed a population-based study using data from the national databases of Urban Basic Medical Insurance between 2013 and 2016, covering approximately 0.50 billion individuals. MMD cases were identified by diagnostic code (International Classification of Diseases, 10th Revision I67.5) or related diagnostic text.
RESULTS
A total of 1987 MMD patients (mean age 44.45±14.30 years, female-to-male ratio 1.12) were identified, representing a national crude incidence of 0.59 (95% CI: 0.49 to 0.68) and a prevalence of 1.01 (95% CI: 0.81 to 1.21) per 100 000 person-years in 2016. Rates were higher in females than in males for the incidence (0.66 vs 0.52) and prevalence (1.05 vs 0.90). And the age-specific rates showed a bimodal distribution, with the highest peak in middle-aged group and the second peak in child group.
CONCLUSIONS
Our results confirm that MMD is relatively common in East Asians, but the rates in China were lower than those in other East Asian countries such as Japan and Korea. The unique epidemiological features, including a relatively weak female predominance and a shift in the highest peak of incidence from children to adults, revealed new sight into MMD. Further research is expected to explore the potential pathogenesis of MMD.
Topics: Adult; Child; China; Female; Humans; Incidence; Male; Middle Aged; Moyamoya Disease; Prevalence; Retrospective Studies
PubMed: 33941642
DOI: 10.1136/svn-2021-000909 -
Neurology India 2023
Topics: Humans; Moyamoya Disease
PubMed: 36861609
DOI: 10.4103/0028-3886.370480 -
Postgraduate Medical Journal Sep 1997Moyamoya disease is a rare cerebrovascular condition of uncertain aetiology commonly affecting young persons. The disease is mainly seen in Japanese patients. We report... (Review)
Review
Moyamoya disease is a rare cerebrovascular condition of uncertain aetiology commonly affecting young persons. The disease is mainly seen in Japanese patients. We report two cases of moyamoya disease in Caucasian women and review the postulated aetiological factors and associated conditions as well as the spectrum of invasive and non-invasive imaging modalities useful in the diagnosis and follow-up of the disease, with particular reference to the developing role of magnetic resonance imaging and angiography.
Topics: Adolescent; Angiography; Female; Humans; Magnetic Resonance Imaging; Middle Aged; Moyamoya Disease; Tomography, X-Ray Computed
PubMed: 9373593
DOI: 10.1136/pgmj.73.863.549 -
International Journal of Medical... 2015During the onset of Moyamoya disease (MMD), progressive occlusion occurs at the end of the intracranial internal carotid artery, and compensatory net-like abnormal... (Review)
Review
During the onset of Moyamoya disease (MMD), progressive occlusion occurs at the end of the intracranial internal carotid artery, and compensatory net-like abnormal vessels develop in the skull base, generating the corresponding clinical symptoms. MMD can affect both children and adults, but MMD in pediatric patients exhibits distinct clinical features, and the treatment prognoses are different from adult patients. Children are the group at highest risk for MMD. In children, the disease mainly manifests as ischemia, while bleeding is the primary symptom in adults. The pathogenesis of MMD in children is still unknown, and some factors are distinct from those in adults. MMD in children could result in progressive, irreversible nerve functional impairment, and an earlier the onset corresponds to a worse prognosis. Therefore, active treatment at an early stage is highly recommended. The treatment methods for MMD in children mainly include indirect and direct surgeries. Indirect surgeries mainly include multiple burr-hole surgery (MBHS), encephalomyosynangiosis (EMS), and encephaloduroarteriosynangiosis (EDAS); direct surgeries mainly include intra- and extracranial vascular reconstructions that primarily consist of superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis. Indirect surgery, as a treatment for MMD in children, has shown a certain level of efficacy. However, a standard treatment approach should combine both indirect and direct procedures. Compared to MMD in adults, the treatment and prognosis of MMD in children has higher clinical significance. If the treatment is adequate, a satisfactory outcome is often achieved.
Topics: Adult; Cerebral Revascularization; Child; Hemorrhage; Humans; Moyamoya Disease; Prognosis; Research
PubMed: 26180513
DOI: 10.7150/ijms.11719 -
Journal of Cerebral Blood Flow and... Apr 2023The influence of hypoperfusion on cognition in patients with Moyamoya disease (MMD) is unclear. This study investigated cognitive function changes in MMD patients...
The influence of hypoperfusion on cognition in patients with Moyamoya disease (MMD) is unclear. This study investigated cognitive function changes in MMD patients without stroke and illustrated the relationship between cognitive impairment and hypoperfusion. We prospectively performed a structured battery of seven neurocognitive tests on 115 adult MMD patients without stroke and 82 healthy controls. Hemodynamic assessment was performed using dynamic susceptibility contrast-enhanced MRI. The best subset regression (BSR) strategy was used to identify risk factors. Global cognition (MoCA), speed of information processing (TMT-A), executive function (TMT-B), visuospatial function (CDT), and verbal memory (CAVLT) were significantly poorer in MMD patients without stroke than in healthy controls. The TMT-B score significantly correlated with cerebral blood flow (CBF) in the bilateral lateral frontal lobes, centrum semiovale, and temporal lobes. The TMT-A and CAVLT scores significantly correlated with CBF in the left centrum semiovale (L-CSO) and temporal lobes. According to the BSR results, age, education, white matter lesions, and hypoperfusion of the L-CSO were risk factors for cognitive impairment. Hypoperfusion leads to multiple cognitive impairments in MMD patients without stroke. The perfusion of particular areas may help evaluate the cognitive function of MMD patients and guide therapeutic strategies.
Topics: Adult; Humans; Moyamoya Disease; Stroke; Cognition; Cognitive Dysfunction
PubMed: 36397212
DOI: 10.1177/0271678X221140349 -
Revista de Neurologia Oct 2021Moyamoya disease is caused because of progressive occlusion of the arterial circle of Willis, leading to a compensatory net-like abnormal vessels development. The... (Observational Study)
Observational Study
INTRODUCTION
Moyamoya disease is caused because of progressive occlusion of the arterial circle of Willis, leading to a compensatory net-like abnormal vessels development. The objective is to describe the number of cases in our center (tertiary hospital).
PATIENTS AND METHODS
Retrospective study. Revision of pediatric medical histories diagnosed of moyamoya disease or moyamoya syndrome (in case of predisposing disease) between 2005 and 2018. Demographic variables were collected, related to diagnosis, risk factors, treatment, and follow-up.
RESULTS
Seven cases were collected with a median age of 6 years and an equitable distribution by sex. Five associated predisposing pathologies (Down syndrome, neurofibromatosis, sickle cell disease, Behcet). The main clinical diagnosis was neurological focus (five cases), followed by epileptic seizures (four), and headache (two). One was asymptomatic at diagnosis. Six strokes were documented, five of them were isquemic. The arteriography (goldstardard) was made in five patients. Five presented bilateral involvement of the vessels, the internal carotid arteries and the middle cerebral arteries were the most affected. Six received acetylsalicylic acid treatment and five of them required antiepileptic drugs. Revascularization surgery (encephaloduroarteriomyosinangiosis) was performed in four patients, and in one, strokes persisted. The most prevalent sequelae were hemiparesis and psychomotor retardation.
CONCLUSIONS
The risk factors presented in our patients match to those described in the literature. The symptoms at the onset can be diverse and ischemic strokes predominate in our series. Revascularization surgery was effective in more than half of the cases. Subsequent follow-up is necessary to assess complications and sequelae.
Topics: Adolescent; Child; Child, Preschool; Female; Humans; Infant; Male; Moyamoya Disease; Retrospective Studies
PubMed: 34617579
DOI: 10.33588/rn.7308.2020624