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The cognitive phenotypes of Creutzfeldt-Jakob disease: comparison with secondary metabolic encephalopathy.

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Title: The cognitive phenotypes of Creutzfeldt-Jakob disease: comparison with secondary metabolic encephalopathy.
Authors: Giovagnoli, Anna Rita (AUTHOR), Di Fede, Giuseppe (AUTHOR), Rossi, Giacomina (AUTHOR), Moda, Fabio (AUTHOR), Grisoli, Marina (AUTHOR), Bugiani, Orso (AUTHOR)
Source: Neurological Sciences. Jun2022, Vol. 43 Issue 6, p3703-3716. 14p. 5 Charts, 1 Graph.
Subjects: Creutzfeldt-Jakob disease, Cerebrospinal fluid examination, Positron emission tomography, Brain diseases, Magnetic resonance imaging, Hyperperfusion
Abstract: Background: Rapidly progressive cognitive impairment is a diagnostic criterion in Creutzfeldt-Jakob disease (CJD), but the diagnosis is usually reached when an analysis of cognitive aspects is no longer possible. Objective: This study aims to delineate the cognitive phenotypes preceding severe dementia in CJD compared to secondary metabolic encephalopathies (SME) with rapid cognitive impairment. Methods: Patients with rapidly progressive neurological symptoms underwent neuropsychological evaluation, analysis of cerebrospinal fluid (CSF) and codon 129 polymorphism of the prion protein gene (PRNP), magnetic resonance imaging (MRI), and single positron emission computed tomography (99mTcSPECT). CSF real-time quaking-induced conversion analysis was applied in CJD patients. Based on literature and clinical expertise, cognitive profiles were correlated with brain areas. Results: Thirty-one patients were diagnosed with CJD (n = 17) or SME; 77 cases of CJD were extracted from the literature. In patients with CJD, verbal initiative, lexical search, long-term memory, attention, and abstract reasoning were the most frequently impaired abilities. Cognitive profiles were mainly related to dysfunction in fronto-temporal areas. Furthermore, they were consistent with areas of hypoperfusion detected by 99mTc SPECT in six patients and cortical and subcortical MRI hyperintensities in eight and 14 patients, respectively, and were similar to those described in the literature. In contrast, cognitive profiles were different from those in SME characterized by visuospatial and constructive deficits relating to posterior brain areas. Conclusion: In CJD, clinical and neuropsychological analyses outline a salient cognitive phenotype suggestive of fronto-temporal dysfunction preceding severe dementia. This phenotype is different from that observed in other rapidly progressive encephalopathies. [ABSTRACT FROM AUTHOR]
Copyright of Neurological Sciences is the property of Springer Nature and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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  Data: The cognitive phenotypes of Creutzfeldt-Jakob disease: comparison with secondary metabolic encephalopathy.
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  Data: <searchLink fieldCode="AR" term="%22Giovagnoli%2C+Anna+Rita%22">Giovagnoli, Anna Rita</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Di+Fede%2C+Giuseppe%22">Di Fede, Giuseppe</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Rossi%2C+Giacomina%22">Rossi, Giacomina</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Moda%2C+Fabio%22">Moda, Fabio</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Grisoli%2C+Marina%22">Grisoli, Marina</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Bugiani%2C+Orso%22">Bugiani, Orso</searchLink> (AUTHOR)
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  Data: <searchLink fieldCode="JN" term="%22Neurological+Sciences%22">Neurological Sciences</searchLink>. Jun2022, Vol. 43 Issue 6, p3703-3716. 14p. 5 Charts, 1 Graph.
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  Data: <searchLink fieldCode="DE" term="%22Creutzfeldt-Jakob+disease%22">Creutzfeldt-Jakob disease</searchLink><br /><searchLink fieldCode="DE" term="%22Cerebrospinal+fluid+examination%22">Cerebrospinal fluid examination</searchLink><br /><searchLink fieldCode="DE" term="%22Positron+emission+tomography%22">Positron emission tomography</searchLink><br /><searchLink fieldCode="DE" term="%22Brain+diseases%22">Brain diseases</searchLink><br /><searchLink fieldCode="DE" term="%22Magnetic+resonance+imaging%22">Magnetic resonance imaging</searchLink><br /><searchLink fieldCode="DE" term="%22Hyperperfusion%22">Hyperperfusion</searchLink>
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  Data: Background: Rapidly progressive cognitive impairment is a diagnostic criterion in Creutzfeldt-Jakob disease (CJD), but the diagnosis is usually reached when an analysis of cognitive aspects is no longer possible. Objective: This study aims to delineate the cognitive phenotypes preceding severe dementia in CJD compared to secondary metabolic encephalopathies (SME) with rapid cognitive impairment. Methods: Patients with rapidly progressive neurological symptoms underwent neuropsychological evaluation, analysis of cerebrospinal fluid (CSF) and codon 129 polymorphism of the prion protein gene (PRNP), magnetic resonance imaging (MRI), and single positron emission computed tomography (99mTcSPECT). CSF real-time quaking-induced conversion analysis was applied in CJD patients. Based on literature and clinical expertise, cognitive profiles were correlated with brain areas. Results: Thirty-one patients were diagnosed with CJD (n = 17) or SME; 77 cases of CJD were extracted from the literature. In patients with CJD, verbal initiative, lexical search, long-term memory, attention, and abstract reasoning were the most frequently impaired abilities. Cognitive profiles were mainly related to dysfunction in fronto-temporal areas. Furthermore, they were consistent with areas of hypoperfusion detected by 99mTc SPECT in six patients and cortical and subcortical MRI hyperintensities in eight and 14 patients, respectively, and were similar to those described in the literature. In contrast, cognitive profiles were different from those in SME characterized by visuospatial and constructive deficits relating to posterior brain areas. Conclusion: In CJD, clinical and neuropsychological analyses outline a salient cognitive phenotype suggestive of fronto-temporal dysfunction preceding severe dementia. This phenotype is different from that observed in other rapidly progressive encephalopathies. [ABSTRACT FROM AUTHOR]
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  Data: <i>Copyright of Neurological Sciences is the property of Springer Nature and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
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              Text: Jun2022
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