Patient with rapidly evolving neurological disease with neuropathological lesions of Creutzfeldt‐Jakob disease, Lewy body dementia, chronic subcortical vascular encephalopathy and meningothelial meningioma

Vita, Maria Gabriella; Tiple, Dorina; Bizzarro, Alessandra; Ladogana, Anna; Colaizzo, Elisa; Capellari, Sabina; Rossi, Marcello; Parchi, Piero; Masullo, Carlo; Pocchiari, Maurizio

doi:10.1111/neup.12343

Cited by 7 publications

(5 citation statements)

References 48 publications

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“…Finally, in few case reports and small series, the CJD‐specific pathology was found to co‐exist with a synucleinopathy [sCJD+dementia with Lewy bodies (LBD) , sCJD+preclinical multiple system atrophy ] or a primary tauopathy [sCJD+progressive supranuclear palsy (PSP) ]. Even more rarely, multiple proteinopathies have been documented in the same patient, namely sCJD+AD+LBD+AGD or CJD+AD+PSP+AGD .…”

Section: Co‐pathology Related To Prion‐like Protein Misfoldingmentioning

confidence: 99%

Recent advances in the histo‐molecular pathology of human prion disease

et al. 2019

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Prion diseases are progressive neurodegenerative disorders affecting humans and other mammalian species. The term prion, originally put forward to propose the concept that a protein could be infectious, refers to PrP Sc , a misfolded isoform of the cellular prion protein (PrP C ) that represents the pathogenetic hallmark of these disorders. The discovery that other proteins characterized by misfolding and seeded aggregation can spread from cell to cell, similarly to PrP Sc , has increased interest in prion diseases. Among neurodegenerative disorders, however, prion diseases distinguish themselves for the broader phenotypic spectrum, the fastest disease progression and the existence of infectious forms that can be transmitted through the exposure to diseased tissues via ingestion, injection or transplantation. The main clinicopathological phenotypes of human prion disease include Creutzfeldt-Jakob disease, by far the most common, fatal insomnia, variably protease-sensitive prionopathy, and Gerstmann-Sträussler-Scheinker disease. However, clinicopathological manifestations extend even beyond those predicted by this classification. Because of their transmissibility, the phenotypic diversity of prion diseases can also be propagated into syngenic hosts as prion strains with distinct characteristics, such as incubation period, pattern of PrP Sc distribution and regional severity of histopathological changes in the brain. Increasing evidence indicates that different PrP Sc conformers, forming distinct ordered aggregates, encipher the phenotypic variants related to prion strains. In this review, we summarize the most recent advances concerning the histo-molecular pathology of human prion disease focusing on the phenotypic spectrum of the disease including co-pathologies, the characterization of prion strains by experimental transmission and their correlation with the physicochemical properties of PrP Sc aggregates.

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Section: Co‐pathology Related To Prion‐like Protein Misfoldingmentioning

confidence: 99%

Recent advances in the histo‐molecular pathology of human prion disease

et al. 2019

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“…In the literature, the clinical and pathological features of six CJD patients with LBs have been described previously (Table 1) [15][16][17][18][19][20]. Although the clinical sequence of illness is variable in CJD, a change in mental status usually occurs before motor symptoms become apparent.…”

Section: Discussionmentioning

confidence: 96%

“…Although the clinical sequence of illness is variable in CJD, a change in mental status usually occurs before motor symptoms become apparent. Only one case has previously been reported in which PD preceded CJD [15]; the initial clinical diagnoses of the other five cases were Alzheimer's disease [16], mood disorder [17], CJD [18], mild cognitive impairment [19], and visual hallucination/delusion [20]. Because the interval between PD onset and CJD onset in the previously reported case was 2.5 years, the present case is-to the best of our knowledge-the first report of a case of PD with a typical long clinical course who later developed neuropathologically confirmed CJD.…”

Section: Discussionmentioning

confidence: 99%

Parkinson’s disease with a typical clinical course of 17 years overlapped by Creutzfeldt–Jakob disease: an autopsy case report

Kubo¹,

Matsubara

Taguchi

et al. 2021

BMC Neurol

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Background Late-stage Parkinson’s disease (PD) often presents with neuropsychiatric symptoms such as dementia, psychosis, excessive daytime sleepiness, apathy, depression, and anxiety. However, neuropsychiatric symptoms are the cardinal features of Creutzfeldt–Jakob disease (CJD), raising the possibility that CJD may be an overlooked condition when it accompanies late-stage PD. Case presentation We describe a female autopsy case of PD with a typical clinical course of 17 years, in which CJD overlapped with PD during the final year of the patient’s life. The patient died aged 85 years. Neuropathological features included widespread Lewy body-related α-synucleinopathy predominantly in the brainstem and limbic system, as well as the typical pathology of methionine/methionine type 1 CJD in the brain. Conclusions Our case demonstrates the clinicopathological co-occurrence of PD and CJD in a sporadic patient. The possibility of mixed pathology, including prion pathology, should be taken into account when neuropsychiatric symptoms are noted during the disease course of PD.

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“…В каждом из них отмечаются особая клиническая картина, например эпистатус и быстро прогрессирующая деменция [52], развитие деменции за 5 месяцев у мужчины 66 лет и измерения в ликворе RT-QuIC (скорости конверсии белка в патологический) при отрицательном 14-3-3 и T тау-белка [53]. В другом случае быстрого прогрессирования деменции обнаружено сочетание морфологических признаков БКЯ, ДТЛ, хронической подкорковой сосудистой энцефалопатии и менингиомы [54].…”

Section: нозологические различия в скорости прогрессирования деменцииunclassified

Old Age Dementia: Clinical Patterns of Progression. Part 1

Mikhailova¹

2020

Psychiatry

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Background: the clinical experience testifies to the fact, that the increase of dementia severity in late age occurs quickly in some cases and in others it proceeds slowly, which is reflected in the total duration of the disease and survival. One of the aspects of dementias research is the study of significant differences in dementia progression rates.The objective of the review was to generalize the obtained data on progression rates of late age dementias of various genesis, first of all due to Alzheimer’s disease and its associated disorders.Material and methods: papers in MedLine/PubMed bases from1990 to 2020 were selected and analyzed according to the key words: “old age”, “dementia”, “Alzheimer’s disease”, “vascular dementia”, “progression”, “progression rate”, “disease trajectories”, as well as relevant papers in the references of the analyzed works.Conclusion: the history of research of old age dementias natural course was presented in the review of scientific publications. According to the results of development of progression rates detection methods, singling out of dementias with rapid and slow increase in the severity of cognitive decline was substantiated. Works devoted to the study of frequency and nosological belonging of dementias with different progression rates were considered. In the most recent studies prognostic models with detection of various trajectories of the course of the disease were developed. The concept of various dementias progression rates admittedly has practical meaning for provision of diagnostic and treatment assistance and planning of medical and social support measures for patients with dementia and their families. Differentiation of dementia progression clinical patterns during formation of comparable groups of patients seems appropriate for investigation of new therapy methods, as well as in clinical-biological studies of pathogenesis.

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Patient with rapidly evolving neurological disease with neuropathological lesions of Creutzfeldt‐Jakob disease, Lewy body dementia, chronic subcortical vascular encephalopathy and meningothelial meningioma

Cited by 7 publications

References 48 publications

Recent advances in the histo‐molecular pathology of human prion disease

Recent advances in the histo‐molecular pathology of human prion disease

Parkinson’s disease with a typical clinical course of 17 years overlapped by Creutzfeldt–Jakob disease: an autopsy case report

Old Age Dementia: Clinical Patterns of Progression. Part 1

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