Histopathology 20: Neurodegeneration Flashcards
What are prion diseases ?
Prions are proteinaceous infections particles. They can cause Prion protein (PrP) to miss fold and become soluble.
This can cause neurodegenerative diseases in humans.
What is the main sign of prion disease ?
Rapid neurodegenerative decline
List 4 Prion types of prion diseases seen in humans ?
CJD- Creutzfeldt-Jakob disease
GSSS- gerstmann-straussler-sheinker syndrome
Kuru
Fatal familial insomnia
List 2 common features of prion diseases on histology ?
Spongiform change
Prion protein deposition
Describe the progression in symptoms of Alzheimer’s disease ?
recent memory loss (cognitive) -> dressing apraxia (parietal lobe) -> executive function problems, personality changes, disinhibition (frontal lobe)
List the classical pathological features of Alzheimer’s disease ?
Extracellular plaques
Neurofibrillary tangles
Cerebral amyloid angiopathy
Neuronal loss (cerebral atrophy)
What is the protein responsible for plaque formation in Alzheimer’s disease ?
Amyloid-beta
How is the Amyloid-beta protein formed ?
APP (amyloid precursor protein) is cleaved by enzymes in the cell membrane.
Why is Tau protein a useful marker in neuropathology (Braak staging) ?
It spreads throughout the brain and the parts it affects is closely related to the symptoms seen in the patient.
What disease with tauopathy is commonly seen in boxers ?
Chronic Traumatic Encephalopathy
Occurs after multiple repetitive traumatic head injuries
The presence of ………. characterises Parkinson’s disease
Lewy bodies
Death of……… cells from the ………….up to the basal ganglia causes Parkinson’s disease
Dopaminergic
Substantial nigra
What are Lewy bodies ?
Accumulation of the protein Alpha-synuclein.
Seen as Smooth hyaline inclusions in the pigmented cells of the substantia nigra.
What is the gold standard test for Parkinson’s disease ?
Alpha-synuclein immunostaining
Where in the brain does Alzheimer’s disease originate ?
The temporal lobe (hippocampus)
Where does Parkinson’s disease originate in the CNS ?
The medulla
List 3 of the main differentials that cause Parkinsonism (Parkinson like symptoms)
MSA - multisystem atrophy
CBD - corticobasal degeneration
PSP - progressive supranuclear palsay
What is considered to be prodromal for Parkinson’s disease ?
Sleep disorders
Where do you get alpha-synuclein deposition in MSA disease ?
Glial cells
Which disease is characterised by Pick Bodies and balloon neurons ?
Pick’s disease
What symptoms does Pick’s disease cause ?
Dysexecutive syndrome
Aggression
Disinhibition
(Frontal lobe)
What are Pick bodies ?
Tau deposits
List 4 areas of cognitive disturbance affected in Dementia ?
Amnesia: Memory loss
Aphasia: Language disorder
Apraxia: Unable to carry out purposeful tasks
Agnosia: Unable to identify objects/ faces
Which parkinson plus syndrome presents with the characteristic feature of “Alien limbs” ?
Corticobasal syndrome (CBD)
Which cells produce myelin ?
oligodendrocytes
List features of Multiple Sclerosis ?
- Optic neuritis
- Parasthesia
- Weakness
- Loss of coordination
- Incontinence
- Depression
Lumbar puncture with CSF sample showing myelin basic protein and proteo-lipid protein.
Most likely diagnosis ?
MS
