Background: The gold standard for evaluation of the severity of moyamoya vasculopathy is the Suzuki grade determined with cerebral catheter angiography (CA). With greater use of magnetic resonance angiography (MRA) it is important to understand if MRA is truly comparable to CA.
Methods: Children with moyamoya were evaluated using the Suzuki score for CA and the modified MRA six-stage Suzuki score to describe the angiographic findings in moyamoya from initial narrowing of the distal internal carotid artery to the "puff of smoke" appearance of the lenticulostriate collaterals and finally to the disappearance of this network of collaterals. Using Cohen kappa we compared Suzuki grade based on CA with MRA in the same patients.
Results: A total of 27 children with moyamoya were reviewed. We calculated a weighted Cohen kappa of 0.49 (P < 0.0001), which is a moderate correlation.
Conclusions: We suggest caution in the reliance on MRA for the diagnosis and evaluation of severity of moyamoya in children.
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http://dx.doi.org/10.1016/j.pediatrneurol.2024.06.008 | DOI Listing |
Korean J Anesthesiol
December 2024
Department of Anesthesiology, Children's Hospital of Fudan University, Shanghai, China.
Background: Delirium in the post-anesthesia care unit (PACU) may be associated with worse outcomes in children with moyamoya disease (MMD). This retrospective study aimed to describe the prevalence of PACU delirium in children with MMD and investigate its risk factors.
Methods: Patients with MMD aged < 15 years who underwent indirect revascularization between January 2014 and October 2023 were included in this study.
Front Neurol
November 2024
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Objective: This study aims to explore the potential contribution of angiographic characteristics in the increased stroke risk among pediatric patients.
Methods: This study retrospectively enrolled pediatric patients with ischemic, hemorrhagic, and asymptomatic moyamoya disease. Their hemispheres were categorized into five groups for the analysis of angiographic characteristics, which included Suzuki's stage, moyamoya vessels, lenticulostriate artery, thalamotuberal artery, thalamoperforating artery, anterior choroidal arteries, posterior choroidal arteries, and posterior cerebral artery involvement.
EBioMedicine
December 2024
Institute of Clinical Medicine, College of Medicine, National Cheng Kung University, Tainan, Taiwan; Research Center of Clinical Medicine, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan. Electronic address:
Background: Impaired mitochondrial protein import machinery leads to phenotypically heterogeneous diseases. Here, we report a recurrent homozygous missense variant in the gene that encodes the translocase of outer mitochondrial membrane 7 (TOMM7) in nine patients with microcephaly, short stature, facial dysmorphia, atrophic macular scarring, and moyamoya disease from seven unrelated families.
Methods: To prove the causality of the TOMM7 variant, mitochondrial morphology, proteomics, and respiration were investigated in CRISPR/Cas9-edited iPSCs-derived endothelial cells.
bioRxiv
November 2024
Center for Genomic Medicine, Massachusetts General Hospital, Boston, MA, USA.
Childs Nerv Syst
November 2024
Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences, Whitefield, EPIP Area, Bangalore, 560066, Karnataka, India.
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