Creutzfeldt-Jakob disease (CJD)

Question 1

Aetiology

Answer 1

• Causes
o Transmissible (iatrogenic): TRANSPLANT SURGERY…
o Genetic mutation
o Most = spontaneous/sporadic

Question 2

Pathophysiology

Answer 2

• Deposits of prion proteins throughout brain → cause irreversible changes to neighbouring tissues

Question 3

CFs

Answer 3

• Rapidly deteriorating dementia, cerebellar and extra-pyramidal signs (+ pyramidal)
• Myoclonus
• Death w/in a year
  IMPORTANT (EXAM): Vascular dementia and CJD will likely both have neurological signs in the question → if says myoclonus (small jerks) think of CJD

Question 4

Pyramidal signs

Answer 4

Pyramidal signs (pyramidal tracts)
• Corticospinal tract → weakness, spasticity (hypertonia), hyperreflexia, plantar extension (upgoing), myoclonus, Babinski sign
• Corticobulbar tract (to face) → signs in face

Question 5

extrapyamidal signs

Answer 5

Extra-pyramidal signs (Extrapyramidal tracts)

• Tremor, chorea, akathisia, rigidity…etc (think parkinson’s)

Question 6

Ix

Answer 6

CT → atrophy of cortex and cerebellum

Question 7

Other causes of dementia

Answer 7

Normal pressure hydrocephalus

Huntington’s disease

Question 8

Normal pressure hydrocephalus

Answer 8

>70
Triad (EXAM):
1.	Dementia (w/ frontal lobe dysfunction: personality…)
2.	Urinary incontinence
3.	Gait disturbance (wide gait)

Question 9

Huntington’s

Answer 9

• Autosomal dominant (so strong FHx)
• Chorea (abnormal involuntary movements) of face/hands/arms/legs
• Gait abnormalities
• Dementia = late feature