Which condition presents with dementia and myoclonus?

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Multiple Choice

Which condition presents with dementia and myoclonus?

Creutzfeldt-Jakob disease presents with rapidly progressive dementia and prominent myoclonus. This prion disorder typically starts with rapid cognitive decline over weeks to months and quickly develops involuntary muscle jerks (myoclonus), often alongside gait disturbance, ataxia, or visual symptoms. Diagnostic clues that support this pattern include EEG showing periodic sharp-wave complexes, MRI diffusion-weighted imaging with cortical ribboning and basal ganglia involvement, and CSF markers such as 14-3-3 protein (though not perfectly specific) or newer RT-QuIC testing for prions. The other conditions don’t characteristically combine dementia with myoclonus: neurosyphilis can cause cognitive decline but myoclonus is not a defining feature; Lewy body dementia usually presents with fluctuating cognition, vivid visual hallucinations, and parkinsonism rather than prominent myoclonus; normal pressure hydrocephalus shows the classic triad of gait disturbance, urinary incontinence, and cognitive impairment without myoclonus.

Creutzfeldt-Jakob disease presents with rapidly progressive dementia and prominent myoclonus. This prion disorder typically starts with rapid cognitive decline over weeks to months and quickly develops involuntary muscle jerks (myoclonus), often alongside gait disturbance, ataxia, or visual symptoms. Diagnostic clues that support this pattern include EEG showing periodic sharp-wave complexes, MRI diffusion-weighted imaging with cortical ribboning and basal ganglia involvement, and CSF markers such as 14-3-3 protein (though not perfectly specific) or newer RT-QuIC testing for prions. The other conditions don’t characteristically combine dementia with myoclonus: neurosyphilis can cause cognitive decline but myoclonus is not a defining feature; Lewy body dementia usually presents with fluctuating cognition, vivid visual hallucinations, and parkinsonism rather than prominent myoclonus; normal pressure hydrocephalus shows the classic triad of gait disturbance, urinary incontinence, and cognitive impairment without myoclonus.

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