1,273
Views
0
CrossRef citations to date
0
Altmetric
Case Reports

PART and ARTAG tauopathies at a relatively young age as a concomitant finding in sporadic Creutzfeldt-Jakob disease

, , , &
Pages 138-142 | Received 11 May 2021, Accepted 17 Jun 2021, Published online: 05 Jul 2021
 

ABSTRACT

Interactions between prion protein (PrP) and tau protein have long been discussed, especially in relation to the pathogenesis of neurodegenerative diseases. The presence of tauopathy in the genetic forms of Creutzfeldt-Jakob disease (CJD) brains is not uncommon. Molecular interactions between PrP and tau protein have been demonstrated in animal models; the role is attributed to the structural properties of misfolded isoform of the host-encoded prion protein (PrPSc) aggregates, especially amyloid, which contributes to the phosphorylation of tau protein, which is reflected in the frequent occurrence of tau pathology in inherited prion amyloidoses. The question is the relationship between PrPSc and hippocampal tau pathology without amyloid deposits (i.e. PART and ARTAG) in sporadic CJD (sCJD). The co-occurrence of these two proteinopathies in sCJD brains is quite rare. These pathological entities have been described in only a few cases of sCJD, all of them were older than 70 years. There have been speculations about the possibility of accelerating the course of pre-existing tauopathy or the possibility of accelerating the ageing process in the CJD brains. Here we present the clinical course and neuropathological findings of a patient with sCJD in whom the above mentioned tauopathies PART and ARTAG, considered to be typical for older age, were found as early as 58 years of age. According to the available information, this case represents an unusually early occurrence of age-related tauopathies not only in relation to sCJD, but also in general.

Disclosure statement

All authors declare no competing interest.

Additional information

Funding

The study was funded by a grant project from the Ministry of Health of the Czech Republic [NV19-04-00090]; grant from the Ministry of Health, Czech Republic for the conceptual development of a research organization [FNOL, 0098892]; by European Regional Development Fund – Project ENOCH [No. Z.02.1.01/0.0/0.0/16_019/0000868] and grant [IGA-LF-2021-020].