Histopathology 20: Neurodegeneration

Question 1

What are prion diseases ?

Answer 1

Prions are proteinaceous infections particles. They can cause Prion protein (PrP) to miss fold and become soluble.
This can cause neurodegenerative diseases in humans.

Question 2

What is the main sign of prion disease ?

Answer 2

Rapid neurodegenerative decline

Question 3

List 4 Prion types of prion diseases seen in humans ?

Answer 3

CJD- Creutzfeldt-Jakob disease
GSSS- gerstmann-straussler-sheinker syndrome
Kuru
Fatal familial insomnia

Question 4

List 2 common features of prion diseases on histology ?

Answer 4

Spongiform change

Prion protein deposition

Question 5

Describe the progression in symptoms of Alzheimer’s disease ?

Answer 5

recent memory loss (cognitive) -> dressing apraxia (parietal lobe) -> executive function problems, personality changes, disinhibition (frontal lobe)

Question 6

List the classical pathological features of Alzheimer’s disease ?

Answer 6

Extracellular plaques
Neurofibrillary tangles
Cerebral amyloid angiopathy
Neuronal loss (cerebral atrophy)

Question 7

What is the protein responsible for plaque formation in Alzheimer’s disease ?

Answer 7

Amyloid-beta

Question 8

How is the Amyloid-beta protein formed ?

Answer 8

APP (amyloid precursor protein) is cleaved by enzymes in the cell membrane.

Question 9

Why is Tau protein a useful marker in neuropathology (Braak staging) ?

Answer 9

It spreads throughout the brain and the parts it affects is closely related to the symptoms seen in the patient.

Question 10

What disease with tauopathy is commonly seen in boxers ?

Answer 10

Chronic Traumatic Encephalopathy

Occurs after multiple repetitive traumatic head injuries

Question 11

The presence of ………. characterises Parkinson’s disease

Answer 11

Lewy bodies

Question 12

Death of……… cells from the ………….up to the basal ganglia causes Parkinson’s disease

Answer 12

Dopaminergic

Substantial nigra

Question 13

What are Lewy bodies ?

Answer 13

Accumulation of the protein Alpha-synuclein.

Seen as Smooth hyaline inclusions in the pigmented cells of the substantia nigra.

Question 14

What is the gold standard test for Parkinson’s disease ?

Answer 14

Alpha-synuclein immunostaining

Question 15

Where in the brain does Alzheimer’s disease originate ?

Answer 15

The temporal lobe (hippocampus)

Question 16

Where does Parkinson’s disease originate in the CNS ?

Answer 16

The medulla

Question 17

List 3 of the main differentials that cause Parkinsonism (Parkinson like symptoms)

Answer 17

MSA - multisystem atrophy
CBD - corticobasal degeneration
PSP - progressive supranuclear palsay

Question 18

What is considered to be prodromal for Parkinson’s disease ?

Answer 18

Sleep disorders

Question 19

Where do you get alpha-synuclein deposition in MSA disease ?

Answer 19

Glial cells

Question 20

Which disease is characterised by Pick Bodies and balloon neurons ?

Answer 20

Pick’s disease

Question 21

What symptoms does Pick’s disease cause ?

Answer 21

Dysexecutive syndrome
Aggression
Disinhibition

(Frontal lobe)

Question 22

What are Pick bodies ?

Answer 22

Tau deposits

Question 23

List 4 areas of cognitive disturbance affected in Dementia ?

Answer 23

Amnesia: Memory loss
Aphasia: Language disorder
Apraxia: Unable to carry out purposeful tasks
Agnosia: Unable to identify objects/ faces

Question 24

Which parkinson plus syndrome presents with the characteristic feature of “Alien limbs” ?

Answer 24

Corticobasal syndrome (CBD)

Question 25

Which cells produce myelin ?

Answer 25

oligodendrocytes

Question 26

List features of Multiple Sclerosis ?

Answer 26

• Optic neuritis
• Parasthesia
• Weakness
• Loss of coordination
• Incontinence
• Depression

Question 27

Lumbar puncture with CSF sample showing myelin basic protein and proteo-lipid protein.

Most likely diagnosis ?

Answer 27

MS