Yasushi Iwasaki
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View article: Revisiting ‘hot cross bun’ sign: a multicentre MRI study of 97 patients with autopsy-confirmed multiple system atrophy
Revisiting ‘hot cross bun’ sign: a multicentre MRI study of 97 patients with autopsy-confirmed multiple system atrophy Open
Background The purpose of this study was to clarify the usefulness of the ‘hot cross bun’ sign (HCBS) as a diagnostic imaging marker in a large cohort of patients with multiple system atrophy (MSA) and spinocerebellar ataxia (SCA). Methods…
View article: Contribution of Comorbid Pathologies to Amyotrophic Lateral Sclerosis with Cognitive or Behavioral Abnormalities
Contribution of Comorbid Pathologies to Amyotrophic Lateral Sclerosis with Cognitive or Behavioral Abnormalities Open
Background: Amyotrophic lateral sclerosis (ALS) often presents with cognitive or behavioral abnormalities. The cortical involvement of TAR DNA-binding protein-43 (TDP-43) pathology is considered a major cause of these abnormalities. Howeve…
View article: AGG Repeat Expansion and Aggregation of BIN1 in Multiple System Atrophy
AGG Repeat Expansion and Aggregation of BIN1 in Multiple System Atrophy Open
Multiple system atrophy (MSA) is a fatal, sporadic α-synucleinopathy characterized by glial cytoplasmic inclusions (GCIs) in oligodendrocytes. No causative gene for MSA has been identified to date. Herein, we report an intronic AGG repeat …
View article: Prion Positivity Detected by Real-Time Quaking-Induced Conversion (RT-QuIC) in the Cadaver of an Elderly Woman Subjected to Forensic Autopsy
Prion Positivity Detected by Real-Time Quaking-Induced Conversion (RT-QuIC) in the Cadaver of an Elderly Woman Subjected to Forensic Autopsy Open
View article: An autopsy case of a 76-year-old woman with progressive supranuclear palsy initially presenting with dropped head and clinical features of pure akinesia with gait freezing
An autopsy case of a 76-year-old woman with progressive supranuclear palsy initially presenting with dropped head and clinical features of pure akinesia with gait freezing Open
We present a case of a 76-year-old woman diagnosed with pathologically confirmed progressive supranuclear palsy (PSP) with pallido-nigral-luysial atrophy, who initially presented with a dropped head. Upon her first visit, neurophysiologica…
View article: An autopsy case of primary progressive multiple sclerosis with minimal acute inflammation and remyelination over an 11-year course
An autopsy case of primary progressive multiple sclerosis with minimal acute inflammation and remyelination over an 11-year course Open
A 32-year-old man presented with the symptoms of a floating sensation, weakness on the right side of the body, and tremor of the right hand. Head MRI was performed, and T2-weighted images showed high-signal lesions around the la…
View article: Subarachnoid Hemorrhage Following Ischemia Due to Dolichoectatic Aneurysm
Subarachnoid Hemorrhage Following Ischemia Due to Dolichoectatic Aneurysm Open
View article: Ultrasensitive detection of TDP-43 and amyloid-β protein aggregates using micelle-assisted seed amplification assay
Ultrasensitive detection of TDP-43 and amyloid-β protein aggregates using micelle-assisted seed amplification assay Open
View article: Age-Related Pathology in Corticobasal Degeneration
Age-Related Pathology in Corticobasal Degeneration Open
Elderly human brains are vulnerable to multiple proteinopathies, although each protein has a different transmission pathway. Tau-immunoreactive astrocytes are well-known in elderly brains. In contrast, astrocytic plaques, a hallmark in cor…
View article: Detection Limitations of Prion Seeding Activities in Blood Samples from Patients with Sporadic Prion Disease
Detection Limitations of Prion Seeding Activities in Blood Samples from Patients with Sporadic Prion Disease Open
Human prion diseases (HPDs) are fatal neurodegenerative disorders characterized by abnormal prion proteins (PrPSc). Numerous techniques have been devised to detect prion seeding activity, each with limitations. Here, we developed a technol…
View article: Preclinical Characterization of the Tau PET Tracer [<sup>18</sup>F]SNFT-1: Comparison of Tau PET Tracers
Preclinical Characterization of the Tau PET Tracer [<sup>18</sup>F]SNFT-1: Comparison of Tau PET Tracers Open
Tau PET tracers are expected to be sufficiently sensitive to track the progression of age-related tau pathology in the medial temporal cortex. The tau PET tracer N-(4-[18F]fluoro-5-methylpyridin-2-yl)-7-aminoimidazo[1,2-a…
View article: Identical Tau Filaments in Subacute Sclerosing Panencephalitis and Chronic Traumatic Encephalopathy
Identical Tau Filaments in Subacute Sclerosing Panencephalitis and Chronic Traumatic Encephalopathy Open
Subacute sclerosing panencephalitis (SSPE) occurs in some individuals after measles infection, following a symptom-free period of several years. It resembles chronic traumatic encephalopathy (CTE), which happens after repetitive head impac…
View article: TDP-43 Proteinopathy and Tauopathy: Do They Have Pathomechanistic Links?
TDP-43 Proteinopathy and Tauopathy: Do They Have Pathomechanistic Links? Open
Transactivation response DNA binding protein 43 kDa (TDP-43) and tau are major pathological proteins of neurodegenerative disorders, of which neuronal and glial aggregates are pathological hallmarks. Interestingly, accumulating evidence fr…
View article: Actin-binding protein filamin-A drives tau aggregation and contributes to progressive supranuclear palsy pathology
Actin-binding protein filamin-A drives tau aggregation and contributes to progressive supranuclear palsy pathology Open
While amyloid-β lies upstream of tau pathology in Alzheimer’s disease, key drivers for other tauopathies, including progressive supranuclear palsy (PSP), are largely unknown. Various tau mutations are known to facilitate tau aggregation, b…
View article: Publisher Correction: Essential roles of plexin-B3+ oligodendrocyte precursor cells in the pathogenesis of Alzheimer’s disease
Publisher Correction: Essential roles of plexin-B3+ oligodendrocyte precursor cells in the pathogenesis of Alzheimer’s disease Open
View article: Correction to: FXTAS is difficult to differentiate from neuronal intranuclear inclusion disease through skin biopsy: a case report
Correction to: FXTAS is difficult to differentiate from neuronal intranuclear inclusion disease through skin biopsy: a case report Open
View article: FXTAS is difficult to differentiate from neuronal intranuclear inclusion disease through skin biopsy: a case report
FXTAS is difficult to differentiate from neuronal intranuclear inclusion disease through skin biopsy: a case report Open
View article: Multiple system atrophy variant with severe hippocampal pathology
Multiple system atrophy variant with severe hippocampal pathology Open
The striatonigral and olivopontocerebellar systems are known to be vulnerable in multiple system atrophy (MSA), showing neuronal loss, astrogliosis, and alpha‐synuclein‐immunoreactive inclusions. MSA patients who displayed abundant neurona…
View article: Case of Neuronal Intranuclear Inclusion Disease With Dynamic Perfusion Changes Lacking Typical Signs on Diffusion-Weighted Imaging
Case of Neuronal Intranuclear Inclusion Disease With Dynamic Perfusion Changes Lacking Typical Signs on Diffusion-Weighted Imaging Open
Neuronal intranuclear inclusion disease (NIID) is a slowly progressive neurodegenerative disorder with a wide range of clinical manifestations, including dementia and peripheral neuropathy.1 NIID is pathologically characterized by eosinoph…
View article: Translation of GGC repeat expansions into a toxic polyglycine protein in NIID defines a novel class of human genetic disorders: The polyG diseases
Translation of GGC repeat expansions into a toxic polyglycine protein in NIID defines a novel class of human genetic disorders: The polyG diseases Open
View article: Pathway from TDP-43-Related Pathology to Neuronal Dysfunction in Amyotrophic Lateral Sclerosis and Frontotemporal Lobar Degeneration
Pathway from TDP-43-Related Pathology to Neuronal Dysfunction in Amyotrophic Lateral Sclerosis and Frontotemporal Lobar Degeneration Open
Transactivation response DNA binding protein 43 kDa (TDP-43) is known to be a pathologic protein in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). TDP-43 is normally a nuclear protein, but affected neuron…
View article: System degeneration in an MM1-type sporadic Creutzfeldt-Jakob disease case with an unusually prolonged akinetic mutism state
System degeneration in an MM1-type sporadic Creutzfeldt-Jakob disease case with an unusually prolonged akinetic mutism state Open
Methionine/methionine type 1 (MM1-type) sporadic Creutzfeldt-Jakob disease (sCJD), known as the 'classic type,' shows typical clinicopathological sCJD findings. In general, patients reach an akinetic mutism state within a few months of dis…
View article: Rapid Progression of White Matter Signal Changes and Frontotemporal Atrophy in Globular Glial Tauopathy
Rapid Progression of White Matter Signal Changes and Frontotemporal Atrophy in Globular Glial Tauopathy Open
View article: MBRS-32. TOPOISOMERASE II β INDUCES NEURONAL, BUT NOT GLIAL, DIFFERENTIATION IN MEDULLOBLASTOMA
MBRS-32. TOPOISOMERASE II β INDUCES NEURONAL, BUT NOT GLIAL, DIFFERENTIATION IN MEDULLOBLASTOMA Open
BACKGROUND We previously reported that Gli3, which was a downstream molecule of Sonic Hedgehog signal, induced neuronal and/or glial differentiation in some types of medulloblastoma (desmoplastic/nodular medulloblastoma and medulloblastoma…
View article: Unclassified four-repeat tauopathy associated with familial parkinsonism and progressive respiratory failure
Unclassified four-repeat tauopathy associated with familial parkinsonism and progressive respiratory failure Open
View article: Clinicopathological findings of a long-term survivor of V180I genetic Creutzfeldt-Jakob disease
Clinicopathological findings of a long-term survivor of V180I genetic Creutzfeldt-Jakob disease Open
The clinical characteristics of genetic Creutzfeldt-Jakob disease (gCJD) with a V180I mutation in the PRNP gene (V180I gCJD) are unique: elderly-onset, gradual progression, sporadic fashion, and cortical oedematous hyper-intensity o…
View article: Postmortem Quantitative Analysis of Prion Seeding Activity in the Digestive System
Postmortem Quantitative Analysis of Prion Seeding Activity in the Digestive System Open
Human prion diseases are neurodegenerative disorders caused by prion protein. Although infectivity was historically detected only in the central nervous system and lymphoreticular tissues of patients with sporadic Creutzfeldt-Jakob disease…
View article: Safety and reliability of computed tomography-guided lipiodol marking for undetectable pulmonary lesions
Safety and reliability of computed tomography-guided lipiodol marking for undetectable pulmonary lesions Open
OBJECTIVES This study aimed to evaluate the safety and reliability of percutaneous computed tomography (CT)-guided lipiodol marking for undetectable pulmonary lesions before video-assisted thoracic surgery (VATS). METHODS We retrospectivel…
View article: A Suspected Case of Alveolar Hemorrhage Caused by Osimertinib
A Suspected Case of Alveolar Hemorrhage Caused by Osimertinib Open
背景．分子標的薬による肺障害は間質性肺炎が一般的だが，オシメルチニブによる肺胞出血を疑う1例を経験したので報告する．症例．75歳女性．2015年12月に右上葉原発の肺腺癌（cT2bN0M0，stage IIA）と診断された．初回EGFR遺伝子変異の検索で，Exon21のL858R及びExon20のT790Mを認めた．外科治療，放射線治療は患者が希望せず薬物治療の方針とした．2016年2月からゲフィチニブを開始したが同年10月に原発巣の増大と同一肺葉内への転移がみられ，同年1…
View article: A Novel Combination of Prion Strain Co-Occurrence in Patients with Sporadic Creutzfeldt-Jakob Disease
A Novel Combination of Prion Strain Co-Occurrence in Patients with Sporadic Creutzfeldt-Jakob Disease Open