scholarly journals Moyamoya Disease Worldwide-Global Burden East and West

2021 ◽  
Author(s):  
Man Mohan Mehndiratta ◽  
Ishu Goyal ◽  
Vasundhara Aggarwal ◽  
Natasha Singh Gulati

The Moyamoya disease [MMD] is a cereberovascular disorder characterized by progressive stenosis of intracranial internal carotid arteries and compensatory collateral formation at the base of the brain, mainly around the circle of Willis. When no particular associated risk factors can be identified, it is termed as Moyamoya disease. However, it may be associated with other neurological and extra- neurological disorders where it is termed as Moyamoya syndrome [MMS]. The condition is predominantly seen in East Asia and has bimodal age of distribution. The clinical manifestations are also age dependant with ischemia predominating in childhood and hemorrhagic manifestations being more common in adults. The pathogenesis is not entirely known, but genetic susceptibility is believed to be an important predisposing factor. The Suzuki staging system is most widely used for evaluation and staging of Moyamoya disease. The gold standard diagnostic modality is cerebral angiography but magnetic resonance imaging [MRA] has also been employed for diagnosis. Treatment is primarily surgical revascularization which is of 3 types: direct, indirect or combined revascularization. Although the role of revascularization surgery has been well established for ischemic MMD, the ideal surgical approach and the role of surgery in hemorrhagic MMD remains controversial.

CNS Spectrums ◽  
2004 ◽  
Vol 9 (7) ◽  
pp. 523-529 ◽  
Author(s):  
Palmiero Monteleone ◽  
Antonio DiLieto ◽  
Eloisa Castaldo ◽  
Mario Maj

AbstractLeptin is an adipocyte-derived hormone, which is involved predominantly in the long-term regulation of body weight and energy balance by acting as a hunger suppressant signal to the brain. Leptin is also involved in the modulation of reproduction, immune function, physical activity, and some endogenous endocrine axes. Since anorexia nervosa (AN) and bulimia nervosa (BN) are characterized by abnormal eating behaviors, dysregulation of endogenous endocrine axes, alterations of reproductive and immune functions, and increased physical activity, extensive research has been carried out in the last decade in order to ascertain a role of this hormone in the pathophysiology of these syndromes. In this article, we review the available data on leptin physiology in patients with eating disorders. These data support the idea that leptin is not directly involved in the etiology of AN or BN. However, malnutrition-induced alterations in its physiology may contribute to the genesis and/or the maintenance of some clinical manifestations of AN and BN and may have an impact on the prognosis of AN.


2019 ◽  
Vol 34 (9) ◽  
pp. 517-529 ◽  
Author(s):  
Ramana Appireddy ◽  
Manish Ranjan ◽  
Bryce A. Durafourt ◽  
Jay Riva-Cambrin ◽  
Walter J. Hader ◽  
...  

Moyamoya disease is a chronic progressive cerebrovascular occlusive disease of the terminal portion of the internal carotid arteries associated with an acquired abnormal vascular network at the base of the brain, often leading to ischemic or hemorrhagic stroke. Moyamoya disease is a relatively common cause of pediatric stroke with a specific racial and well-identified clinical and imaging phenotype. Moyamoya disease is more prevalent in East Asian countries compared with other geographic regions with a higher incidence of familial cases and clinically more aggressive form. Moyamoya disease is one of the few causes of stroke that is amenable to effective surgical revascularization treatment. There are various surgical options available for revascularization, including the direct, indirect, or combined bypass techniques, each with variable responses. However, due to the heterogeneity of the diseases, different clinical course, geographical variables associated with the disease, and availability of a wide variety of surgical revascularization procedures, optimal selection of a surgical candidate and the surgical technique becomes challenging, particularly in the pediatric population. This brief review presents pertinent literature of clinical options for the diagnosis and surgical treatment of moyamoya disease in children.


2013 ◽  
Vol 119 (4) ◽  
pp. 961-965 ◽  
Author(s):  
Tomohito Hishikawa ◽  
Koji Tokunaga ◽  
Kenji Sugiu ◽  
Isao Date

Object There is no description of the change in the posterior cerebral artery (PCA) in the diagnostic criteria of moyamoya disease (MMD). However, PCAs are often involved in the clinical setting, and an understanding of the significance of PCA lesions is therefore of great importance when evaluating the disease progression and predicting prognosis. The aim of this study was to assess the difference in posterior circulation involvement in pediatric and adult patients with MMD. Methods The records of 120 consecutive patients with MMD were reviewed. The clinical manifestations at diagnosis were evaluated on the basis of symptoms and CT and MRI findings. The degree of steno-occlusive internal carotid artery (ICA) lesions and the existence of steno-occlusive PCA lesions were evaluated by observing a total of 240 ICAs and PCAs on angiography. Angiographic correlation between anterior and posterior circulation was assessed in pediatric and adult patients with MMD. Results Seventeen (26%) of 66 pediatric patients and 18 (33%) of 54 adult patients exhibited steno-occlusive PCA lesions. There was no significant difference in the prevalence of PCA lesions between pediatric and adult patients with MMD (p = 0.36). The prevalence of infarction in pediatric and adult patients with PCA involvement was significantly higher than that in pediatric and adult patients without PCA involvement (p = 0.0003 and p = 0.003, respectively). There was no significant difference in the distribution of infarction areas between pediatric and adult patients with PCA involvement (p = 0.62). On the basis of the staging system used, steno-occlusive lesions in ICAs ipsilateral to PCAs with lesions were in significantly advanced stages compared with lesions in ICAs ipsilateral to PCAs without lesions in both pediatric and adult cases (p < 0.0001 and p = 0.0008, respectively). Pediatric patients had less advanced steno-occlusive lesions in ICAs ipsilateral to PCAs with lesions compared with adults (p < 0.05). Conclusions The clinical significance of posterior circulation involvement in MMD was similar between pediatric and adult patients. The only significant difference was that less advanced ICA lesions could complicate posterior circulation involvement in pediatric patients.


2016 ◽  
Vol 91 ◽  
pp. 473-482 ◽  
Author(s):  
Shouhei Noshiro ◽  
Takeshi Mikami ◽  
Katsuya Komatsu ◽  
Aya Kanno ◽  
Rei Enatsu ◽  
...  

2010 ◽  
Vol 5 (3) ◽  
pp. 297-301 ◽  
Author(s):  
Ho Jun Seol ◽  
Duk Chul Shin ◽  
Yoo Seok Kim ◽  
Eun Bo Shim ◽  
Seung-Ki Kim ◽  
...  

Object Bilateral intimal thickening of the distal internal carotid arteries (ICAs) and the development of many collateral vessels in the base of the brain characterize moyamoya disease (MMD). Although the etiology of and the reason why MMD is limited to the major intracranial vessels remain unclear, flow dynamics, such as shear stress, may be related to its smooth-muscle cell migration. Therefore, this study was performed to determine the local hemodynamic factor, which concerns the predominance of specific anatomical sites, such as the distal ICA in the early stage and the proximal posterior cerebral artery (PCA) in the advanced stage of MMD. Methods The authors simulated the hemodynamics in the circle of Willis using computational models of 2D geometries of the distal ICA and PCA. A finite-element commercial package, automatic dynamics incremental nonlinear analysis (ADINA), was used to simulate blood flow in these arteries. Results Numerical results demonstrated that shear stress was relatively low at the ICA region. The distribution of shear stress was related to the predisposing area of MMD. Conclusions Diminished shear stress may promote stenosis of the distal ICA, which is a major pathological region in MMD.


2021 ◽  
Author(s):  
Raúl Espert ◽  
Marien Gadea

Moyamoya disease (MMD) is an occlusive cerebrovascular disease characterized by progressive stenosis or occlusion in the terminal portion of the bilateral internal carotid arteries, and affect both children and adults. In this pathology, which presents itself through ischemia or cerebral hemorrhage, an unusual compensatory vascular network (moyamoya vessels) develops at the brain´s base in the form of collateral channels. MMD can present clinically as hemiparesis, dysarthria, aphasia, headache, seizures, visual deficits, syncope, or personality changes. Neuropsychologically, and even in the absence of obvious stroke, patients often present impaired attention, memory, behavior, and executive functions. This book chapter reviews the current literature regarding the neuropsychological deficits of MMD both in children and adults.


Author(s):  
I. N. Semenenya

The article reviews the information on the problem of psychosomatic disorders (PSDs) widespread in human population. Attention is drawn to the insufficient awareness of general practitioners in this field of pathology. The true-life situations are considered that the cause development of PSDs and the role of negative thoughts and an emotional factor in their development. A general scheme of the PSD pathogenesis is suggested, which is based on the shift in the balance of the excitation and inhibition processes in CNS towards the prevalence of the former ones. Suppression of the inhibitory processes results in the decrease of the excitation thresholds of different afferent systems and in the development of general hyperesthesia that causes the organism hyperreactivity to the external and internal actions, including the subthreshold ones and stimuli. This underlies the mechanism of PSD clinical manifestations. A possible role of CNS stagnant centers of excitement in the development of PSDs and their ability to migrate in the brain, which reflects the migration of PSD symptoms in the organism, are viewed. Mental, neurologic, cardiovascular, respiratory, digestive, urination, sexual and skin manifestations of PSDs are discussed. General approaches to treating PSD patients are considered. The fundamental importance of the mood factor in the PSD development and alleviation is substantiated.


2018 ◽  
pp. 67-72
Author(s):  
V. M. Makhov ◽  
A. A. Balakhonov ◽  
M. A. Isaikina ◽  
Yu. A. Doronina

The article shows the dynamics of evaluation of prognostic factors of irritable bowel syndrome. Evolution of the views indicates an increasing evaluation of inflammation and the role of markers of inflammation of cytokines, especially alpha-TNF in clinical manifestations. The rationale for using a complex drug based on affinity purified release-active antibodies to alpha-TNF, to the brain-specific protein S-100 and histamine-colourant is presented.


2021 ◽  
Vol 19 ◽  
Author(s):  
Xin Zhang ◽  
Weiping Xiao ◽  
Qing Zhang ◽  
Ding Xia ◽  
Peng Gao ◽  
...  

: Moyamoya disease (MMD) is a chronic cerebrovascular disease characterized by progressive stenosis of the arteries of the circle of Willis, with the formation of the collateral vascular network at the base of the brain. Its clinical manifestations are complicated. Numerous studies have attempted to clarify the clinical features of MMD, including its epidemiology, genetic characteristics, and pathophysiology. With the development of neuroimaging techniques, various neuroimaging modalities with different advantages have deepened the understanding of MMD in structural, functional, spatial, and temporal dimensions. At present, the main treatment for MMD focuses on neurological protection, cerebral blood flow reconstruction, and neurological rehabilitation, such as pharmacological treatment, surgical revascularization, and cognitive rehabilitation. In this review, we discuss recent progress in understanding the clinical features, neuroimaging evaluation, and treatment of MMD.


Author(s):  
Bogdan Alexandru Gheban ◽  
Ioana Andreea Rosca ◽  
Maria Crisan

Introduction. The pineal gland is a photo-neuro-endocrine organ situated inside the brain, that secretes serotonin, melatonin and N,N-dymethyltriptamine. This narrative review will address the latest information gathered on this function of the gland as well as the unknown roles it may have. The different histological and pathological findings of the pineal gland have demonstrated a role in clinical manifestations of numerous endocrine, neurological and psychiatric pathologies. Materials. For this narrative review we used the NCBI website database PubMed. The search terms were “Pineal Gland” AND/OR “histology, melatonin, DMT, pathology”. Total number of articles included were 86. Results: We have reviewed physiological information of melatonin and DMT, anatomical, histological and histopathological information on the pineal gland and its role in endocrine, neurological and psychiatric pathology. Conclusion. The role of melatonin in immunity and its potential therapeutic effects show promising potential for further research. DMT seems to have a role in psychiatric pathology and potential therapeutic effects. Proper tumoral screening and diagnostic protocol are required.


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