Creutzfeldt-Jakob Disease


Case Discussion
An 84-yo woman has developed rapidly progressive dementia over 4 months. She has a low-grade fever, is very rigid, and has myoclonic jerks when startled. EEG shows triphasic sharp wave complexes.

This pattern is consistent with which diagnosis?

A. Creutzfeldt-Jakob Disease
B. Subdural hematoma
C. Cerebral vasculitis
D. HIV dementia
E. Herpes encephalitis

 
Characteristics
  • Rapidly progressive dementia over several months with myoclonus
  • Frequently have periodic synchronous bi- or triphasic sharp wave complexes on EEG
  • Tend to be younger patients
  • Transmissible (viral-like “prions”)
  • Rare (1 per million in US)
  • “Variant” CJD = mad cow disease

 
Dementia Alzheimer's Dementia
Minor Neuro-cognitive Disorder
“Pseudodementia” (Dementia Syndrome of Depression)
Vascular Dementia
Diffuse Lewy Body Dementia
Frontotemporal Dementia
Normal Pressure Hydrocephalus
Creutzfeldt-Jakob Disease