Dementia

Question 1

Normal Pressure Hydrocephalus

Sx
Imaging
Lx
Tx

Answer 1

Triad - urinary incontinence/urgency, dementia/confusion, gait disturbances - ataxic or practice

Ix - Hydrocephalus with min cortical atrophy
LP - high pressure, normal counts

Rx - Shunt (30-50% will improve)

Question 2

Headache exacerbated by coughing/valsalva

Answer 2

Intracranial hypotension

Question 3

HIV related dementia

-cardinal features

Answer 3

Psychomotor retardation, apathy, impaired memory

CD4 <200

Question 4

Wernicke’s encephalopathy

Korsakoff

Answer 4

W - confusion, ataxia, ophtalmoplegia

K - similar to above, chronic. Confabulation and Poor recall is main feature

Question 5

Alzheimers

Age group
Risk Factors
Genetics

Answer 5

> 60 yoa, prevalence doubled every 5 years after 65
RF - age, fly hx
Genetics - Apolipoprotein (APP) e4, Presenilin -1, Presenelin 2.
Others - Trisomy, DM, Head trauma

Question 6

Alzheimers

Pathophysiology

Answer 6

1. Neurofibrillary tangles
   - tau becomes hyperphosphorylated
2. Amyloid beta plaques
   - due to improper cleaving of amyloid protein
3. Decreased neurotransmitters esp Ace and nicotinic cholinergic

Question 7

Alzheimers classic features

Answer 7

Memory impairments first
Then language and visuospatial
May have symmetric akinetic-rigid dystonic syndrome.

50% of patients with “Mild Cognitive Impairment” will progress to AD over 4 years

Late features
Capgras syndrome - belief caregiver is an imposter
Disrupted sleep-wake patterns

Question 8

Imaging AD

Answer 8

Postetior predominant cortical atrophy

Question 9

Early prominent gait disturbances

Mild memory loss

Answer 9

Vascular or NPH

Question 10

Resting tremor, stooped posture, bradykinesia

Answer 10

PD

Question 11

Fluctuating alertness, visual hallucinations

Answer 11

LBD

Question 12

Loss of proprioception and vibration

Answer 12

B12 def

Question 13

Rapid progression, prominent psych and myoclonic features

Answer 13

CJD

Question 14

Prominent behavioural changes, focal ant-predominant atrophy on imaging

Answer 14

FTD

Question 15

AD Management

Answer 15

Cholinesterase inhibitors - donepezil, galantamine, rivastigmine
NMDA receptor antagonists - memantine

Others
Vit E
Vit B, omega 3 fatty acid

No evidence/benefit
Ginko biloba - no evidence, not harmful
Statins
NSAIDs

Harmful - Oestrogen replacement

Question 16

Cholinesterase inhibitors
MOA
AE

Answer 16

Donepezil, Galantamine, Rivastigmine
Decreases cholinesterase activity, increases available Ach

Can use for 6m and cont IF clinically meaningful response

Donepezil - heart block
Rivastigmine - GI
Galantamine - GI

Question 17

Memantine
MOA
AE

Answer 17

Memantine
○ Glutamate, the main excitatory neurotransmitter in the CNS, binds to NDMA receptor
○ Excessive stimulation leads to excitotoxicity and damage
○ No evidence of benefit in milder disease

AE- dizziness, confusion, hallucinations

Question 18

LBD

Age group
Risk Factors
Genetics

Answer 18

Mean age 75 years
Distinctive - hallucinations, cog fluctuation, parkinsonism, sleep d/o
M:F = 4:1

Genetics - alpha-synuclein gene
Others - Presenilin 1 and 2 + APP e4 (similar to AD)

Question 19

LBD Pathophysiology

Answer 19

profound deficiency of Ach - correlates with degree of hallucination but not severity of dementia

Dopamine loss in caudate nuclear and substantial nigra

Abnormal alpha-synuclein deposits –> neuronal death –> profound dopamine dysfunction

Abnormal protein inclusions
alpha-synuclein protein aka Lewy bodies

Question 20

LBD Features

Answer 20

Central feature - progressive cog decline/dementia

Core features - fluctuating cognition, recurrent visual hallucinations, spontaneous features of parkinsonism (but must present AFTER the onset of dementia)

Supporting - REM sleep d/o, low dopamine, neuroleptic sensitivity (usually with typical antipsychotics), autonomic dysfunction, syncope etc

*memory impairment comes later unlike in AD

Question 21

LBD Mx

Answer 21

Cholinesterase inhibitors - greater benefit in LBD than AD
NMDA receptor antagonists
Atypical antipsychotics - quetiapine
Levodopa/carbidopa - for parkinsonian sx

Question 22

FTD

features, age on onset, genetics

Answer 22

Social and emotional disturbance - apathy, disinhibition, compulsivity, overeating

often occurs <65 yoa, highly heritable

Genetics - C9ORF72, GRN, MAPT

Question 23

FTD pathophysio

Answer 23

MAPT gene – Tau
Progranulin gene - ubiquitin inclusion
FTLD-TDP43

Question 24

FTD diagnosis, management

Answer 24

3 of 6

• disinhibition
• apathy/inertia
• loss of sympathy/empathy
• compuslvie behavious
• hyperoralitity
• dysexecutive neuropsychological profile

Mx - avoid typical antipsychotics
-SSRI
-Behaviour modifications
-Cholinesterase inhibitors/memantine - don't work 
Prognosis is poor, more rapid than AD

Question 25

Corticobasal syndrome presentation pathopys Mx

Answer 25

slow progressive dementia + movement disorder p/w asymmetric rigidity, myoclonus, dystonia and apraxia, sometimes have alien limb --> eventually bilateral Tau deposition, can occur with overlap No treatment ?SSRI

Question 26

Parkinson's Plus Syndrome Pathophys Clinical syndrome

Answer 26

Similar to Parkinsons but.. - poor response to levodopa - early autonomic system involvement - early onset post instability with frequenters falls - more visual hallucinations in demnetia - supranuclear gaze palsy

Question 27

Cause of death in elderly population in Aus 1. 2.

Answer 27

1. IHD | 2. Dementia

Question 28

Mild cog impairment aka mild neurocognitive d/o

Answer 28

mild cog impairment, decline from previous | not severe enough to interfere with independence of ADLs

Question 29

Vascular dementia

Answer 29

unilateral spastic weakness or increase tendon reflexes evidence of "focal" brain damange unevenly distributed cog issues ie memory, reasoning, thinking etc

Question 30

LATE - Limbic-Predominant Age-Related TDP-43 Encephalopathy

Answer 30

New dementia type - characterized by the presence of hyperphosphorylated TDP-43

Question 31

Memory clinic benefits

Answer 31

One Australian RCT found improved psychosocial status for carers at six months after visiting a memory clinic compared to those visiting a GP (Logiudice 1999). An RCT and economic evaluation in the Netherlands found no evidence of a significant difference in cost between memory clinics and general practitioner care

Question 32

SSRI in dementia

Answer 32

citalopram - strongest evidence | good as first line for agitation

Question 33

Agitation and aggression in dementia - pharmaco

Answer 33

risperidone | olanzapine

Question 34

Prevention of cognitive decline - FINGER Trial 2015

Answer 34

Improvement in cognition with diet, exercise, vascular risk monitoring and cognitive training

Question 35

MSA

Answer 35

Multisystems atrophy Lewy bodies, have Parkinsons ike features Autonomic dysfunction ++ and cerebellar symptoms MRI - hot cross bun sign

Question 36

PSP

Answer 36

Progressive Supranuclear plasy Vertical gaze palsy, postural instability Frontal lobe sx, Parkinsons like symptoms - bradykinesia, dementia, dysarthria MRI - hummingbird sign

Question 37

Parkinsons Dementia

Answer 37

78% of patients with Parkinsons Problems with cognition, mood behaviour and thought usually executive function Depression, apathy, anxiety - common