Current issues about the morphological features of Creutzfeldt-Jakob disease (CJD): review article

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Background. Creutzfeldt-Jakob disease (CJD) is a difficult to diagnose prion disease, characterized by the development of rapidly progressive dementia and a long incubation period, which leads to death within the first year in 90% of cases. Despite the existence of criteria for intravital diagnosis and modern technologies, histotyping of the material still remains the gold standard for making a final diagnosis. The sporadic form remains the most common among all types of CJD.

Objective. Analysis of current data on CJA, systematization of the information obtained to facilitate the differential diagnosis of CJD types.

Methods. Data from 11 studies with a total of 817 confirmed cases of CJD were used for this work. The phenotypic features for each known type and subtype were analyzed, and the systematic sequence of distribution of the PrPsc prion protein for the two most common types of CJD was also indicated.

Results. Due to the studies reviewed, we are convinced that the true diversity of CJD histotypes is much wider than previously thought. Along with typical M1, M2C, M2T, VV1, and VV2 CJD forms, researchers distinguish transitional forms – VV1–VV2, and subtypes with specific morphological features – MV 1C–2PL.

Conclusion. The obtained data can be applied in practice if it is necessary to differentiate the types of CJD

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作者简介

M. Kuznetsova

Pirogov Russian National Research Medical University

Email: vitaliysidnyaev@mail.ru
ORCID iD: 0000-0001-8243-5902

Department of Topographic Anatomy and Operative Surgery n.a. Acad. Yu.M. Lopukhin, Institute of Anatomy and Morphology n.a. Acad. Yu.M. Lopukhin

俄罗斯联邦, Moscow

M. Svishcheva

Moscow Financial and Industrial University “Synergy”

Email: vitaliysidnyaev@mail.ru
ORCID iD: 0000-0001-9825-1139

Department of Medical and Biological Disciplines, Faculty of Medicine

俄罗斯联邦, Moscow

Vitaly Sidnyaev

Moscow Financial and Industrial University “Synergy”

编辑信件的主要联系方式.
Email: vitaliysidnyaev@mail.ru
ORCID iD: 0009-0002-5327-7794

Clinical Psychologist, Student, Department of Medical and Biological Disciplines, Faculty of Medicine

俄罗斯联邦, Moscow

参考

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2. Fig. 1. Spongiosis with small vacuoles (cerebral cortex, SBC MM/MV1, stained with hematoxylin and eosin) [4]

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3. Fig. 2. PrPSc distribution scheme for MM1 genotype [6]

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4. Fig. Z.L- Diffuse distribution of PrPSc in the deep layers of the cortex; B - plaque-like deposits of PrPSc in the cerebellum (sBKJ W2, IGH, x400) [7]

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5. Fig. 4. PrPSc distribution scheme for the W2 genotype [6]

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6. Fig. 5. Spongiosis with vacuoles of intermediate size (SBKYA - W1, stained with hematoxylin and eosin) [4]

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7. Fig. 6. A, B - Large merging vacuoles (hematoxylin and eosin, x200) and near-vacuole deposition of PrPSc in the temporal lobe cortex (sBKJ - MM2C, staining with hematoxylin and eosin, IGH, x400). [7]

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8. Fig. 7. k, B. Unicentric plaques of the Kuru type in the transition zone between the granular and cellular layers of the cerebellum (SBKYA - MV2K, stained with hematoxylin and eosin, IHC, x400) [7].

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9. Fig. 8. Severe neuron lesions and reactive gliosis in the absence of spongiose changes in the medial-dorsal thalamic nucleus (A) and olive (B) (sBKJ MM2T, stained with hematoxylin and eosin, x200) [7]

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