Cognitive Neurology

Question 1

what are the three criteria for a diagnosis of a dementia?

Answer 1

evidence of significant
cognitive decline

deficits interfere with everyday activities

not better explained by another process

Question 2

what is the main clinical feature of transient global amnesia?

Answer 2

abrupt onset amnesia

anterograde more commonly than retrograde

Question 3

how long does a transient global amnesia last?

Answer 3

4-6 hours usually

always under 24

Question 4

what age group is affected by transient global amnesia?

Answer 4

> 50’s

Question 5

what factors could possible trigger a transient global amnesia?

Answer 5

emotion

changes in temperature

Question 6

what are the clinical features of transient epileptic amnesia?

Answer 6

forgetful, repetitive questioning

short lived

Question 7

what more serious symptom are transient epileptic amnesias associated with?

Answer 7

temporal lobe seizures

Question 8

what drugs do transient epileptic amnesias respond to?

Answer 8

anti epileptic medications

Question 9

what are the clinical features of a functional cognitive impairment?

Answer 9

everyday forgetfulness impacting on function

fluctuating symptoms

Question 10

name the most common prion disease

Answer 10

Creutzfeldt-Jakob disease

Question 11

what is the pathophysiology behind Creutzfeldt-Jakob disease?

Answer 11

prion protein becomes misfolded and can’t be broken down and causes neurodegeneration

one misfolded causes a domino effect - changes all it touches

Question 12

how is Creutzfeldt-Jakob disease diagnosed?

Answer 12

biomarkers
MRI C
CSF tests

Question 13

what can be seen histologically in Creutzfeldt-Jakob disease?

Answer 13

spongiform change later on

Question 14

what mediates limbic encephalitis?

Answer 14

inflammation and antibodies

Question 15

how does limbic encephalitis present?

Answer 15

short term memory deficits
seizures
behavioural changes

Question 16

what is seen on MRI and CSF in limbic encephalitis?

Answer 16

inflammatory changes

antibodies in CSF

Question 17

what can limbic encephalitis be associated with?

Answer 17

underlying autoimmune disease or malignancy

Question 18

how is limbic encephalitis managed?

Answer 18

treat underlying cause

immunosuppression

Question 19

what histopathological features are seen in Alzheimer’s disease?

Answer 19

extracellular amyloid plaques

intracellular neurofibrillary tangles

Question 20

what effect do extracellular amyloid plaques have in Alzheimer’s disease?

Answer 20

disrupt normal cell function

induces apoptosis

Question 21

what effect do intracellular neurofibrillary tangles have in Alzheimer’s disease?

Answer 21

disrupt the cytoskeleton leading to cell death

Question 22

what is the initial symptom of Alzheimer’s disease?

Answer 22

general forgetfulness

Question 23

what does degeneration of the hippocampus and parietal lobes in Alzheimer’s disease lead to?

Answer 23

apraxia

visuospatial difficulties

Question 24

what is early onset Alzheimer’s disease?

Answer 24

Alzheimer’s disease in people under 65

often associated with genetic influences

Question 25

what is sporadic Alzheimer's disease?

Answer 25

Alzheimer's disease in people over 65

Question 26

name two atypical presentations of Alzheimer's disease

Answer 26

posterior cortical atrophy | progressive primary aphasia

Question 27

how does posterior cortical atrophy present?

Answer 27

visuospatial disturbance often referred from ophthalmology

Question 28

how does progressive primary aphasia present?

Answer 28

language dominant symptoms initially followed by further cognitive decline

Question 29

name three types of progressive primary aphasia and the language issue associated

Answer 29

semantic - naming logogenic - repeating non fluent - effortful

Question 30

what four investigations can be done for Alzheimer's disease?

Answer 30

MRI SPECT CSF amyloid ligand imaging

Question 31

what is seen on MRI in Alzheimer's disease?

Answer 31

atrophy of temporal/parietal lobes

Question 32

what is seen on SPECT in Alzheimer's disease?

Answer 32

temperoparietal metabolism decreased

Question 33

what is seen in CSF in Alzheimer's disease?

Answer 33

decreased amyloid: increased tau ratio

Question 34

what is the management of Alzheimer's disease?

Answer 34

manage vascular risk factors | acetylcholine boosting treatments

Question 35

what two acetylcholine boosting treatments are available for Alzheimer's disease?

Answer 35

cholinesterase inhibitors | NMDA receptor blocker

Question 36

name two cholinesterase inhibitors that can be used for Alzheimer's disease

Answer 36

rivastigmine | galantamine

Question 37

name an NMDA receptor blocker that can be used for Alzheimer's disease

Answer 37

memantine

Question 38

when does frontotemporal dementia come on?

Answer 38

early onset - usually <65

Question 39

what is the pathophysiology behind frontotemporal dementia?

Answer 39

protein aggregation leads to cell damage, resulting in neurodegeneration

Question 40

what three investigations may be done for frontotemporal dementia?

Answer 40

MRI SPECT CSF

Question 41

what is seen in MRI in frontotemporal dementia?

Answer 41

atrophy of the fronts-temporal lobes

Question 42

what is seen on SPECT in frontotemporal dementia?

Answer 42

reduced frontotemporal metabolism

Question 43

what is seen in CSF in frontotemporal dementia?

Answer 43

increased tau | normal amyloid

Question 44

what is the management of frontotemporal dementia?

Answer 44

trial of trazodone or anti-psychotics to help behavioural features

Question 45

what safety management should be implemented in frontotemporal dementia?

Answer 45

controlled access to things like food, money and the internet structured activities

Question 46

what legal consideration needs to be thought about in frontotemporal dementia?

Answer 46

power of attorney for future choices

Question 47

when does vascular dementia come on?

Answer 47

late onset - majority of patients are over 65

Question 48

what are the two core criteria for a diagnosis of vascular dementia?

Answer 48

presence of cerebrovascular disease a clear temporal relationship between the onset of dementia and the cerebrovascular disease

Question 49

how does vascular dementia present?

Answer 49

reduced attention executive dysfunction slowed processing

Question 50

how is vascular dementia managed?

Answer 50

manage vascular risk factors | cholinesterase inhibitor

Question 51

when does dementia with Lewy bodies come on?

Answer 51

late onset - majority >65

Question 52

what are the core criteria for the diagnosis of dementia with Lewy bodies?

Answer 52

fluctuating cognition recurrent well formed visual hallucinations +/- presence of extra pyramidal features

Question 53

what investigations are done for dementia with Lewy bodies?

Answer 53

none really - a clinical diagnosis

Question 54

how is dementia with Lewy bodies managed?

Answer 54

small dose levodopa | trial cholinesterase inhibitors

Question 55

when does Parkinson's disease dementia come on?

Answer 55

late onset, most >65 usually after 20 years of having Parkinson's

Question 56

how does Parkinson's disease dementia present?

Answer 56

symptoms of Parkinson's dementia may have hallucinations

Question 57

when do symptoms of Huntington's come on?

Answer 57

30-50

Question 58

what causes Huntington's?

Answer 58

expansion of the CAG trinucleotide repeat in the huntingtin mean = produces a neurodegenerative protein

Question 59

what is seen on MRI in Huntington's?

Answer 59

loss of caudate heads

Question 60

where is a patient >65 with gradual onset dementia and no additional neurology referred to?

Answer 60

old age psychiatry

Question 61

where is someone <65 or with any unusual features of additional neurology referred to?

Answer 61

neurology