Neurodegeneration Flashcards
What are prion diseases
A series of diseases with a common molecular pathology.
Prion = proteinaceous infectious only.
What is the pathology of prion disease
A transmissible factor, with no DNA or RNA involved.
Involves the misfolding of PrP proteins.
What are some prion diseases in humans (4)
Creutzfeldt-Jakob disease (CJD).
Gerstmann-Straussler-Sheinker syndrome.
Kuru
Fatal familial insomnia
What is the histopathology of prion disease (2)
Spongiform change
Prion protein deposits
What is New Variant CJD (vCJD)
A prion disease.
A sporadic neuropsychiatric disorder.
How does vCJD differ to CJD
vCJD has a longer duration than CJD
What age group does vCJD affect
Patients <45 years old
What are the clinical features of vCJD (2)
Cerebellar ataxia
Dementia
What is vCJD linked to
BSE
What are the neuropathological features of alzheimer’s disease (4)
Extracellular plaques (senile plaques)
Neurofibrillary tangles
Cerebral amyloid angiopathy (CAA)
Neuronal loss (cerebral atrophy)
What is the protein deposited and accumulated in AD
Amyloid-beta plaques.
What is amyloid-beta formed from
Byproduct of APP processing.
What are the deposits seen in AD (2)
Amyloid-beta.
Tau
What does CTE stand for
Chronic traumatic encephalopathy
What is CTE
A form of dementia characterised by repeated head trauma (e.g. in boxers) with an early age of onset
What is the histological hallmark of PD
Lewy bodies
What is the protein found within lewy bodies
Alpha-synuclein
What is considered the gold standard for diagnosing PD
Alpha-synuclein immunostaining
What is the staging system for PD called
Braak
What are some causes of Parkinsonism (8)
Idiopathic Parkinson's disease. Drug-induced Parkinsonism Multiple system atrophy Progressive supranuclear palsy Corticobasal degeneration Vascular pseudoparkinsonism Alzheimer's changes Fronto-temporal neurodegenerative disorders
What are the histological hallmarks of Picks disease (4)
Fronto-temporal atrophy
Marked gliosis and neuronal loss
Balloon neurons
Tau positive Pick bodies.
What is the inheritance pattern of FTDP-17 syndromes
Autosomal dominant
What is the structure of tau (6)
Single gene on 17q21
16 exons
Alternative splicing gives rise to 6 isoforms
3R or 4R tau (microtubule binding domains)
Two further inserts with unknown function
Shortest form (3R0/0N)
What is used to help diagnose dementias molecularly
Tau molecular analysis
Where to get the rest of the info for this lecture
Year 4 notes!
