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Free access

Edward R. Smith, Giuseppe Lanzino, Gary K. Steinberg, and Bin Xu

Free access

Chidinma M. Wilson, Nolan J. Brown, and Donald K. E. Detchou

Free access

Bhanu Jayanand Sudhir, Arun Gowda Keelara, Easwer Harihara Venkat, Ken Kazumata, and Ananthalakshmy Sundararaman

OBJECTIVE

Moyamoya angiopathy (MMA) affects the distal internal carotid artery and is designated as moyamoya disease (MMD) when predisposing conditions are absent, or moyamoya syndrome (MMS) when it occurs secondary to other causes. The authors aimed to investigate the reason for this anatomical site predilection of MMA. There is compelling evidence to suggest that MMA is a phenomenon that occurs due to stereotyped mechanobiological processes. Literature regarding MMD and MMS was systematically reviewed to decipher a common pattern relating to the development of MMA.

METHODS

A systematic review was conducted to understand the pathogenesis of MMA in accordance with PRISMA guidelines. PubMed MEDLINE and Scopus were searched using “moyamoya” and “pathogenesis” as common keywords and specific keywords related to six identified key factors. Additionally, a literature search was performed for MMS using “moyamoya” and “pathogenesis” combined with reported associations. A progressive search of the literature was also performed using the keywords “matrix metalloprotease,” “tissue inhibitor of matrix metalloprotease,” “endothelial cell,” “smooth muscle cell,” “cytokines,” “endothelin,” and “transforming growth factor” to infer the missing links in molecular pathogenesis of MMA. Studies conforming to the inclusion criteria were reviewed.

RESULTS

The literature search yielded 44 published articles on MMD by using keywords classified under the six key factors, namely arterial tortuosity, vascular angles, wall shear stress, molecular factors, blood rheology/viscosity, and blood vessel wall strength, and 477 published articles on MMS associations. Information obtained from 51 articles that matched the inclusion criteria and additional information derived from the progressive search mentioned above were used to connect the key factors to derive a network pattern of pathogenesis.

CONCLUSIONS

Based on the available literature, the authors have proposed a unifying theory for the pathogenesis of MMA. The moyamoya phenomenon appears to be the culmination of an interplay of vascular anatomy, hemodynamics, rheology, blood vessel wall strength, and a plethora of intricately linked mechanobiological molecular mediators that ultimately results in the mechanical process of occlusion of the blood vessel, stimulating angiogenesis and collateral blood supply in an attempt to perfuse the compromised brain.

Free access

Gueliz Acker, Davide Giampiccolo, Kerstin Rubarth, Robert Mertens, Anna Zdunczyk, Juliane Hardt, Daniel Jussen, Heike Schneider, Tizian Rosenstock, Vera Mueller, Thomas Picht, and Peter Vajkoczy

OBJECTIVE

Motor cortical dysfunction has been shown to be reversible in patients with unilateral atherosclerotic disease after cerebral revascularization. Moyamoya vasculopathy (MMV) is a rare bilateral stenoocclusive cerebrovascular disease. The aim of this study was to analyze the corticospinal excitability and the role of bypass surgery in restoring cortical motor function in patients by using navigated transcranial magnetic stimulation (nTMS).

METHODS

Patients with bilateral MMV who met the criteria for cerebral revascularization were prospectively included. Corticospinal excitability, cortical representation area, and intracortical inhibition and facilitation were assessed by nTMS for a small hand muscle (first dorsal interosseous) before and after revascularization. The clinically and/or hemodynamically more severely affected hemisphere was operated first as the leading hemisphere. Intra- and interhemispheric differences were analyzed before and after direct or combined revascularization.

RESULTS

A total of 30 patients with bilateral MMV were examined by nTMS prior to and after revascularization surgery. The corticospinal excitability was higher in the leading hemisphere compared with the non-leading hemisphere prior to revascularization. This hyperexcitability was normalized after revascularization as demonstrated in the resting motor threshold ratio of the hemispheres (preoperative median 0.97 [IQR 0.89–1.08], postoperative median 1.02 [IQR 0.94–1.22]; relative effect = 0.61, p = 0.03). In paired-pulse paradigms, a tendency for a weaker inhibition of the leading hemisphere was observed compared with the non-leading hemisphere. Importantly, the paired paradigm also demonstrated approximation of excitability patterns between the two hemispheres after surgery.

CONCLUSIONS

The study results suggested that, in the case of a bilateral chronic ischemia, a compensation mechanism between both hemispheres seemed to exist that normalized after revascularization surgery. A potential role of nTMS in predicting the efficacy of revascularization must be further assessed.

Free access

Joseph H. Garcia, Ramin A. Morshed, Ethan A. Winkler, Yi Li, Christine K. Fox, Heather J. Fullerton, Caleb Rutledge, Angad S. Beniwal, Michael T. Lawton, Adib A. Abla, Nalin Gupta, and Steven W. Hetts

OBJECTIVE

Moyamoya is a progressive arteriopathy that predisposes patients to stroke due to stenosis of the intracranial internal carotid arteries and their proximal branches. Despite the morbidity caused by this condition, the ability to accurately predict prognosis for individual patients remains challenging. The goal of this study was to develop a systematic scoring method based on parenchymal findings on preoperative brain MRI to predict long-term outcomes for surgically treated pediatric patients with moyamoya.

METHODS

A retrospective surgical cohort of pediatric patients (≤ 18 years of age at the time of the initial surgery) with moyamoya from a single center were studied. Radiological variables with existing correlations between outcomes in moyamoya or other vascular diseases were chosen to score preoperative MRI based on easily defined parenchymal findings that could be rapidly assessed and used to make a numeric score. Calculated scores were correlated with clinical outcome measures using the Pearson correlation coefficient and area under the receiver operating characteristic curve (AUROC).

RESULTS

A total of 35 children with moyamoya disease or moyamoya syndrome were included in the study, with a median follow-up time of 2.6 years from the time of surgery. The pediatric moyamoya MRI score (PMMS) consists of ischemic changes (0–2; 0 = none, 1 = focal, 2 = diffuse), encephalomalacia (0–2; 0 = none, 1 = focal, 2 = diffuse), and hemorrhage (0–1; 0 = not present, 1 = present). PMMSs were highly correlated with pediatric modified Rankin Scale scores at the last follow-up (r = 0.7, 95% CI 0.44–0.84; p < 0.001) as a six-point scale, and when dichotomized (AUROC = 0.85).

CONCLUSIONS

The PMMS was found to be a simple tool based on preoperative MRI data that could be quickly and easily calculated and correlated with disability. This scoring method may aid future development of predictive models of outcomes for children with moyamoya disease and moyamoya syndrome.

Free access

Qian-Nan Wang, Xiang-Yang Bao, Zheng-Xing Zou, Xiao-Peng Wang, Qian Zhang, De-Sheng Li, Ya-Qun Zhao, and Lian Duan

OBJECTIVE

This prospective study was designed to confirm the role of atorvastatin in collateral circulation formation induced by encephaloduroarteriosynangiosis (EDAS) in patients with moyamoya disease (MMD).

METHODS

Patients who were diagnosed with MMD at the Department of Neurosurgery in the Fifth Medical Center of Chinese PLA General Hospital, Beijing, China, between June 2017 and May 2018 were included. Blood samples were obtained from an antecubital vein and were analyzed using flow cytometry. Endothelial progenitor cells (EPCs) were defined as CD34brCD133+CD45dimKDR+. All patients included in the study underwent EDAS. Patients voluntarily chose whether to undergo atorvastatin treatment after EDAS. The correlation between atorvastatin and good postoperative collateral circulation was evaluated.

RESULTS

A total of 106 patients with MMD were included in this study. Fifty-three patients (50%) received atorvastatin treatment. The baseline characteristics did not display statistically significant differences between the atorvastatin-treated and non-atorvastatin groups. Seventy-eight (42.9%) of the 182 hemispheres investigated postoperatively were classified as grade A collateral circulation, 47 (25.8%) as grade B, and 57 (31.3%) as grade C. Multivariate analysis revealed that only atorvastatin was significantly correlated with good collateral circulation after EDAS (p = 0.041).

CONCLUSIONS

The results of this prospective clinical trial have indicated that atorvastatin administered at 20 mg daily is safe and effective for the formation of postoperative collateral induced by EDAS.

Free access

Eitaro Ishisaka, Atsushi Watanabe, Yasuo Murai, Kazutaka Shirokane, Fumihiro Matano, Atsushi Tsukiyama, Eiichi Baba, Shunsuke Nakagawa, Tomonori Tamaki, Takayuki Mizunari, Rokuya Tanikawa, and Akio Morita

OBJECTIVE

Quasi-moyamoya disease (QMMD) is moyamoya disease (MMD) associated with additional underlying diseases. Although the ring finger protein 213 (RNF213) c.14576G>A mutation is highly correlated with MMD in the Asian population, its relationship to QMMD is unclear. Therefore, in this study the authors sought to investigate the RNF213 c.14576G>A mutation in the genetic diagnosis and classification of QMMD.

METHODS

This case-control study was conducted among four core hospitals. A screening system for the RNF213 c.14576G>A mutation based on high-resolution melting curve analysis was designed. The prevalence of RNF213 c.14576G>A was investigated in 76 patients with MMD and 10 patients with QMMD.

RESULTS

There were no significant differences in age, sex, family history, and mode of onset between the two groups. Underlying diseases presenting in patients with QMMD were hyperthyroidism (n = 6), neurofibromatosis type 1 (n = 2), Sjögren’s syndrome (n = 1), and meningitis (n =1). The RNF213 c.14576G>A mutation was found in 64 patients (84.2%) with MMD and 8 patients (80%) with QMMD; no significant difference in mutation frequency was observed between cohorts.

CONCLUSIONS

There are two forms of QMMD, one in which the vascular abnormality is associated with an underlying disease, and the other in which MMD is coincidentally complicated by an unrelated underlying disease. It has been suggested that the presence or absence of the RNF213 c.14576G>A mutation may be useful in distinguishing between these disease types.

Free access

Fumiaki Kanamori, Kinya Yokoyama, Akinobu Ota, Kazuhiro Yoshikawa, Sivasundaram Karnan, Mikio Maruwaka, Kenzo Shimizu, Shinji Ota, Kenji Uda, Yoshio Araki, Sho Okamoto, Satoshi Maesawa, Toshihiko Wakabayashi, and Atsushi Natsume

OBJECTIVE

Moyamoya disease (MMD) is a rare cerebrovascular disease characterized by progressive occlusion of the internal carotid artery and the secondary formation of collateral vessels. Patients with MMD have ischemic attacks or intracranial bleeding, but the disease pathophysiology remains unknown. In this study, the authors aimed to identify a gene expression profile specific to the intracranial artery in MMD.

METHODS

This was a single-center, prospectively sampled, retrospective cohort study. Microsamples of the middle cerebral artery (MCA) were collected from patients with MMD (n = 11) and from control patients (n = 9). Using microarray techniques, transcriptome-wide analysis was performed.

RESULTS

Comparison of MCA gene expression between patients with MMD and control patients detected 62 and 26 genes whose expression was significantly (p < 0.001 and fold change > 2) up- or downregulated, respectively, in the MCA of MMD. Gene set enrichment analysis of genes expressed in the MCA of patients with MMD revealed positive correlations with genes involved in antigen processing and presentation, the dendritic cell pathway, cytokine pathway, and interleukin-12 pathway, and negative correlations with genes involved in oxidative phosphorylation and DNA repair. Microarray analysis was validated by quantitative polymerase chain reaction.

CONCLUSIONS

Transcriptome-wide analysis showed upregulation of genes for immune responses and downregulation of genes for DNA repair and oxidative phosphorylation within the intracranial artery of patients with MMD. These findings may represent clues to the pathophysiology of MMD.

Open access

Ayako Takarada, Kiyoyuki Yanaka, Kuniyuki Onuma, Kazuhiro Nakamura, Nobuyuki Takahashi, and Eiichi Ishikawa

BACKGROUND

Aplastic or twig-like middle cerebral artery (Ap/T-MCA) is a congenital MCA anomaly. It may present with symptoms of both hemorrhage and ischemia, similar to moyamoya disease, and hemodynamic stress may play an essential role in the development of symptoms in both clinical entities. The optimal treatment remains controversial in symptomatic patients with Ap/T-MCA. This report discussed the treatment method for a patient with Ap/T-MCA with unruptured aneurysms who presented with intraventricular hemorrhage (IVH) treated by aneurysm clipping and bypass surgery.

OBSERVATIONS

In a 46-year-old woman with a sudden headache, computed tomography showed left IVH. Magnetic resonance angiography showed a left MCA aneurysm and MCA trunk stenosis. Three-dimensional angiography demonstrated a plexiform arterial network and multiple aneurysms arising from the MCA and in the plexiform network, leading to the diagnosis of Ap/T-MCA harboring unruptured aneurysms. The patient was successfully treated by craniotomy with aneurysm clipping and bypass surgery to prevent further intracranial hemorrhages and/or aneurysm rupture.

LESSONS

Especially in cases such as Ap/T-MCA, in which hemodynamic stress has a significant effect, the optimal treatment method should be based on vascular morphology and the impact of hemodynamic stress.

Open access

Arata Nagai, Hidenori Endo, Kenichi Sato, Tomohiro Kawaguchi, Hiroki Uchida, Shunsuke Omodaka, Yasushi Matsumoto, and Teiji Tominaga

BACKGROUND

Arteriovenous malformation (AVM) of the trigeminal nerve root (TNR) is a rare subtype of the lateral pontine AVM. Most of them are diagnosed when they bleed or exert trigeminal neuralgia. Venous congestive edema is a rare phenomenon caused by TNR AVMs.

OBSERVATIONS

An 82-year-old man was admitted with progressive limb weakness and dysphasia. Magnetic resonance imaging (MRI) revealed extensive edema of the medulla oblongata and the upper cervical cord with signal flow void at the C3 anterior spinal cord. Vertebral angiography revealed a small nidus fed mainly by the pontine perforating arteries (PPAs). The anterior pontomesencephalic vein (AMPV) was dilated, functioning as the main drainage route. This suggests that venous hypertension triggered the brainstem and upper cervical cord edema. MRI with gadolinium enhancement showed that the nidus was located around the right TNR. Because the nidus sat extrinsically on the pial surface of the right TNR’s base, microsurgical obliteration with minimum parenchymal injury was achieved. Postoperative MRI showed disappearance of the brainstem and cervical cord edema with improved clinical symptoms.

LESSONS

TNR AVM is rarely associated with brainstem and upper cervical cord edema caused by venous hypertension of the congestive drainage system.