Review
Cell Biology
Xiaofang Xu, Huikang Xu, Zhaocai Zhang
Summary: Cerebral amyloid angiopathy (CAA) is a disease characterized by the deposition of amyloid beta (A beta) and other amyloid proteins in the brain's blood vessels, leading to cerebrovascular and brain damage. CAA is often associated with cardiac injury, involving A beta, tau, and transthyroxine amyloid (ATTR). A beta acts as the main damaging factor, causing coronary artery atherosclerosis, aortic valve calcification, and cardiomyocyte degeneration. The progression of CAA can further impact the heart through amyloidosis, inflammation, and cerebral hemorrhage. The interaction between CAA and cardiac injury creates a vicious cycle that accelerates the progression of both conditions.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2023)
Article
Clinical Neurology
L. Grangeon, G. Quesney, M. Verdalle-Cazes, S. Coulette, D. Renard, A. Wacongne, T. Allou, N. Olivier, Y. Boukriche, G. Blanchet-Fourcade, P. Labauge, C. Arquizan, S. Canaple, O. Godefroy, O. Martinaud, P. Verdure, M. Quillard-Muraine, J. Pariente, E. Magnin, G. Nicolas, C. Charbonnier, D. Maltete, M. Formaglio, N. Raposo, X. Ayrignac, D. Wallon
Summary: This study aimed to compare the clinical and radiological outcomes of CAA-related inflammation (CAA-ri) and non-inflammatory CAA. The results showed that CAA-ri patients had a higher institutionalization rate, mortality rate, and more severe cognitive impairment and loss of autonomy compared to non-inflammatory CAA patients. MRI analysis revealed a higher number of cerebral microbleeds (CMB) in CAA-ri patients, and CSF analysis showed lower levels of Ass42. The presence of the APOE epsilon 4 allele may partially contribute to the increased severity in CAA-ri.
JOURNAL OF NEUROLOGY
(2022)
Article
Neurosciences
Mengke Zhang, Ruiwen Che, Wenbo Zhao, Hailiang Sun, Changhong Ren, Jin Ma, Wenbo Hu, Milan Jia, Chuanjie Wu, Xin Liu, Xunming Ji
Summary: This study aimed to explore the relationship between computed tomography (CT)-based cerebral small vessel disease (SVD) markers and the clinical outcomes in patients with cerebral amyloid angiopathy (CAA)-related intracerebral hemorrhage (ICH). The results showed that the SVD markers based on CT could predict the short-term outcome more effectively in patients with CAA-ICH. Further studies are needed to validate these findings and identify modifiable factors for preventing CAA-ICH development.
CNS NEUROSCIENCE & THERAPEUTICS
(2023)
Article
Clinical Neurology
Jeremy F. Kellie, Bruce C. Campbell, Rosie Watson, Adrian J. Praeger, Girish Nair, Anand Murugasu, Christopher C. Rowe, Colin L. Masters, Steven Collins, Catriona McLean, Nawaf Yassi
Summary: Recent reports suggest the possibility of neurosurgical transmission of cerebral amyloid angiopathy (CAA), leading to intracerebral hemorrhage in young adults. However, such cases are rare in standard clinical practice.
Review
Clinical Neurology
Aikaterini Theodorou, Lina Palaiodimou, Konark Malhotra, Christina Zompola, Aristeidis H. Katsanos, Ashkan Shoamanesh, Efstathios Boviatsis, Efthimios Dardiotis, Martha Spilioti, Simona Sacco, David J. Werring, Charlotte Cordonnier, Andrei Alexandrov, George R. Paraskevas, Georgios Tsivgoulis
Summary: This study conducted a systematic review and meta-analysis on the clinical, neuroimaging, genetic, and cerebrospinal fluid biomarker characteristics of patients with cerebral amyloid angiopathy-related inflammation (CAA-ri). The results showed that cognitive decline was the most common clinical feature, while hyperintense T2/fluid-attenuated inversion recovery white matter lesions and lobar cerebral microbleeds were the most prevalent neuroimaging findings. 34% of patients with CAA-ri had the ApoE epsilon 4/epsilon 4 genotype.
Review
Clinical Neurology
Jhih-Yong Yang, Yung-Tsai Chu, Hsin-Hsi Tsai, Jiann-Shing Jeng
Summary: This study retrospectively described two cases of CAA-ri and investigated the longitudinal changes of amyloid PET. The results showed focal decreases in amyloid load after the inflammatory process. The study also revealed variable findings related to amyloid burden in post-inflammatory regions in previously reported cases of CAA-ri with available amyloid PET.
FRONTIERS IN NEUROLOGY
(2023)
Article
Clinical Neurology
Derrick N. Okine, David S. Knopman, Thomas H. Mosley, Dean F. Wong, Michelle C. Johansen, Keenan A. Walker, Clifford R. Jack Jr, Kejal Kantarci, James R. Pike, Jonathan Graff-Radford, Rebecca F. Gottesman
Summary: This study evaluated the association between CMB patterns and cerebral A beta deposition and found that a lobar-only pattern of CMBs or superficial siderosis is most strongly associated with brain A beta, while a mixed CMB pattern does not have an elevated risk.
Article
Clinical Neurology
Joan Marti-Fabregas, Pol G. Camps-Renom, Jonathan G. Best, Anna Ramos-Pachon, Marina Guasch-Jimenez, Alejandro Martinez-Domeno, Daniel M. Guisado-Alonso, Beatriz M. Gomez-Anson, Gareth Ambler, Duncan Wilson, Keon-Joo Lee, Jae-Sung Lim, Hee-Joon Bae, Masayuki Shiozawa, Masatoshi Koga, Kazunori G. Toyoda, Michael G. Hennerici, Hugues Chabriat, Eric Jouvent, Debbie Yuen Kwun Wong, Henry Mak, Kui Kai Lau, Young Dae Kim, Tae-Jin Song, Ji-Hoe Heo, Sebastian Eppinger, Thomas Gattringer, Ender Uysal, Derya Selcuk Demirelli, Natan Bornstein, Einor Ben Assayag, Hen A. Hallevi, Jeremy A. Molad, Masashi Nishihara, Jun Tanaka, Hideo Hara, Yusuke B. Yakushiji, Shelagh B. Coutts, Eric A. Smith, Alexandros A. Polymeris, Benjamin Wagner, David A. Seiffge, Philippe A. Lyrer, Nils T. Peters, Stefan T. Engelter, Rustam Al-Shahi Salman, Hans Rudolf Jager, Gregory Y. H. Lip, Martina Goeldlin, Leonidas Panos, Christopher Charles G. Karayiannis, Thanh G. K. Phan, Velandai K. Srikanth, Nicolas Christ, Sarah Gunkel, Felix W. Fluri, Thomas W. Leung, Yannie O. Y. Soo, Winnie Chu, Jill Abrigo, Carmen Barbato, Simone Browning, Robert Simister, Anne-Marie Mendyk, Regis Bordet, Saima Hilal, Bibek Gyanwali, Christopher Chen, Simon Jung, Dilek Necioglu Orken, David Werring, Luis Prats-Sanchez
Summary: This study aimed to investigate the risk of future cerebrovascular events in patients with ischemic stroke (IS) or transient ischemic attack (TIA) and cortical superficial siderosis (cSS), as well as the benefits and safety of antithrombotic drugs for secondary prevention. The study found that cSS was associated with an increased risk of stroke, and the risk of IS was higher than that of intracranial hemorrhage (ICrH) in patients receiving antiplatelet or anticoagulant treatment alone, while the risk of ICrH was higher than that of IS in patients receiving both treatments. Therefore, the study suggests that using antiplatelet or anticoagulant drugs alone is safe in patients with cSS, but combined antithrombotic therapy might be hazardous.
Article
Cell Biology
Wenjun Deng, Shuzhen Guo, Susanne J. van Veluw, Zhanyang Yu, Su Jing Chan, Hajime Takase, Ken Arai, MingMing Ning, Steven M. Greenberg, Eng H. Lo, Brian J. Bacskai
Summary: This study explores the involvement of vascular dysfunction in the cognitive impairment of Alzheimer's disease and cerebral amyloid angiopathy. The findings suggest that the accumulation of beta-amyloid in the brain blood vessel walls disrupts neurovascular coupling, activates the cell cycle in quiescent endothelial cells, and enhances inflammation, contributing to the vascular pathology of these diseases.
Article
Neurosciences
Claire Muller
Summary: Recently, there has been increased recognition of iatrogenic cerebral amyloid angiopathy (iCAA) cases globally. This case study examines the clinicoradiological features of a probable iCAA case in Australia, with extensive history documentation and MRI data spanning two decades. The study highlights the aggressive course and early disease manifestations of iCAA, providing insights for the more commonly seen sporadic cases.
FRONTIERS IN NEUROSCIENCE
(2023)
Article
Clinical Neurology
Chi Hyuk Oh, Jin San Lee
Summary: In this case report, an 83-year-old female with hepatitis B virus-related liver cirrhosis presented with symptoms of hepatic encephalopathy, including innumerable cerebral microbleeds on brain MRI. Proper medical management led to symptom resolution within 12 hours, suggesting a potential link between severe liver dysfunction and the presence of cerebral microbleeds in the brain.
Article
Chemistry, Multidisciplinary
E. Ruifang, Yaling Shi, Weiwei Wang, Ming Qi
Summary: This study investigated the mechanism by which callistephin mitigates the formation of A beta aggregation and associated CAA, showing that callistephin inhibits A beta fibrillization and reduces cytotoxicity against CVSMCs. These findings suggest potential protective effects of callistephin against CAA through regulation of membrane leakage and downregulation of inflammatory proteins.
ARABIAN JOURNAL OF CHEMISTRY
(2022)
Article
Clinical Neurology
Benedetta Storti, Isabella Canavero, Maria Magdalena Gabriel, Antonella Capozza, Nicola Rifino, Mario Stanziano, Luca Tagliabue, Anna Bersano
Summary: Iatrogenic cerebral amyloid angiopathy (iCAA) is a specific type of cerebral amyloid angiopathy, and its diagnosis is increasing. It is hypothesized that iCAA may occur as a late consequence of past neurosurgical interventions involving dural patch grafts. Although positron emission tomography (PET) scans and beta-amyloid levels in cerebrospinal fluid (CSF) are auxiliary criteria, the definite diagnosis still relies on histopathological examination.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Review
Clinical Neurology
Mariagiovanna Cozza, Lucia Amadori, Virginia Boccardi
Summary: Cerebral Amyloid Angiopathy (CAA) is a neurological disorder characterized by the deposition of amyloid plaques in the walls of cerebral blood vessels. This article provides insights into its pathogenesis, diagnosis, and treatment options. Accurate diagnosis of CAA is crucial for its management, and experimental treatments targeting amyloid beta clearance and anti-inflammatory approaches offer hope for future therapeutic advancements.
JOURNAL OF THE NEUROLOGICAL SCIENCES
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Dora Chen, Michelle Roytman, Kyriakos A. Kirou, Babak B. Navi, Andrew D. Schweitzer
Summary: Cerebral Amyloid Angiopathy (CAA) is a cerebrovascular disease common in elderly patients, associated with cognitive decline and intracranial hemorrhage. Inflammatory forms of CAA-ri/ABRA cause rapid neurocognitive decline but are highly responsive to corticosteroid treatment.
Article
Medicine, General & Internal
Yosuke Takeuchi, Toshiya Nomura, Keiichi Nakahara, Mitsuharu Ueda
Summary: We report a case of a 58-year-old man who presented with progressive gait disturbance and was diagnosed with autoimmune polyglandular syndrome. Laboratory examinations revealed cobalamin deficiency and the presence of multiple autoantibodies. Cobalamin replenishment worsened his gait, but additional steroid therapy was effective in treating his symptoms.
Editorial Material
Cardiac & Cardiovascular Systems
Koji Takahashi, Hiroe Morioka, Daisuke Sasaki, Nobuhisa Yamamura, Sohei Kitazawa, Mitsuharu Ueda, Tomoki Sakaue, Daijiro Enomoto, Shigeki Uemura, Takafumi Okura, Shuntaro Ikeda
JOURNAL OF NUCLEAR CARDIOLOGY
(2023)
Editorial Material
Cardiac & Cardiovascular Systems
Shoko Maeda-Ogata, Nobuhiro Tahara, Atsuko Tahara, Munehisa Bekki, Akihiro Honda, Yoichi Sugiyama, Sachiyo Igata, Toshi Abe, Yoshiki Sekijima, Mitsuharu Ueda, Yukio Ando, Yoshihiro Fukumoto
JOURNAL OF NUCLEAR CARDIOLOGY
(2023)
Letter
Pathology
Mizue Goto, Masayoshi Tasaki, Mitsuharu Ueda
PATHOLOGY INTERNATIONAL
(2023)
Article
Clinical Neurology
Satoshi Yamashita, Akihito Nagatoshi, Yosuke Takeuchi, Ichizo Nishino, Mitsuharu Ueda
Summary: Spinal muscular atrophy (SMA), an autosomal-recessive lower motor neuron disease, leads to progressive muscle wasting and weakness. A patient with adult-onset SMA was found to have myopathic changes, characterized by accumulation of phosphorylated p62 and TDP-43 in muscle cells. This suggests that abnormal protein aggregation may play a role in the myopathic pathology of SMA.
Article
Neuroimaging
Seigo Shindo, Kazutaka Uchida, Shinichi Yoshimura, Nobuyuki Sakai, Hiroshi Yamagami, Kazunori Toyoda, Yuji Matsumaru, Yasushi Matsumoto, Kazumi Kimura, Reiichi Ishikura, Manabu Inoue, Fumihiro Sakakibara, Makoto Nakajima, Mitsuharu Ueda, Takeshi Morimoto
Summary: This study aimed to evaluate the efficacy and safety of EVT in stroke patients with large ischemic core, and whether IVT has additional beneficial effects in these patients.
JOURNAL OF NEUROINTERVENTIONAL SURGERY
(2023)
Article
Clinical Neurology
Satoshi Yamashita, Nozomu Tawara, Kentaro Hara, Mitsuharu Ueda
Summary: This study retrospectively compared the clinical characteristics of ALS patients with early onset (age 74 or younger) and late onset (age 75 or older). It was found that late-onset females were more likely to have bulbar-onset ALS with lower body mass index, while late-onset males had more frequent bulbar and respiratory symptoms and lower forced vital capacity at the initial examination in both groups. Early intervention for bulbar and respiratory symptoms may be useful for prolonging survival in late-onset patients.
JOURNAL OF THE NEUROLOGICAL SCIENCES
(2023)
Article
Clinical Neurology
Satoshi Namitome, Kazutaka Uchida, Seigo Shindo, Shinichi Yoshimura, Nobuyuki Sakai, Hiroshi Yamagami, Kazunori Toyoda, Yuji Matsumaru, Yasushi Matsumoto, Kazumi Kimura, Reiichi Ishikura, Manabu Inoue, Mikiya Beppu, Fumihiro Sakakibara, Manabu Shirakawa, Mitsuharu Ueda, Takeshi Morimoto
Summary: The increased risk of intracranial hemorrhage with multiple passes in endovascular therapy (EVT) for large vessel occlusion with a large ischemic core is a concern. This study explored the effect of the number of EVT passes on patients and found that successful reperfusion within 2 passes was associated with better clinical outcomes.
Editorial Material
Clinical Neurology
Toshiya Nomura, Fujio Fujiki, Mitsuharu Ueda
Summary: This case report presents a 63-year-old man who experienced sensory abnormalities in his feet and was initially misdiagnosed with chronic inflammatory demyelinating polyradiculoneuropathy.
Meeting Abstract
Clinical Neurology
Marco Luigetti, Jonas Wixner, Mitsuharu Ueda, Wilson Marques Junior, Samir Dalia, Seth Arum, Cecilia Hale, Patrick Jay, Kelley Capocelli, David Adams
JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Hidetaka Hayashi, Seitaro Oda, Masafumi Kidoh, Shinpei Yamaguchi, Fumihiro Yoshimura, Seiji Takashio, Hiroki Usuku, Yasunori Nagayama, Takeshi Nakaura, Mitsuharu Ueda, Kenichi Tsujita, Toshinori Hirai
Summary: This study aimed to investigate the application of CT-ECV as an alternative to CMR-ECV in patients with cardiac amyloidosis (CA). The results showed a significant correlation between CT-ECV and CMR-ECV in CA patients.
EUROPEAN RADIOLOGY
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Yasunori Nagayama, Koya Iwashita, Natsuki Maruyama, Hiroyuki Uetani, Makoto Goto, Daisuke Sakabe, Takafumi Emoto, Kengo Nakato, Shinsuke Shigematsu, Yuki Kato, Sentaro Takada, Masafumi Kidoh, Seitaro Oda, Takeshi Nakaura, Masahiro Hatemura, Mitsuharu Ueda, Akitake Mukasa, Toshinori Hirai
Summary: This study compared the image quality of deep learning-based reconstruction (DLR), model-based (MBIR), and hybrid iterative reconstruction (HIR) algorithms for lower-dose (LD) unenhanced head CT with those of standard-dose (STD) HIR images. The results showed that DLR and MBIR reconstructions had lower image noise and higher gray and white matter (GM-WM) contrast, while LD-HIR reconstructions had poorer noise texture. LD-DLR reconstructions had better image quality.
EUROPEAN RADIOLOGY
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Koji Takahashi, Yoshiyasu Hiratsuka, Daisuke Sasaki, Tomoki Sakaue, Daijiro Enomoto, Hiroe Morioka, Shigeki Uemura, Takafumi Okura, Shuntaro Ikeda, Taizo Kono, Takaaki Iwamura, Nobuhisa Yamamura, Sohei Kitazawa, Mitsuharu Ueda
Summary: Our study found that there is variation in the uptake of Tc-99m-PYP in skeletal trunk muscles of patients with ATTR-CA. The abdominal oblique muscle is most frequently affected, while the psoas major muscle shows minimal uptake of the tracer.
CLINICAL NUCLEAR MEDICINE
(2023)
Article
Pathology
Aina Yamaguchi, Masayoshi Tasaki, Mitsuharu Ueda, Yukio Ando, Hironobu Naiki
Summary: This study analyzed the latest subtype frequency of systemic amyloidosis in autopsy cases in Japan, revealing that ATTR is the most predominant subtype among the current cases.
PATHOLOGY INTERNATIONAL
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Masafumi Kidoh, Seitaro Oda, Seiji Takashio, Yawara Kawano, Hidetaka Hayashi, Kosuke Morita, Takafumi Emoto, Shinsuke Shigematsu, Fumihiro Yoshimura, Takeshi Nakaura, Yasunori Nagayama, Masao Matsuoka, Mitsuharu Ueda, Kenichi Tsujita, Toshinori Hirai
Summary: This study evaluated the diagnostic performance of myocardium-to-lumen R1 ratio on postcontrast T1 maps for detecting cardiac amyloidosis in a large patient sample, and found that it showed excellent diagnostic performance comparable to that of MRI ECV. Both MRI ECV and postcontrast myocardium-to-lumen R1 ratio exhibited high sensitivity and specificity for detecting cardiac amyloidosis.
RADIOLOGY-CARDIOTHORACIC IMAGING
(2023)
