Cerebral small vessel disease markers and long-term prognosis in spontaneous intracerebral hemorrhage: the HAGAKURE-ICH study.

We investigated the effects of individual and cumulative cerebral small vessel disease (SVD) markers on long-term clinical outcomes in spontaneous intracerebral hemorrhage (sICH) patients. This prospective, single-center cohort study was conducted from 2012 to 2019. SVD markers, including lacunae, cerebral microbleeds, white matter hyperintensity (WMH), and perivascular spaces in the basal ganglia, were assessed to calculate a summary SVD score.

Involvement of the nigrostriatal system in Gerstman-Sträussler-Scheinker disease with the PRNP-P102L mutation.

Introduction: Gerstmann-Sträussler-Scheinker disease (GSS) is an autosomal-dominant inherited prion disease most often associated with the human prion protein gene (PRNP)-P102L mutation. Although patients manifest considerable phenotypic heterogeneity, the involvement of the nigrostriatal system has not been well-studied.

Methods: We performed dopamine transporter single-photon emission computed tomography (DAT-SPECT) using I-ioflupane to investigate the nigrostriatal system function in nine patients with the PRNP-P102L mutation.

Efgartigimod Successfully Ameliorated Acute Exacerbation of Myasthenia Gravis with Anti-muscle-specific Kinase Antibodies.

We herein report two patients with anti-muscle-specific kinase (MuSK) antibody-positive myasthenia gravis who experienced rapid deterioration of weakness, particularly respiratory muscle weakness, necessitating non-invasive positive pressure ventilation (NIPPV) and were treated with efgartigimod. After treatment initiation, a rapid reduction in IgG levels and recovery from clinical symptoms were observed. NIPPV was no longer required two to three weeks after the first infusion of efgartigimod.

Fulminant demyelinating disease of the central nervous system effectively treated with a combination of decompressive craniectomy and immunotherapy: A case report and literature review.

Key Clinical Message: Accurately identifying fulminant demyelinating diseases is important for sudden onset of asymmetric cerebral white matter lesions with mass effect. Initially, immunotherapy should be administered; however, surgical intervention should be performed with poor response to medical management and evident signs of cerebral herniation.

Abstract: A case of fulminant demyelinating disease of the central nervous system that required decompressive craniectomy 8 days after symptom onset is presented.

Cerebral Small Vessel Disease Burden for Bleeding Risk during Antithrombotic Therapy: Bleeding with Antithrombotic Therapy 2 Study.

Objective: This study was undertaken to determine the excess risk of antithrombotic-related bleeding due to cerebral small vessel disease (SVD) burden.

Methods: In this observational, prospective cohort study, patients with cerebrovascular or cardiovascular diseases taking oral antithrombotic agents were enrolled from 52 hospitals across Japan between 2016 and 2019. Baseline multimodal magnetic resonance imaging acquired under prespecified conditions was assessed by a central diagnostic radiology committee to calculate total SVD score.

Associations for progression of cerebral small vessel disease burden in healthy adults: the Kashima scan study.

To investigate the association between vascular risk factors and progression of cerebral small vessel disease (SVD), we conducted a longitudinal study with neurologically healthy cohort composed mostly of middle-aged adults (n = 665, mean age, 57.7 years). Subjects, who had both baseline data of brain health examinations including MRI and follow-up MRI at least 1 year after the baseline MRI, were included this study.

Customization of a passive surgical support robot to specifications for ophthalmic surgery and preliminary evaluation.

Purpose: To customize a passive surgery support robot for ophthalmic surgery and preliminarily evaluate its performance.

Study Design: Prospective observational study.

Methods: The range of motion of the arm was analyzed during ophthalmic surgery and, based on this analysis, a commercially available passive robot was customized for surgical support for ophthalmic surgery; following which a prototype robot was constructed.

Overlapping rheumatoid meningitis with anti-N-methyl-D-aspartate receptor encephalitis: A case report.

A 66-year-old woman in treatment for rheumatoid meningitis was found to be positive for anti-N-methyl-D-aspartate receptor (NMDAR) antibodies in the cerebrospinal fluid, and intravenous immunoglobulin improved her psychiatric symptoms. The co-existence of NMDAR antibodies should be considered in cases of poor response to treatments or atypical symptoms in rheumatoid meningitis.

Dysautonomia associated with immune checkpoint inhibitors.

Objective: The purpose of this study is to report the clinical characteristics of dysautonomia associated with immune checkpoint inhibitors (ICIs).

Methods: We reported two patients with autoimmune autonomic ganglionopathy (AAG) occurring as immune-related adverse events (irAEs). We also performed a review of previous case reports presenting dysautonomia during ICI therapy.

Hypertension, cerebral Amyloid, aGe Associated Known neuroimaging markers of cerebral small vessel disease Undertaken with stroke REgistry (HAGAKURE) prospective cohort study: Baseline characteristics and association of cerebral small vessel disease with prognosis in an ischemic stroke cohort.

Introduction: Cerebral small vessel disease (SVD) is one of the leading causes of stroke; each neuroimaging marker of SVD is correlated with vascular risk factors and associated with poor prognosis after stroke. However, longitudinal studies investigating the association between comprehensive SVD burden scoring system, "total SVD score" - which encompasses the established neuroimaging markers of lacunae, cerebral microbleeds (CMBs), white matter hyperintensities (WMH) including periventricular hyperintensities, and perivascular spaces in basal ganglia- and clinical outcomes are limited. The aim of this study is to determine the association between SVD burden and long-term prognosis in patients with ischemic stroke.

SARS-CoV-2-related Myelin Oligodendrocyte Glycoprotein Antibody-associated Disease: A Case Report and Literature Review.

We herein report a case of myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. A 24-year-old woman developed unilateral optic neuritis 3 weeks after contracting coronavirus disease 2019 (COVID-19), followed by intracranial demyelinating lesions and myelitis. Since serum anti-MOG antibody was positive, we diagnosed MOG antibody-associated disease.

[Central nervous system involvement of graft versus host disease after allogeneic hematopoietic stem cell transplantation for adult T cell leukemia].

A 54-year-old woman was diagnosed with acute adult T-cell leukemia (ATL) in November 2015 and underwent allogeneic hematopoietic stem cell transplantation in March 2016. Cognitive impairment appeared suddenly around May 2019, and MRI of the brain showed cerebral white matter lesions. Cerebrospinal fluid examination showed no significant findings other than elevated protein.

[Multiple cerebral infarctions in the deep perforator regions in a case of idiopathic hypereosinophilic syndrome].

A 61-year-old man was admitted to our hospital due to cerebral infarction in the pons and the right putamen. On admission (day 3 from symptom onset), laboratory testing showed a white blood cell count of 13,100/μl with hypereosinophilia of 3,734/μl. As deep vein thrombosis was detected on contrast-enhanced CT, we started anticoagulation therapy.

Proportion of intracerebral haemorrhage due to cerebral amyloid angiopathy in the East and West: Comparison between single hospital centres in Japan and the United Kingdom.

Purpose: We investigated whether the proportion of intracerebral haemorrhage (ICH) due to cerebral amyloid angiopathy (CAA) differs between patients admitted to hospitals in the East and the West.

Methods: This international cross-sectional study included consecutive spontaneous ICH patients admitted to one stroke centre in the United Kingdom (Western centre origin) and one in Japan (Eastern centre origin) during the same period. We classified spontaneous ICH into "CAA-related" or "other" using the Edinburgh CT-based diagnostic criteria.

Detrimental Effect of Chronic Hypertension on Leptomeningeal Collateral Flow in Acute Ischemic Stroke.

Background and Purpose- We aimed to evaluate the effect of chronic hypertension on acute leptomeningeal collateral flow in patients with large-vessel ischemic stroke using digital subtraction angiography, which is the gold standard for the assessment of collateral circulation. Methods- Of the consecutive ischemic stroke patients from October 2011 to December 2017 seen in our institution, patients with acute occlusion of the M1 segment of the middle cerebral artery confirmed on initial digital subtraction angiography were enrolled. Chronic hypertension was defined as its documentation before the index stroke or as the administration of antihypertensive medications before onset.

Detection of Stenosis Progression in Intracranial Vertebral Artery Dissection Using Carotid Ultrasonography.

Background: The purpose of this study was to assess whether carotid ultrasonography indices detect arterial stenosis progression in patients with vertebral artery (VA) dissection.

Methods: This was a retrospective, single-center, observational study that enrolled patients with intracranial VA dissection who were admitted from January 2011 to June 2017. Magnetic resonance angiography (MRA) was done on admission and followed up at a median 20 days after onset (interquartile range [IQR] 9-58 days), and ultrasonography was performed at a median of 22 (interquartile range 7-56) days.

Clinical and Radiological Characteristics of Intracranial Artery Dissection Using Recently Proposed Diagnostic Criteria.

Background: Data on the clinical and radiological characteristics of intracranial artery dissection (IAD) have remained limited. Our purpose was to reveal the clinical and radiological characteristics of IAD according to diagnostic criteria for IAD as recently reported by a group of international experts.

Methods: Patients were retrospectively enrolled using a prospective single-center stroke registry between 2011 and 2016.

Early Achievement of Blood Pressure Lowering and Hematoma Growth in Acute Intracerebral Hemorrhage: Stroke Acute Management with Urgent Risk-Factor Assessment and Improvement-Intracerebral Hemorrhage Study.

Background: Previous studies have revealed that hematoma growth mainly occurs during the first 6 h after the onset of spontaneous intracerebral hemorrhage (ICH). Early lowering of blood pressure (BP) may be beneficial for preventing hematoma growth. However, relationships between timing of BP lowering and hematoma growth in ICH remain unclear.

Two-Year Outcomes of Anticoagulation for Acute Ischemic Stroke With Nonvalvular Atrial Fibrillation - SAMURAI-NVAF Study.

Background: We determined the 2-year long-term risk-benefit profile in patients with stroke or transient ischemic attack (TIA) receiving warfarin or direct oral anticoagulants (DOACs) for nonvalvular atrial fibrillation (NVAF) using a prospective, multicenter, observational registry in Japan.

Methods and results: NVAF patients within 7 days after onset of ischemic stroke/TIA were enrolled in 18 stroke centers. Outcome measures included ischemic and bleeding events and death in the 2-year follow-up period.

[Pre-critical MRI findings of an Alzheimer's disease patient with pathologically proven cerebral amyloid angiopathy related lobar hemorrhage].

An 85-year-old woman with untreated hypertension was admitted with a disturbance of consciousness. On admission, brain CT revealed a lobar intracerebral hemorrhage with a midline shift. An intracranial hematoma was evacuated via a life-saving craniotomy.