Macrohistory of Moyamoya Disease Analyzed Using Artificial Intelligence.

INTRODUCTION: Moyamoya disease is characterized by progressive stenotic changes in the terminal segment of the internal carotid artery and the development of abnormal vascular networks called moyamoya vessels. The objective of this review was to provide a holistic view of the epidemiology, etiology, clinical findings, treatment, and pathogenesis of moyamoya disease. A literature search was performed in PubMed using the term "moyamoya disease," for articles published until 2021. RESULTS: Artificial intelligence (AI) clustering was used to classify the articles into 5 clusters: (1) pathophysiology (23.5%); (2) clinical background (37.3%); (3) imaging (13.2%); (4) treatment (17.3%); and (5) genetics (8.7%). Many articles in the "clinical background" cluster were published from the 1970s. However, in the "treatment" and "genetics" clusters, the articles were published from the 2010s through 2021. In 2011, it was confirmed that a gene called Ringin protein 213 (RNF213) is a susceptibility gene for moyamoya disease. Since then, tremendous progress in genomic, transcriptomic, and epigenetic profiling (e.g., methylation profiling) has resulted in new concepts for classifying moyamoya disease. Our literature survey revealed that the pathogenesis involves aberrations of multiple signaling pathways through genetic mutations and altered gene expression. CONCLUSION: We analyzed the content vectors in abstracts using AI, and reviewed the pathophysiology, clinical background, radiological features, treatments, and genetic peculiarity of moyamoya disease.

Choreo-ballistic movement after thalamotomy in a patient with Lewy body dementia.

Thalamotomy is an established treatment for medically refractory tremor. Major complications of thalamotomy include cognitive disturbance, paralysis, dysphagia, sensory loss, and speech disturbance. Hemiballism is a rare complication after thalamotomy. We herein present a 74-year-old female patient who developed choreo-ballistic movement after thalamotomy. She was diagnosed with Lewy body dementia at the age of 70 years and exhibited worsening bilateral hand tremor. Her tremor was severe and pharmacoresistant. Left thalamotomy was planned with the trajectory passing through the ventralis intermedius (VIM) nucleus to the posterior subthalamic area (PSA). The right VIM nucleus and PSA were both coagulated with one trajectory, resulting in the immediate amelioration of right hand tremor. However, four days after surgery, choreo-ballistic movement appeared in the right leg and persisted for six months. Furthermore, tremor recurred after one month. Postoperative MRI showed a small coagulated lesion in the subthalamic nucleus. Although choreo-ballistic movement is a rare complication, it needs to be considered, particularly in patients in which the inferior border of the VIM nucleus is targeted.

Arterial spin-labeling magnetic resonance imaging after revascularization of moyamoya disease.

Arterial spin labeling (ASL) magnetic resonance imaging (MRI) is a technique for depicting cerebral perfusion without contrast medium. The purpose of this study was to determine whether ASL can be used to detect hyperperfusion after revascularization for moyamoya disease as effectively as N-isopropyl-[123I]ß-iodoamphetamine ((123)I-IMP) single-photon emission computed tomography (SPECT). Fifteen consecutive patients with moyamoya disease were included in the study. All patients underwent surgical revascularization. Postoperatively, regional cerebral blood flow (rCBF) was measured by flow-sensitive alternating inversion recovery (FAIR) ASL and (123)I-IMP SPECT during the acute stage, and rCBF of the operative side was compared with the other side. The asymmetry ratio (AR) was then calculated from the rCBF as measured using each modality. The postoperative AR of ASL was moderately correlated with that of (123)I-IMP SPECT (y = 0.180x + 0.819; R = 0.80; P = .0003). In this series, 2 patients (13.3%) suffered symptomatic hyperperfusion after revascularization and accordingly exhibited increased AR of ASL. Our data indicate that early increases in rCBF in patients with hyperperfusion could be detected using FAIR ASL supplemental to (123)I-IMP SPECT after revascularization. Our data indicate that FAIR ASL is a convenient method for evaluating hyperperfusion that can be performed repeatedly without the use of contrast medium or radioisotopes.

Suprasellar hemangioblastoma.

A 59-year-old woman presented with disturbance of consciousness and decreased visual acuity caused by a suprasellar mass identified on MRI. A bifrontal interhemispheric approach allowed removal of the top and lateral sides of the tumor from the wall of the third ventricle. The hypothalamus appeared to be the origin of the mass, which proved to be hemangioblastoma, a rare tumor in this location.