Neurodegenerative diseases histopath

Question 1

General description of neurodengerative diseases

Answer 1

Progressive, irreversible conditions leading to neuronal loss. Usually du to accumulation of mis-folded proteins (IC or EC). Result in dementia

Question 2

Define dementia & define agnosia, aphasia, apraxia

Answer 2

Global impairment of cognitive function + personality without change in consciousness.
Includes memory prob + at least 1 of the following: agnosia (loss of ability to recognise), apraxia - can’t carry out learned purposeful tasks, aphasia - language receptive/ expressive

Question 3

Pathological protein that is mis-folded in each of the following: Alzheimer’s, Dementia with LB, Frontotemporal dementia linked to Chr 17, Pick’s disease & corticobasal degen

Answer 3

Tau & beta-amyloid
Alpha-synuclein & ubiquitin
Tau
Tau
Tau

Question 4

Alzheimer’s dementia brain changes: (4)

Answer 4

General atrophy
widened sulci
Narrow gyri
enlarged ventricles - most marked in frontal & temporal with loss of cholinergic neurons

Question 5

Diagnosis of Alzheimer’s (2) & treatment (3)

Answer 5

Clinical diagnosis. Senile plaques of beta-amyloid protein & neurofibrillary tangles of tau protein
Rx is symptomatic: anti-cholinesterases, nAChR agnoists, glutamate antagonists

Question 6

Dementia with LB: Clinical features (5)

Answer 6

Psychological disturbances occur early
Visual hallucinations (liliputian?)
Day-to day fluctuations in cognitive performance
Motor signs of Parkinsonism
Recurrent falls & syncope
(Pathologically indistinguishable from PD)

Question 7

Idiopathic PD: Pathophysiology, Clin features (5), Histology (2)

Answer 7

Loss of stim of motor cortex by basal ganglia - due to death of dopaminergic neurons in substantia nigra
TRAP: Tremor, Rigidity, Akinesia, Postural instability +/- psych features - PD dementia, hallucinations
LBs present in affected neurons - alpha synuclein protein is main component, mutations reported in familial PD

Question 8

MS: what is it? Age group, Presentation (2), classifications (2)

Answer 8

Autoimmune demyelinating disease
Focal sx: optic neuritis, poor coordination
Classification: primary progressive (10%) and relapsing & remitting

Question 9

Path of MS & EMQ giveaways (2)

Answer 9

MS plaques showing sharp margins of myelin loss

EMQ - Myelin basic protein & PROTEO-LIPID protein

Question 10

Multiple system atrophy: what is it? Shy Drager, Striatonigral, Olivopontocerebellar

Answer 10

Degenerative neurodisorder present very similar to PD, but poor response to PD meds
Shy Drager - autonomic dysfunction
Striatonigral - movement difficulty
Olivopontocerebellar - difficulty with balance and coordination