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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| Language: | English |
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PAGEPress Publications
2025-06-01
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| 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 |
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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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| format | Article |
| id | doaj-art-b9c8026c39204f07a5ec2c69d5e960a4 |
| institution | Kabale University |
| issn | 1877-9344 1877-9352 |
| language | English |
| publishDate | 2025-06-01 |
| publisher | PAGEPress Publications |
| 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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