Clinical features of ten Japanese children with cerebrovascular Moyamoya disease are reported and non-Japanese paediatric cases of the disease are reviewed from the world literature. The most common initial manifestations were headache in four cases (40%), motor deficit and convulsion in three cases (30%). As their recurrent and/or residual symptoms, eight children (80%) developed motor deficit consisting of hemiplegia in five cases and paresis or weakness of the extremities in three cases, and four (40%) had headaches. The mode of presentation in our cases was similar to that of non-Japanese cases, in addition to a female preponderance. Electroencephalographic findings of prominent high voltage delta bursts following hyperventilation and slowness of returning to the normal pattern, seen in all hyperventilated cases, is one of the features. Of 15 carotid arteries visualised in ten patients, sites of occlusion or stenosis were seen between the bifurcation of the posterior communicating artery and that of the anterior cerebral artery or the middle cerebral artery in 13 arteries, with a vascular network in the basal ganglia. This study suggests that if hyperventilation procedures produce prominent high voltage delta bursts during electroencephalography in children with headache and/or motor deficit, cerebrovascular disease, especially Moyamoya disease, should be suspected.

Cerebrovascular Moyamoya disease.

Background A case of progressive irradiation-induced cerebral vasculopathy with abnormal netlike vessels and transdural anastomoses (moyamoya syndrome) is presented. Radiological findings in an additional 40 cases reported in the literature are analyzed, and their clinical relevance is discussed. Case Description A 19-year-old woman presented with recurrent ischemic brain lesions after radiation therapy for treatment of a craniopharyngioma during childhood. Cerebral angiography 6 and 12 years after completion of radiation therapy revealed progressive cerebral arterial occlusive disease involving the internal carotid artery on either side of the circle of Willis, with abnormal netlike vessels and transdural anastomoses (moyamoya syndrome). Conclusions Extensive similarities between irradiation-induced cerebral vasculopathy and primary moyamoya syndrome (Nishimoto’s disease) support the notion that both disorders share common pathophysiological mechanisms. The occurrence of moyamoya-like vascular changes ma...

Progressive Cerebral Occlusive Disease After Radiation Therapy

Background and Purpose—Renal artery lesions in moyamoya disease have been described sporadically in several case reports. The purpose of this study is to evaluate the angiographic findings of renal artery lesions in moyamoya disease and to determine the prevalence of renal artery lesions in patients with moyamoya disease. Methods—Eighty-six consecutive patients with idiopathic moyamoya disease were prospectively examined with both cerebral angiography and abdominal aortography. The findings of abdominal aortography were reviewed for the presence and appearance of renal artery lesions and compared with the clinical data and cerebral angiographic findings. Results—Of 86 patients with idiopathic moyamoya disease, 7 patients (8%) were found to have renal artery lesions. Six patients (7%) had stenosis in the renal artery, and 1 patient (1%) had a small saccular aneurysm in the renal artery. Two patients (2%) with a marked renal artery stenosis presented with renovascular hypertension, which resulted in an intr...

https://www.ahajournals.org/doi/pdf/10.1161/01.STR.31.3.733

Renal Artery Lesions in Patients With Moyamoya Disease Angiographic Findings

BACKGROUND AND PURPOSE: Encephaloduroarteriosynangiosis (EDAS) has become the main treatment for moyamoya disease, a chronically progressive cerebrovascular occlusive disease in children. We aimed to assess the utility of perfusion-weighted MR imaging for evaluating hemodynamic changes before and after EDAS. METHODS: Thirteen patients with angiographically confirmed moyamoya disease who underwent EDAS were investigated, and results were compared with those of a control group (n = 5). Perfusion MR imaging was performed before and after EDAS by using a T2*-weighted contrast material-enhanced technique. Relative cerebral blood volume (rCBV) and time to peak enhancement (TTP) maps were calculated. Relative ratios of rCBV and TTP in the middle cerebral artery (MCA) and basal ganglia were measured and compared with those of the posterior cerebral artery (PCA). Changes in hemodynamic parameters between pre- and post-EDAS perfusion maps were investigated. RESULTS: The mean rCBV ratio of MCA to PCA in the patient group was slightly higher than that in the control group, without statistical significance. All 13 patients showed a delayed TTP in the MCA before EDAS compared with the control group (P = .0006), and the TTP after EDAS was significantly reduced (P = .0002). In the basal ganglia, shortening of the TTP was demonstrated before EDAS, but no significant change was observed after EDAS. CONCLUSION: Perfusion-weighted MR imaging can be applied for evaluating postoperative changes in cerebral blood flow in moyamoya disease. Shortening of the TTP in the MCA of the hemisphere operated on is a marker for the development of collateral circulation from the external carotid artery to the internal carotid artery.

http://www.ajnr.org/content/ajnr/24/4/741.full.pdf

Postoperative evaluation of moyamoya disease with perfusion-weighted MR imaging: initial experience

A case is reported with occlusion and stenosis of the internal carotid arteries in association with basal telangiectasia. Fifteen years after postoperative irradiation for an optic glioma, radiological signs typical of the moyamoya syndrome were observed. Radiation therapy is discussed as the cause of the vascular damage in this case.

Moyamoya syndrome as a complication of radiation therapy: Case report

The authors conclude, on the basis of personal cases and despite several unanswered questions, that the angiographic picture known as Nishimoto's disease, covers two distinct entities, viz. a true congenital disease characterized by arterial stenosis occurring early in embryonal development, with persistence of a patent arterial capillary network at the base of the skull which plays a part as a collateral pathway for the cerebral blood flow, and secondly, angiographic appearances which are similar but unilateral in distribution, related to acquired stenosis of the main arterial trunks. Only the first group should be called Nishimoto's disease.

Nishimoto's disease: significance of its angiographic appearances.

[Moya-Moya or Nishimoto's disease. Apropos of 3 cases].

Return to work (RTW) after a coronary event remains a major concern. This systematic review and meta-analysis of prospective studies published between January 1988 and August 2020, aim to evaluate the prevalence of RTW after a coronary event (myocardial infarction, acute coronary syndrome, angina pectoris) and to assess the determinants of RTW (such as follow-up duration, date of recruitment, country, gender, occupational factors, etc.). PRISMA and MOOSE guidelines were followed. Study quality was assessed using the Newcastle-Ottawa Scale. Random-effects models were carried out to determine pooled prevalence estimates and 95% confident interval. A total of 43 prospective studies (34,964 patients) were investigated. RTW overall random effects pooled prevalence was estimated at 81.1% [95% CI 75.8-85.8]. Country, year of implementation or gender did not significantly modify the prevalence estimates. Lower level of education and degraded left ventricular ejection fraction decreased RTW prevalence estimates (respectively, 76.1% vs 85.6% and 65.3% vs 77.8%). RTW prevalence estimates were higher for white-collars (81.2% vs 65.0% for blue-collars) and people with low physical workload (78.3% vs 64.1% for elevated physical workload).Occupational physical constraints seem to have a negative role in RTW while psycho-logical factors at work are insufficiently investigated. A better understanding of the real-life working conditions influencing RTW would be useful to maintain coronary patients in the labor market. 

/pdf/prevalence-and-determinants-of-return-to-work-after-various-271m7smk.pdf

Prevalence and determinants of return to work after various coronary events: meta-analysis of prospective studies

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Nishimoto's disease: significance of its angiographic appearances.

[Moya-Moya or Nishimoto's disease. Apropos of 3 cases].

Prevalence and determinants of return to work after various coronary events: meta-analysis of prospective studies