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Case Report: Three Case Reports of Rapidly Progressive Dementias and Narrative Review
Rapidly progressive dementia (RPD) is a heterogeneous group of diseases characterized by cognitive impairment and other neurological disorders developed in a short span of fewer than 2 years. Currently viewed as new and infrequent entities, most medical personnel have little understanding of it. Nev...
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Published in: | Case reports in neurology 2022-09, Vol.14 (3), p.441-455 |
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creator | Clavijo, Carlos Andrés Portilla Buenaventura, Ana María Benavides Albornoz, Galo Santiago Muñoz Cabrera, Juan José Murillo Reyes, María Camila Chauvez Gallego, Alejandra Hurtado González, Carlos Alberto Ospina Otalvaro, Sebastian Marmolejo Escobar, Carlos Steven Quebrada Mera, Karen Julieth Lenis, Paola Andrea Gutiérrez Arango García, Lina María Lucumí, Armando |
description | Rapidly progressive dementia (RPD) is a heterogeneous group of diseases characterized by cognitive impairment and other neurological disorders developed in a short span of fewer than 2 years. Currently viewed as new and infrequent entities, most medical personnel have little understanding of it. Nevertheless, they significantly compromise many patients’ quality of life. Here, we drive 3 clinical cases that evolve as RPD with different etiologies. Case 1:70-year-old woman presented to the emergency with neuropsychiatric syndrome for 18 days. The researchers identified inflammatory cerebrospinal fluid (CSF), protein 14-3-3-positive T-tau protein, MRI: T2 and FLAIR hyperintensities in bilateral caudate nuclei with diffusion restriction, EEG shows a generalized periodic pattern with triphasic wave morphology. Case 2:29-year-old man with cognitive impairment and faciobrachial dystonia seizure. The diagnosis was confirmed by achieving elevated antibodies against voltage-gated potassium channels. Case 3:A 49-year-old woman with encephalopathy and myoclonic seizures; EEG and MRI showed subtle changes. The patient also had a normal CSF but a positive CBA serologic NMDA-R antibody test. We described fundamental aspects of RPD to allow made differential diagnoses in patients with cognitive impairment and encephalopathy. Establishing an early and accurate diagnosis can benefit patients with RPD etiologies that are treatable and even reversible, decreasing in morbidity and mortality. |
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Currently viewed as new and infrequent entities, most medical personnel have little understanding of it. Nevertheless, they significantly compromise many patients’ quality of life. Here, we drive 3 clinical cases that evolve as RPD with different etiologies. Case 1:70-year-old woman presented to the emergency with neuropsychiatric syndrome for 18 days. The researchers identified inflammatory cerebrospinal fluid (CSF), protein 14-3-3-positive T-tau protein, MRI: T2 and FLAIR hyperintensities in bilateral caudate nuclei with diffusion restriction, EEG shows a generalized periodic pattern with triphasic wave morphology. Case 2:29-year-old man with cognitive impairment and faciobrachial dystonia seizure. The diagnosis was confirmed by achieving elevated antibodies against voltage-gated potassium channels. Case 3:A 49-year-old woman with encephalopathy and myoclonic seizures; EEG and MRI showed subtle changes. The patient also had a normal CSF but a positive CBA serologic NMDA-R antibody test. We described fundamental aspects of RPD to allow made differential diagnoses in patients with cognitive impairment and encephalopathy. Establishing an early and accurate diagnosis can benefit patients with RPD etiologies that are treatable and even reversible, decreasing in morbidity and mortality.</description><identifier>ISSN: 1662-680X</identifier><identifier>EISSN: 1662-680X</identifier><identifier>DOI: 10.1159/000525701</identifier><identifier>PMID: 36636277</identifier><language>eng</language><publisher>Basel, Switzerland: S. Karger AG</publisher><subject>Alzheimer's disease ; Antibodies ; Autoimmune diseases ; Case reports ; Case Series - General Neurology ; Convulsions & seizures ; Creutzfeldt-Jakob disease ; Dementia ; Encephalitis ; Etiology ; Hallucinations ; Memory ; Morphology ; nmdar ; Patients ; Potassium ; Proteins ; rapidly progressive dementia ; voltage-gated potassium channel complex</subject><ispartof>Case reports in neurology, 2022-09, Vol.14 (3), p.441-455</ispartof><rights>2022 The Author(s). Published by S. Karger AG, Basel</rights><rights>Copyright © 2022 by The Author(s). Published by S. Karger AG, Basel.</rights><rights>2022 The Author(s). Published by S. Karger AG, Basel . This work is licensed under the Creative Commons Attribution – Non-Commercial License http://creativecommons.org/licenses/by-nc/3.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><rights>Copyright © 2022 by The Author(s). Published by S. Karger AG, Basel 2022</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c482t-e56c9b2365d09daf7412a78013d41bb7555ff32284241b2ff32b72ad092bcf2c3</citedby><orcidid>0000-0001-9486-8529</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC9830281/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC9830281/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,727,780,784,885,27635,27924,27925,53791,53793</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/36636277$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Clavijo, Carlos Andrés</creatorcontrib><creatorcontrib>Portilla Buenaventura, Ana María</creatorcontrib><creatorcontrib>Benavides Albornoz, Galo Santiago</creatorcontrib><creatorcontrib>Muñoz Cabrera, Juan José</creatorcontrib><creatorcontrib>Murillo Reyes, María Camila</creatorcontrib><creatorcontrib>Chauvez Gallego, Alejandra</creatorcontrib><creatorcontrib>Hurtado González, Carlos Alberto</creatorcontrib><creatorcontrib>Ospina Otalvaro, Sebastian</creatorcontrib><creatorcontrib>Marmolejo Escobar, Carlos Steven</creatorcontrib><creatorcontrib>Quebrada Mera, Karen Julieth</creatorcontrib><creatorcontrib>Lenis, Paola Andrea Gutiérrez</creatorcontrib><creatorcontrib>Arango García, Lina María</creatorcontrib><creatorcontrib>Lucumí, Armando</creatorcontrib><title>Case Report: Three Case Reports of Rapidly Progressive Dementias and Narrative Review</title><title>Case reports in neurology</title><addtitle>Case Rep Neurol</addtitle><description>Rapidly progressive dementia (RPD) is a heterogeneous group of diseases characterized by cognitive impairment and other neurological disorders developed in a short span of fewer than 2 years. Currently viewed as new and infrequent entities, most medical personnel have little understanding of it. Nevertheless, they significantly compromise many patients’ quality of life. Here, we drive 3 clinical cases that evolve as RPD with different etiologies. Case 1:70-year-old woman presented to the emergency with neuropsychiatric syndrome for 18 days. The researchers identified inflammatory cerebrospinal fluid (CSF), protein 14-3-3-positive T-tau protein, MRI: T2 and FLAIR hyperintensities in bilateral caudate nuclei with diffusion restriction, EEG shows a generalized periodic pattern with triphasic wave morphology. Case 2:29-year-old man with cognitive impairment and faciobrachial dystonia seizure. The diagnosis was confirmed by achieving elevated antibodies against voltage-gated potassium channels. Case 3:A 49-year-old woman with encephalopathy and myoclonic seizures; EEG and MRI showed subtle changes. The patient also had a normal CSF but a positive CBA serologic NMDA-R antibody test. We described fundamental aspects of RPD to allow made differential diagnoses in patients with cognitive impairment and encephalopathy. 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Currently viewed as new and infrequent entities, most medical personnel have little understanding of it. Nevertheless, they significantly compromise many patients’ quality of life. Here, we drive 3 clinical cases that evolve as RPD with different etiologies. Case 1:70-year-old woman presented to the emergency with neuropsychiatric syndrome for 18 days. The researchers identified inflammatory cerebrospinal fluid (CSF), protein 14-3-3-positive T-tau protein, MRI: T2 and FLAIR hyperintensities in bilateral caudate nuclei with diffusion restriction, EEG shows a generalized periodic pattern with triphasic wave morphology. Case 2:29-year-old man with cognitive impairment and faciobrachial dystonia seizure. The diagnosis was confirmed by achieving elevated antibodies against voltage-gated potassium channels. Case 3:A 49-year-old woman with encephalopathy and myoclonic seizures; EEG and MRI showed subtle changes. The patient also had a normal CSF but a positive CBA serologic NMDA-R antibody test. We described fundamental aspects of RPD to allow made differential diagnoses in patients with cognitive impairment and encephalopathy. Establishing an early and accurate diagnosis can benefit patients with RPD etiologies that are treatable and even reversible, decreasing in morbidity and mortality.</abstract><cop>Basel, Switzerland</cop><pub>S. Karger AG</pub><pmid>36636277</pmid><doi>10.1159/000525701</doi><tpages>15</tpages><orcidid>https://orcid.org/0000-0001-9486-8529</orcidid><oa>free_for_read</oa></addata></record> |
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subjects | Alzheimer's disease Antibodies Autoimmune diseases Case reports Case Series - General Neurology Convulsions & seizures Creutzfeldt-Jakob disease Dementia Encephalitis Etiology Hallucinations Memory Morphology nmdar Patients Potassium Proteins rapidly progressive dementia voltage-gated potassium channel complex |
title | Case Report: Three Case Reports of Rapidly Progressive Dementias and Narrative Review |
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