Dementia

Question 1

leading cause of dementia?

Answer 1

Alzheimer’s disease

Question 2

Alzheimer’s disease

1) risk factors?
2) histology?
3) pathology?
4) which areas of the brain tend to be affected first?

Answer 2

1) age, family history (mutations in APP or PSEN genes), head injury, Down’s syndrome
2) excess accumulation of extracellular B-amyloid plaques and intracellular neurofibrillary tangles (aggregates of Tau protein)
3) widespread cortical atrophy: narrowing of gyri, widening of sulci
4) nucleus basalis of meynert, entorhinal cortex and hippocampus

Question 3

which inherited gene
1) decreases
2) increases
risk of Alzhiemer’s?

Answer 3

1) ApoE2
2) ApoE4
(4 greater than 2> increases risk)

Question 4

clinical features of Alzheimer’s disease?

Answer 4

gradually progressive worsening memory. Appointments forgotten, items misplaced, inability to remember recent events: impaired semantic memory. Some lack insight (collateral important)

difficulty planning, decision making, learning new tasks

not ass with behaviour/ personality change in early stages (unlike frontotemporal or vascular dementia)

Question 5

diagnosis of alzheimer’s?

Answer 5

clinical
supported by tests e.g. MMSE
+ MRI and SPECT to diff from FTD and vascular dementia
CSF: phosphorylated tau protein, decreased B-amyloid

Question 6

management of Alzheimer’s disease?

Answer 6

MDT: social support, prognostic information, advice on legal/ benefits issues

CBT if anxiety/ depression

Mild-mod: acetylcholinersterase inhibitors e.g. Donepezil, galantamine, rivastigmine

2nd line/ severe: memantine (NMDA receptor antagonist)

Question 7

complication complication of Alzheimer’s disease?

Answer 7

haemorrhagic stroke: beta-amyloid deposits causing weakening of cerebral vasculature (cerebral amyloid angiopathy)

Question 8

what is frontotemporal dementia?

Answer 8

group of neurodegenerative disorders characterised by relatively focal frontal and temporal cerebral atrophy

Question 9

3 types of frontotemporal dementia? (inc which is most common)

Answer 9

behavioural variant (most common)
primary progressive aphasia
- semantic dementia
- progressive non-fluent aphasia

Question 10

Ix in frontotemporal dementia?

Answer 10

MRI

LP to differentiate from AD

Question 11

2nd most common form of dementia in the western population?

Answer 11

vascular dementia (due to vascular changes secondary to cerebrovascular diseases)

Question 12

symptoms of vascular dementia?

Answer 12

attentional impairments, behavioural changes (disinhibition), cognitive slowing, focal neuro problems, difficulty walking/ falls

Question 13

diagnosis of vascular dementia?

Answer 13

MRI: infarcts, white matter changes, patchy reduced attenuation in the cortex

Question 14

What is creutzfeldt-jakob disease?

Answer 14

transmissible prion neurodegenerative disease which causes cell death (sponiform changes in cortex) and astrocytosis

Question 15

presentation of creutzfeldt-jakob disease?

Answer 15

rapidly progressing dementia, ataxia, myoclonic jerks

Question 16

Ix in creutzfeldt-jakob disease?

Answer 16

EEG best initial test: pseudoperiodic sharp wave activity

CSF: elevated 14-3-3 protein

Question 17

symptoms of behavioural variant of FTD?

Answer 17

loss of inhibition, apathy, repetitive behaviours and difficulty planning

Memory is preserved in early disease and behavioural symptoms are more pronounced

Question 18

symptoms of semantic dementia variant of FTD?

Answer 18

impaired word comprehension, object naming (‘animal’ for ‘dog). Loss of recognition of familiar objects/ faces

Question 19

symptoms of progressive non-fluent aphasia variant of FTD?

Answer 19

slow/ hesitant speech, word-finding deficits, agrammatism

may have orofacial apraxia (test by coughing, swallowing)

Question 20

Which gene is most associated with early-onset Alzheimer’s disease?

Answer 20

presenilin 1 (PSEN1)