Creutzfeldt-Jacob disease

Question 1

Causes of CJD

Answer 1

-Transmissable prion ( proteinaceous particle)
-Sporadic
-New variant
-Familial
-Acquired:
Cadaveric growth
Dura mater grafts
Blood transfusions

Question 2

Sporadic CJD properties

Answer 2

• Usually late onset >60

- Very rapid progression

Question 3

Variant CJD properties

Answer 3

Usually early onset <40 years
Associated with Bovine spongy encephalopathy - eating infected material
Early behavioural changes are more obvious
Longer course

Question 4

Symptoms :

• Early
• Late

Answer 4

• Initial behavioural abnormalities
• Dementia
• Myoclonus (spasmic episodes which occur in one group of muscles)

Progresses to global neurological decline
-Motor abnormalities
Cerebellar ataxia ( Ataxia is a neurological sign consisting of lack of voluntary coordination of muscle movements that can include gait abnormality, speech changes and abnormalities in eye movements.)
Extrapyrimidal: tremor, rigidity, bradykinesia, dystonia
Pyrimidal: hyper-reflexia, spasticity, weakness

• Cortical blindness (blindness due to damage to the cortex)
• Seizures

Question 5

Differential for CJD

Answer 5

• Alzheimers
• Inflammatory encephalopathies
• CNS vasculitis

Question 6

Prognosis of CJD

Answer 6

• fast progression

- usually death within 6 months

Question 7

Investigations for CJD

Answer 7

MRI :
-Pulvinar sign in Variant CJD (Symmetrical hyper intensity)
EEG 
-General periodic complexes
-Normal in initial stages
CSF:
-Raised protein or normal 
-Immunoassay 14-3-3 raised (quite non-specific)