Zebra Day: Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob Disease is a prion disease which causes progressive dementia and myoclonic seizure activity/jerks. One variant causes psychiatric disturbances, ranging from new-onset depression preceding the dementia and myoclonus, to hallucinations and delusions. EEG will show focal periodic sharp wave pattern. The 14-3-3 protein in CSF is a marker for CJD, but is not always present. Sometimes, total protein in the CSF is high as well. MRI, showing hyperintense signals in parts of the brain, can also help in diagnosis.

Unfortunately, there is no cure for CJD, or for any of the other prion diseases. Treatment is supportive and palliative only.