What is the typical clinical presentation of Creutzfeldt-Jakob disease?

The typical clinical presentation of Creutzfeldt-Jakob disease (CJD) is characterized by progressive dementia, bilateral rigidity, myoclonus, stupor, and ultimately leads to death. CJD is a rare, degenerative brain disorder caused by prion proteins that misfold and aggregate, leading to severe neurological damage.

In the early stages, patients may present with mood changes, memory loss, or personality changes, which can develop into more pronounced cognitive decline and confusion, ultimately resulting in severe dementia. Along with cognitive symptoms, motor symptoms such as bilateral rigidity and myoclonus, which are sudden involuntary muscle contractions, are hallmark signs of the disease. Stupor or decreased responsiveness can occur as the disease progresses toward its terminal stages.

The rapid progression of symptoms is a key feature, typically culminating in death within months to a few years post-diagnosis. This clinical trajectory distinguishes CJD from other neurological conditions where symptoms may present differently and where the timeline of symptom progression can vary significantly.

The other options reflect symptoms or clinical features that do not align with the typical progression of CJD. For instance, while seizures may occur in some cases, they are not central to the CJD presentation unlike the combination of rapid cognitive