Differential diagnosis of prion diseases in the vast chapter of degenerative encephalopathies, a challenge for the internist: two case reports compared

Prion diseases, or transmissible spongiform encephalopathies, fall under the big chapter of differential diagnosis of degenerative diseases of the central nervous system. The cause is prions, which are altered forms of the prion protein (PrP). In pathology, PrP changes its form, becoming a prion, i...

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Main Authors: Filomena Pietrantonio, Angela Ciamei, Gabriele Angelo Vassallo, Luca Moriconi, Margherita Lordi
Format: Article
Language:English
Published: PAGEPress Publications 2025-06-01
Series:Italian Journal of Medicine
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Online Access:https://www.italjmed.org/ijm/article/view/2075
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author Filomena Pietrantonio
Angela Ciamei
Gabriele Angelo Vassallo
Luca Moriconi
Margherita Lordi
author_facet Filomena Pietrantonio
Angela Ciamei
Gabriele Angelo Vassallo
Luca Moriconi
Margherita Lordi
author_sort Filomena Pietrantonio
collection DOAJ
description Prion diseases, or transmissible spongiform encephalopathies, fall under the big chapter of differential diagnosis of degenerative diseases of the central nervous system. The cause is prions, which are altered forms of the prion protein (PrP). In pathology, PrP changes its form, becoming a prion, i.e., an infectious protein capable of inducing normal PrP to assume the pathological form. The accumulation of PrP in brain cells creates very small bubbles (vacuoles) that lead to the formation of microscopic holes that make the brain tissue resemble a sponge (hence the term spongiform encephalopathies). These are very rare diseases with a very long latency. After the first symptoms appear, the disease evolves rapidly. There is no treatment, and the outcome is often inauspicious. Two clinical cases of prion disease occasionally found in patients admitted from emergency rooms to the Department of Internal Medicine are described.
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institution Kabale University
issn 1877-9344
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language English
publishDate 2025-06-01
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record_format Article
series Italian Journal of Medicine
spelling doaj-art-b9c8026c39204f07a5ec2c69d5e960a42025-08-20T03:27:44ZengPAGEPress PublicationsItalian Journal of Medicine1877-93441877-93522025-06-0110.4081/itjm.2025.2075Differential diagnosis of prion diseases in the vast chapter of degenerative encephalopathies, a challenge for the internist: two case reports comparedFilomena Pietrantonio0https://orcid.org/0000-0003-1119-0869Angela Ciamei1Gabriele Angelo Vassallo2Luca Moriconi3Margherita Lordi4Internal Medicine Unit, Medical Area Department, Ospedale dei Castelli, Local Health Authority Roma 6, Rome; St. Camillus University of Health Sciences, RomeInternal Medicine Unit, Medical Area Department, Ospedale dei Castelli, Local Health Authority Roma 6, RomeBarone Lombardo Hospital, CanicattìInternal Medicine Unit, Local Health Authority Rieti Medical Department, S. Camillo De Lellis Hospital, RietiGeriatric Intensive Care Unit, AOU Careggi di Firenze, Florence Prion diseases, or transmissible spongiform encephalopathies, fall under the big chapter of differential diagnosis of degenerative diseases of the central nervous system. The cause is prions, which are altered forms of the prion protein (PrP). In pathology, PrP changes its form, becoming a prion, i.e., an infectious protein capable of inducing normal PrP to assume the pathological form. The accumulation of PrP in brain cells creates very small bubbles (vacuoles) that lead to the formation of microscopic holes that make the brain tissue resemble a sponge (hence the term spongiform encephalopathies). These are very rare diseases with a very long latency. After the first symptoms appear, the disease evolves rapidly. There is no treatment, and the outcome is often inauspicious. Two clinical cases of prion disease occasionally found in patients admitted from emergency rooms to the Department of Internal Medicine are described. https://www.italjmed.org/ijm/article/view/2075Prion diseasesdegenerative encephalopathiesdifferential diagnosisinternal medicine roleresearch model
spellingShingle Filomena Pietrantonio
Angela Ciamei
Gabriele Angelo Vassallo
Luca Moriconi
Margherita Lordi
Differential diagnosis of prion diseases in the vast chapter of degenerative encephalopathies, a challenge for the internist: two case reports compared
Italian Journal of Medicine
Prion diseases
degenerative encephalopathies
differential diagnosis
internal medicine role
research model
title Differential diagnosis of prion diseases in the vast chapter of degenerative encephalopathies, a challenge for the internist: two case reports compared
title_full Differential diagnosis of prion diseases in the vast chapter of degenerative encephalopathies, a challenge for the internist: two case reports compared
title_fullStr Differential diagnosis of prion diseases in the vast chapter of degenerative encephalopathies, a challenge for the internist: two case reports compared
title_full_unstemmed Differential diagnosis of prion diseases in the vast chapter of degenerative encephalopathies, a challenge for the internist: two case reports compared
title_short Differential diagnosis of prion diseases in the vast chapter of degenerative encephalopathies, a challenge for the internist: two case reports compared
title_sort differential diagnosis of prion diseases in the vast chapter of degenerative encephalopathies a challenge for the internist two case reports compared
topic Prion diseases
degenerative encephalopathies
differential diagnosis
internal medicine role
research model
url https://www.italjmed.org/ijm/article/view/2075
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