Sporadic Creutzfeldt-Jakob disease with extremely long 14-year survival period.

ABSTRACT:

Background and purpose

Sporadic Creutzfeldt-Jakob disease is a rapidly progressing and highly variable neurodegenerative disease with heterogeneous clinical presentation and a median survival time from diagnosis to death of 4-6 months.

Methods

We report a rare case of a 61-year-old woman with a history of initially rapidly progressive dementia, with subsequent development of pyramidal and extrapyramidal signs and with an unusually long survival period of 14 years. Initial magnetic resonance imaging evaluation, single-photon emission computed tomography, and electroencephalogram did not show relevant alterations.

Results

The postmortem examination of the brain showed diffuse spongiform change, gliosis, and neuronal loss along with abnormal immunostaining of prion prote

SUBMITTER: Kortazar-Zubizarreta I 

PROVIDER: S-EPMC8457091 | biostudies-literature | 2021 Sep

REPOSITORIES: biostudies-literature