Week 2 lecture 2- Neurodegenerative disorders Flashcards
Classifications of neurodegenerative disorders
Syndromic vs nosological
Three neurodegenerative disorder
Alzheimer’s disease
Frontotemporal dementia
Parkinson’s disease
Classifications of dementia
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
Neurodegenerative diseases characterised by:
Progressive degeneration of cortical neurons
-Causing loss of function
-Cell death, loss of volume and structure
3 categories of dementia in DSM5
- Mild- Difficulties with instrumental activities of daily living (eg. housework, managing money)
- Moderate- Difficulties with basic activities of daily living (eg. feeding, dressing)
- Severe: Fully dependent
Clinical Dementia Rating (CDR)
-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
Alzheimers disease
-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
Neuropathology of AD
Cortical Neuron loss
-Temporal- Hippocampus (Mediotemporal Atrophy)
-Parietal
-Other cortical/subcortical areas
Amyloid beta plaques
Neurofibrillary tangle TAU
Mediotemporal atrophy
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
Dementia symptoms and criteria
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
Language presentation
Logopenic PPA (Primary progressive aphasia)- Impairment in word finding
Visuospatial presentation
Posterior Cortical Atrophy (PCA)
Impairment in visuospatial cognition
Executive dysfunction
Behavioural-dysexecutive variant
Impairments in executive functions, behavioural changes (disinhibition, apathy)
AD dementia- Examinations
-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
Indicators of alzheimers in neuropsychological exam
-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
Neuropsychological tests for memory
- 15 Words test (Can make a distinction between immediate recall and delayed recall)
- Rey Complex Figure Test
- Doors
Neuropsychological tests for attention and processing speed
- WAIS IV – Digit span
- Reaction time task
- Letter Digit Substitution Test
- Trail Making Test A
Neuropsychological tests for visuo-perception/motor function
Rey Complex Figure Test (copy)
* The Visual Object and Space Perception Battery (VOSP)
* Trail Making Test A
* Reaction time task
Neuropsychological tests for language
- Semantic and phonemic fluency
- Boston Naming Test
Neuropsychological tests for executive functions
- Trail Making Test B
- BADS Key Search, Zoo maps
Neuropsychological tests for social cognition
- FEEST, Ekman 60 Faces Test: Emotion
Recognition
Frontotemporal dementia
Lot less common than alzheimers
2-10% of patients with dementia
Onset between 50-60
Neuropathology of FTD
- 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
3 variants of FTD dementia
- Behavioural variant
- Primary progressive aphasia
-Nonfluent/ agrammatic variant, or Progressive Nonfluent Aphasia (PNFA)
-Semantic variant, or semantic dementia - Logopenic variant: Alzheimers disease
