Infections Flashcards
What is prion disease
Group of neurodegenerative diseases caused by prions which accumulate and damage nerve cells
What is the most common prion disease
CJD
What causes CJD
Sporadic - protein -> prion
New variant - consuming meat from cow with bovine spongiform encephalopathy
Familial - genetic
Acquired - infection
How does sporadic present
Rapidly progressive dementia Myoclonic jerks >60 Behaviour abnormality Cerebellar ataxia Parkinsonism - Tremor / rigid / bradykinesia Weakness Hyperreflexia / UMN sign Seizure Blidness
What is prognosis
Death in 6 months
13 if new variant
How does new variant present
Younger onset
Early behaviour change
Anxiety / withdrawal / dysphonia
Better prognosis
How do you Dx
MRI = hyperextense
EEG
CSF = usually normal
Immunoassay for brain protein
How do you Rx
No treatment currently
What are differentials
Viral encephalitis PML HIV dementia Lyme's / neurosyphillis Whipple's Alzheimer's Lewybody Tumour
What is Reye’s
Fatty infiltration of liver, pancreas, kidney and brain
What causes Reye’s
Post viral infection
Aspirin use
Common in children
What are the symptoms
Severe progressive encephalopathy Confusion Seizures N+V Cerebral oedema Coma Abnormal LFT Hypoglycaemia
How do you treat
Supportive
What are other rare infections in brain / affect nerves
Poliomyelitis Lyme's Neurosyphillis Rabies Tetanus Botulism
What does poliovirus 1,2,3 (enterovirus) cause and where does it affect
Poliomyelitis
Affects anterior horn cell to affect LMN