Week 2 lecture 2- Neurodegenerative disorders

Question 1

Classifications of neurodegenerative disorders

Answer 1

Syndromic vs nosological

Question 2

Three neurodegenerative disorder

Answer 2

Alzheimer’s disease
Frontotemporal dementia
Parkinson’s disease

Question 3

Classifications of dementia

Answer 3

Syndromic
For DSM4: Dementia
-Cognitive decline in 2 or more neurocognitive domains
For DSM5: Major Neurocognitive disorder
-Significant cognitive decline in one or more cognitive domains
-Interference with everyday life
Mild Neurocognitive Disorder (MCI-Mild Cognitive Impairment in DSM 4)
-Moderate cognitive decline in one or more cognitive domains
-No interference with everyday life

Question 4

Neurodegenerative diseases characterised by:

Answer 4

Progressive degeneration of cortical neurons
-Causing loss of function
-Cell death, loss of volume and structure

Question 5

3 categories of dementia in DSM5

Answer 5

1. Mild- Difficulties with instrumental activities of daily living (eg. housework, managing money)
2. Moderate- Difficulties with basic activities of daily living (eg. feeding, dressing)
3. Severe: Fully dependent

Question 6

Clinical Dementia Rating (CDR)

Answer 6

-0 (none) to 3 (severe)
-Originally based on a structured interview with patient or close relative
-Now based on whole exam
-If it is 2 or higher not allowed to drive

Question 7

Alzheimers disease

Answer 7

-60-70% of patients with dementia suffer from alzheimers disease
-Most patients are over 65
-Age is most common cause of dementia
-Incidence of dementia increases with age

Question 8

Neuropathology of AD

Answer 8

Cortical Neuron loss
-Temporal- Hippocampus (Mediotemporal Atrophy)
-Parietal
-Other cortical/subcortical areas
Amyloid beta plaques
Neurofibrillary tangle TAU

Question 8

Mediotemporal atrophy

Answer 8

Under 75 yrs: Score of 2 or higher is abnormal
Over 75 yrs: Score of 3 or higher is abnormal
High correlation between these and alzheimer’s disease
Some people have deposits of these in their brain but don’t have dementia
Presence of these is not enough to develop alzheimer’s disease

Question 9

Dementia symptoms and criteria

Answer 9

Cognitive deficits in 2 or more cognitive domains
Insidious onset- Symptoms develop within months
Must be cognitive decline in that period
Initial and most prominent cognitive deficits are:
-Amnestic presentation:
Impairment in memory and other domains
-Nonamnestic presentation:
Language presentation
Visuospatial presentation
Executive dysfunction

Question 10

Language presentation

Answer 10

Logopenic PPA (Primary progressive aphasia)- Impairment in word finding

Question 11

Visuospatial presentation

Answer 11

Posterior Cortical Atrophy (PCA)
Impairment in visuospatial cognition

Question 12

Executive dysfunction

Answer 12

Behavioural-dysexecutive variant
Impairments in executive functions, behavioural changes (disinhibition, apathy)

Question 13

AD dementia- Examinations

Answer 13

-Medical exam
-Observation
-History taking
-Patient, spouse, children
-Complaints vs impairments
-Cognition
- Neuropsychological screening (MMSE/MOCA)
-Neuropsychological exam (Over age of 70 this is not specific enough)
-Addition
-MRI
-CSF
-PET scans

Question 14

Indicators of alzheimers in neuropsychological exam

Answer 14

-Lowering of A beta proteins in CSF
-Rise of Tau
-If its negative you dont have it but if its positive you dont necessarily have it

Question 15

Neuropsychological tests for memory

Answer 15

• 15 Words test (Can make a distinction between immediate recall and delayed recall)
• Rey Complex Figure Test
• Doors

Question 16

Neuropsychological tests for attention and processing speed

Answer 16

• WAIS IV – Digit span
• Reaction time task
• Letter Digit Substitution Test
• Trail Making Test A

Question 17

Neuropsychological tests for visuo-perception/motor function

Answer 17

Rey Complex Figure Test (copy)
* The Visual Object and Space Perception Battery (VOSP)
* Trail Making Test A
* Reaction time task

Question 18

Neuropsychological tests for language

Answer 18

• Semantic and phonemic fluency
• Boston Naming Test

Question 19

Neuropsychological tests for executive functions

Answer 19

• Trail Making Test B
• BADS Key Search, Zoo maps

Question 20

Neuropsychological tests for social cognition

Answer 20

• FEEST, Ekman 60 Faces Test: Emotion
  Recognition

Question 21

Frontotemporal dementia

Answer 21

Lot less common than alzheimers
2-10% of patients with dementia
Onset between 50-60

Question 22

Neuropathology of FTD

Answer 22

• Cortical neuron loss
  -Frontal
  -Temporal (anterior)
• TDP-43 aggregates (50%)
• Neurofibrillary TAU tangles –
  Pick’s bodies (45%)
• Fused in sarcoma protein (5%)
• 20-30% hereditary, autosomal
  dominant
  -Atrophy can be symmetrical or asymmetrical

Question 23

3 variants of FTD dementia

Answer 23

1. Behavioural variant
2. Primary progressive aphasia
   -Nonfluent/ agrammatic variant, or Progressive Nonfluent Aphasia (PNFA)
   -Semantic variant, or semantic dementia
3. Logopenic variant: Alzheimers disease

Question 24

Behavioral variant dementia- Symptoms and criteria

Answer 24

• Progressive deterioration of behaviour and/or cognition