All::Neurology::Diseases::Creutzfeldt-Jakob disease

Intro

What is Creutzfeldt-Jakob disease?

a rapidly progressive neurological condition caused by prion proteins

What is the common presentation of Creutzfeldt-Jakob disease?

  • dementia (rapid onset)
  • myoclonus

What is seen in CSF in Creutzfeldt-Jakob disease?

usually normal

What is seen in EEG in Creutzfeldt-Jakob disease?

biphasic, high amplitude sharp waves (only in sporadic CJD)

What is seen in MRI in Creutzfeldt-Jakob disease?

hyperintense signals in the basal ganglia and thalamus

What symptoms are seen in New variant CJD?

psychological symptoms such as anxiety, withdrawal and dysphonia are the most common presenting features

What age is New variant CJD typically seen?

25 years

What is the most common cause of Creutzfeldt-Jakob disease?

Sporadic CJD, caused by the spontaneous misfolding of prion-protein in an individual

What age is Sporadic CJD typically seen?

65 years