Can new drugs stop a deadly set of brain-eating diseases?
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Hope in the fight against deadly prion diseases, and side effects of organic agriculture
BY
Sarah Crespi
,
Katherine Irving
,
Meredith Wadman
Podcast
21 Mar 2024
My friend Charlie Clark began complaining that his eyeglasses were faulty in September 2023. A trip to the optician didn’t fix them; they were still “out of alignment,” he said. The same month, his insomnia,...
Source: Science of Aging Knowledge Environment - March 21, 2024 Category: Geriatrics Source Type: research
Clinical characteristics and diagnostics of human spongiform encephalopathies: an update
Nervenarzt. 2024 Mar 19. doi: 10.1007/s00115-024-01644-2. Online ahead of print.ABSTRACTHuman spongiform encephalopathies are rare transmissible neurodegenerative diseases of the brain and the nervous system that are caused by misfolding of the physiological prion protein into a pathological form and its deposition in the central nervous system (CNS). Prion diseases include Creutzfeldt-Jakob disease (CJD, sporadic or familial), Gerstmann-Straussler-Scheinker syndrome (GSS) and fatal familial insomnia (FFI). Prion diseases can be differentiated into three etiological categories: spontaneous (sporadic CJD), inherited (famili...
Source: Der Nervenarzt - March 20, 2024 Category: Neurology Authors: Peter Hermann Stefan Goebel Inga Zerr Source Type: research
Clinical characteristics and diagnostics of human spongiform encephalopathies: an update
Nervenarzt. 2024 Mar 19. doi: 10.1007/s00115-024-01644-2. Online ahead of print.ABSTRACTHuman spongiform encephalopathies are rare transmissible neurodegenerative diseases of the brain and the nervous system that are caused by misfolding of the physiological prion protein into a pathological form and its deposition in the central nervous system (CNS). Prion diseases include Creutzfeldt-Jakob disease (CJD, sporadic or familial), Gerstmann-Straussler-Scheinker syndrome (GSS) and fatal familial insomnia (FFI). Prion diseases can be differentiated into three etiological categories: spontaneous (sporadic CJD), inherited (famili...
Source: Der Nervenarzt - March 20, 2024 Category: Neurology Authors: Peter Hermann Stefan Goebel Inga Zerr Source Type: research
Clinical characteristics and diagnostics of human spongiform encephalopathies: an update
Nervenarzt. 2024 Mar 19. doi: 10.1007/s00115-024-01644-2. Online ahead of print.ABSTRACTHuman spongiform encephalopathies are rare transmissible neurodegenerative diseases of the brain and the nervous system that are caused by misfolding of the physiological prion protein into a pathological form and its deposition in the central nervous system (CNS). Prion diseases include Creutzfeldt-Jakob disease (CJD, sporadic or familial), Gerstmann-Straussler-Scheinker syndrome (GSS) and fatal familial insomnia (FFI). Prion diseases can be differentiated into three etiological categories: spontaneous (sporadic CJD), inherited (famili...
Source: Der Nervenarzt - March 20, 2024 Category: Neurology Authors: Peter Hermann Stefan Goebel Inga Zerr Source Type: research
Clinical characteristics and diagnostics of human spongiform encephalopathies: an update
Nervenarzt. 2024 Mar 19. doi: 10.1007/s00115-024-01644-2. Online ahead of print.ABSTRACTHuman spongiform encephalopathies are rare transmissible neurodegenerative diseases of the brain and the nervous system that are caused by misfolding of the physiological prion protein into a pathological form and its deposition in the central nervous system (CNS). Prion diseases include Creutzfeldt-Jakob disease (CJD, sporadic or familial), Gerstmann-Straussler-Scheinker syndrome (GSS) and fatal familial insomnia (FFI). Prion diseases can be differentiated into three etiological categories: spontaneous (sporadic CJD), inherited (famili...
Source: Der Nervenarzt - March 20, 2024 Category: Neurology Authors: Peter Hermann Stefan Goebel Inga Zerr Source Type: research
Clinical characteristics and diagnostics of human spongiform encephalopathies: an update
Nervenarzt. 2024 Mar 19. doi: 10.1007/s00115-024-01644-2. Online ahead of print.ABSTRACTHuman spongiform encephalopathies are rare transmissible neurodegenerative diseases of the brain and the nervous system that are caused by misfolding of the physiological prion protein into a pathological form and its deposition in the central nervous system (CNS). Prion diseases include Creutzfeldt-Jakob disease (CJD, sporadic or familial), Gerstmann-Straussler-Scheinker syndrome (GSS) and fatal familial insomnia (FFI). Prion diseases can be differentiated into three etiological categories: spontaneous (sporadic CJD), inherited (famili...
Source: Der Nervenarzt - March 20, 2024 Category: Neurology Authors: Peter Hermann Stefan Goebel Inga Zerr Source Type: research
Clinical characteristics and diagnostics of human spongiform encephalopathies: an update
Nervenarzt. 2024 Mar 19. doi: 10.1007/s00115-024-01644-2. Online ahead of print.ABSTRACTHuman spongiform encephalopathies are rare transmissible neurodegenerative diseases of the brain and the nervous system that are caused by misfolding of the physiological prion protein into a pathological form and its deposition in the central nervous system (CNS). Prion diseases include Creutzfeldt-Jakob disease (CJD, sporadic or familial), Gerstmann-Straussler-Scheinker syndrome (GSS) and fatal familial insomnia (FFI). Prion diseases can be differentiated into three etiological categories: spontaneous (sporadic CJD), inherited (famili...
Source: Der Nervenarzt - March 20, 2024 Category: Neurology Authors: Peter Hermann Stefan Goebel Inga Zerr Source Type: research
Clinical characteristics and diagnostics of human spongiform encephalopathies: an update
Nervenarzt. 2024 Mar 19. doi: 10.1007/s00115-024-01644-2. Online ahead of print.ABSTRACTHuman spongiform encephalopathies are rare transmissible neurodegenerative diseases of the brain and the nervous system that are caused by misfolding of the physiological prion protein into a pathological form and its deposition in the central nervous system (CNS). Prion diseases include Creutzfeldt-Jakob disease (CJD, sporadic or familial), Gerstmann-Straussler-Scheinker syndrome (GSS) and fatal familial insomnia (FFI). Prion diseases can be differentiated into three etiological categories: spontaneous (sporadic CJD), inherited (famili...
Source: Der Nervenarzt - March 20, 2024 Category: Neurology Authors: Peter Hermann Stefan Goebel Inga Zerr Source Type: research
Clinical characteristics and diagnostics of human spongiform encephalopathies: an update
Nervenarzt. 2024 Mar 19. doi: 10.1007/s00115-024-01644-2. Online ahead of print.ABSTRACTHuman spongiform encephalopathies are rare transmissible neurodegenerative diseases of the brain and the nervous system that are caused by misfolding of the physiological prion protein into a pathological form and its deposition in the central nervous system (CNS). Prion diseases include Creutzfeldt-Jakob disease (CJD, sporadic or familial), Gerstmann-Straussler-Scheinker syndrome (GSS) and fatal familial insomnia (FFI). Prion diseases can be differentiated into three etiological categories: spontaneous (sporadic CJD), inherited (famili...
Source: Der Nervenarzt - March 20, 2024 Category: Neurology Authors: Peter Hermann Stefan Goebel Inga Zerr Source Type: research
Clinical characteristics and diagnostics of human spongiform encephalopathies: an update
Nervenarzt. 2024 Mar 19. doi: 10.1007/s00115-024-01644-2. Online ahead of print.ABSTRACTHuman spongiform encephalopathies are rare transmissible neurodegenerative diseases of the brain and the nervous system that are caused by misfolding of the physiological prion protein into a pathological form and its deposition in the central nervous system (CNS). Prion diseases include Creutzfeldt-Jakob disease (CJD, sporadic or familial), Gerstmann-Straussler-Scheinker syndrome (GSS) and fatal familial insomnia (FFI). Prion diseases can be differentiated into three etiological categories: spontaneous (sporadic CJD), inherited (famili...
Source: Der Nervenarzt - March 20, 2024 Category: Neurology Authors: Peter Hermann Stefan Goebel Inga Zerr Source Type: research
A case report of fatal familial insomnia with cerebrospinal fluid leukocytosis during the COVID-19 epidemic and review of the literature
. (Source: Prion)
Source: Prion - January 16, 2024 Category: Biology Authors: Zheng WangYueqi HuangShuqi WangJiefang ChenGesang MeiduoMan JinXiaoying ZhangAffiliated Mental Health Center & Hangzhou Seventh People ’s Hospital, Zhejiang University School of Medicine, Hangzhou, China Source Type: research
From parasomnia to agrypnia excitata - An illustrative case on diagnostic approach
We report a case of fatal familial insomnia who initially presented with persistent limb movements in sleep, which later progressed to a state of agrypnia excitata. Here, the evaluation of abnormal movements in sleep is discussed using a step-by-step diagnostic approach. Although no cure is available for fatal familial insomnia, prompt recognition of this condition is important to facilitate proper management, including the involvement of interdisciplinary neuropalliative care. (Source: Parkinsonism and Related Disorders)
Source: Parkinsonism and Related Disorders - March 1, 2023 Category: Neurology Authors: Si-Lei Fong, Alfand Marl F. Dy Closas, Thien Thien Lim, Phooi Leng Lean, Ee Chin Loh, Shen-Yang Lim, Ai Huey Tan Source Type: research
Altered energy metabolism in Fatal Familial Insomnia cerebral organoids is associated with astrogliosis and neuronal dysfunction
by Simote T. Foliaki, Anna Smith, Benjamin Schwarz, Eric Bohrnsen, Catharine M. Bosio, Katie Williams, Christina D. Orr ú, Hailey Lachenauer, Bradley R. Groveman, Cathryn L. Haigh
Fatal familial insomnia (FFI) is a rare neurodegenerative disease caused by a dominantly inherited single amino acid substitution (D178N) within the prion protein (PrP). No in vitro human brain tissue model for this disease has previously been available. Consequently, how this mutation exerts its damaging effect on brain cells is still unknown. Using CRISPR-Cas9 engineered induced pluripotent stem cells, we made D178N cerebral organoids and com...
Source: PLoS Genetics - January 19, 2023 Category: Genetics & Stem Cells Authors: Simote T. Foliaki Source Type: research
Preventive pharmacological treatment in subjects at risk for fatal familial insomnia: science and public engagement
Volume 16, Issue 1, December 2022, Page 66-77 . (Source: Prion)
Source: Prion - June 23, 2022 Category: Biology Authors: Gianluigi Forloni Ignazio Roiter Vladimiro Artuso Manuel Marcon Walter Colesso Elviana Luban Ugo Lucca Mauro Tettamanti Elisabetta Pupillo Veronica Redaelli Francesco Mariuzzo Giulia Boscolo Buleghin Alice Mariuzzo Fabrizio Tagliavini Roberto Chiesa Anna Source Type: research
