Clinical findings in Creutzfeldt-Jakob disease mimicking dementia with Lewy bodies

Rare histotype of sporadic Creutzfeldt-Jakob disease, clinically suspected as corticobasal degeneration

“…However, they found that the clinical features most prevalent in sCJD-CBS cases were alien limb phenomena, limb apraxia, rigidity, sensory loss, myoclonus and cognitive impairment, all of which were present in the case we have presented. Marin et al reported a case of suspected CJD causing a clinical syndrome similar to DLB,10 however this report also lacked pathological confirmation of the diagnosis. In a previous cohort study, 46% of sCJD patients met clinical diagnostic criteria for DLB 11.…”

Creutzfeldt‐Jakob Disease: an under‐Recognized Cause of Dementia

“…By sharing common features with other dementia types [3][4][5][6][7] and being rare 8 (with an incidence rate estimated as one to two cases per million people per year), CJD can easily be missed or misdiagnosed. The diagnosis of CJD is based on clinical and instrumental (EEG, cerebrospinal fluid, magnetic resonance imaging) parameters, 9 but special attention is required to make a correct diagnosis.…”

The role of microglia in prion diseases and possible therapeutic targets: a literature review

“…Creutzfeldt-Jakob disease (CJD) is a rare and fatal condition that leads to progressive neurodegeneration due to gliosis, vacuolation of central nervous system tissue, and loss of neurons[ 1 ].It is a human type of transmissible spongiform encephalopathy and it is classified into four different phenotypes, the most common being the sporadic CJD (sCJD) – characterized by the absence of previous risk factors[ 2 ]. Although some defend the role of an agent with a nucleic acid genome as a possible aetiology, the accumulation of misfolded forms of cellular prion protein (PrP C ), known as infectious prion proteins (PrP Sc ), is a well-established mechanism of disease development[ 3 ].…”