Degenerative diseases Flashcards

1
Q

what does degenerative disease in the motor neurons cause

A

motor neurone disease (MND)

in upper and lower motor neurons (UMN +/- LMN)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what does degenerative disease in the cerebral cortex cause

A

alzheimers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what does degenerative disease in the basal ganglia and brain stem cause (2)

A

parkinsons

huntingtons

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

is dementia a normal part of the ageing process

A

no just happens in a lot of older people

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what % of >80yo have dementia

A

20%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

do you need to inform the DVLA if you have dementia

A

yes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what is the most common cause of dementia

A

alzheimers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what causes alzheimers

A

multifactorial - genetic (family history) and environmental

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

if someone presents with alzheimers <65yo what do you need to ask

how do they often present

A

family history of early onset alzhiemers

visuospatial disturbance - present to ophthalmology
aphasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what genetic disorder predisposes someone to alzheimers

A

down syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what happens to the brain pathologically in alzhemiers (3)

A
cortical atrophy (frontal, parietal and temporal lobe) 
ventricular dilation - to compensate for cortical atrophy
amyloid angiopathy (plaques)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what condition can ventricular dilation in alzheimers predispose/cause

A

normal pressure hydrocephalus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

alzhiemers brain with amyloid angiopathy, what does it stain with

A

congo red

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

which area of the brain is affected first in alzheimers

A

nucleus basalis of meynert

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what type of tangles are classical in alzheimers

A

neurofibrillary tangles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

early presentation of alzheimers (3)

A

changes in mood/personality
forgetfulness
anosognosia - lack of insight

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

later presentation of alzheimers

A
sundowning - confusion worse at night 
memory loss - book case analogy 
psychosis, hallucinations 
agnosia -cant recognize self in mirror
decreased mobility
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

first line investigation for alzheimers

other examples

A

MMSE - probs diagnostic

MoCA
ACE-R (adenbrookes)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

what invetsigaion would you want to do to rule out other causes of memory loss if you suspect its not alzheimers (not routinely done)

A

CT/MRI - should see atrophy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

treatment of alzheimers

A

rivastigmine (cholinesterase inhibitor)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

how does rivastigmine work in alzheimers

A

slows down disease process (don’t cure it)

inhibits cholinesterase = boosts ACh which is being broken down faster than normal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

what can you use to treat alzheimers late stage with behavioural disturbance

A

memantine

blocks glutamate (glutamate overactivation in alzheimers causes neuronal damage)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

after alzheimers, what is the second most common type of dementia

A

vascular dementia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

risk factors for vascular dementia (3)

A

hypertension
previous stroke
CVD - smoker, high BMI

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

how is the deterioration in alzheimers

A

smooth deterioration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

how is the deterioration in vascular dementia

what is the onset like

A

step like deterioration

sudden onset

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

how does CVD cause vascular dementia

A

lacunar infarcts = hypoxia to small areas of brain = ischaemia = dementia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

apart from memory loss, what is the most common presentation of vascular dementia

A

personality change - depression

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

investigations for vascular dementia

A

MMSE (MoCA, ACE-R)
SPECT scan - specific for vascular dementia, know this
BP and assess CVD risk factors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

treatment of vascular dementia

A

treat CVD - antihypertensives etc

NOT ???? unlike alzheimers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

what other condition is lewy body dementia associated with

A

parkinsons

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

if someone gets memory problems then movement disorder (= parkinsonism) what is it called

A

lewy body dementia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

if someone gets movement disorder then memory problems (= parkinsonism) what is it called

A

parkinsons dementia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

what part of the brian is degenerated in lewy body dementia and parkinsons

what colour change occurs

A

subtantia nigra

black to pale

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

histology appearance of lewy body dementia

A

lewy bodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

what type of hallucinations are common in lewy body dementia

A

visual hallucinations - children (not threatening)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

does the movement disorder (parkinsons) occur before or after the memory loss associated with lewy body dementia

A

can be either

usually before but can be after

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

what cognitive problem do people with lewy body dementia have

A

fluctuating level of attention

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

how can you differentiate between parkinsons and lewy body dementia

A

DaT scan

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

treatment of lewy body dementia

A

levodopa (same as parkinsons)

NOT antipyschotics

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

which type of dementia usually presents in people <65yo

A

frontotemporal dementia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

another name for frontotemporal dementia

A

picks disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

what other degenerative disease is frontotemporal dementia associated with

A

motor neurone disease (MND)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

which lobe is more significantly affected (by atrophy) in frontotemporal dementia

A

more frontal lobe

also temporal lobe

45
Q

histology of frontotemporal lobe dementia

do these always occur, when they do occur, what is it called

A

picks cells

don’t always occur
when they do occur = picks disease

46
Q

early presentation of frontotemporal lobe dementia (3)

A

frontal lobe problems

personality change eg loss of empathy
weird behavior
compulsive behavior - cant just have 1 biscuit

47
Q

later presentation of frontotemporal lobe dementia (3)

A

brocas aphasia (in frontal lobe)
memory loss
reduced attention

48
Q

investigations for frontotemporal lobe dementia
what do you see

what don’t you bother doing bc isn’t diganostic

A

MRI/SPECT - see atrophy of frontal/temporal lobes

don’t do MMSE - not a classic feature, not diagnostic

49
Q

treatment for fronttemporal dementia

A

nothing really, juts symptomatic

antipyschotics for behavior

50
Q

most common cause of pseudo dementia (dementia but no changes to brain)

A

depression

51
Q
older person 
acute change in cognition 
temporary amnesia (memory loss) 
lasts 4-6 hours 
triggered by emotion/temp
A

transient global amnesia

52
Q

transient lobal amnesia but recurrent

temporal lobe seizures

A

transient epileptic amnesia

53
Q

transient epileptic amnesia treatment

A

sodium valproate

54
Q

common cause of functional cognitive disorder (memory loss eg forgetting keys)

A

mood disorder

55
Q

pathophysiology of mad cow disease (Creutzfeldt-Jakob disease (CJD))

A

abnormal proteins (prions) = neurodegeneration

56
Q

presentation of mad cow disease (Creutzfeldt-Jakob disease (CJD))

A

memory loss (from neurodegeneration)

57
Q

are males or females more likely to get parkinsons

A

males

58
Q

pathophysiology of parkinsons

A

degermation of substantia nigra

59
Q

where in the brain is substantia nigra

A

basal ganglia

60
Q

what colour is substantia nigra normally

what colour is substantia nigra in parkinsons

A

black (nigra = black)

pale

61
Q

whattype of cells are lost from substantia nigra in parkinsons

A

dopaminergic cell loss

62
Q

which pathway (direct or indirect) is suppressed in Parkinson’s

what does this cause

A

direct pathway is suppressed

patient wants to be able to do something, but physically cant (direct pathway isn’t functioning)

63
Q

is parkinsons usually symmetrical or worse on one side

A

worse on one side

64
Q

what type of tremor in parkinsons (2)

when does it occur

A

resting tremor - eg when walking, vanished when hands move

rollingpin tremor - thumbs moves like a rolling pin

65
Q

why do parkinsons patients have a ‘masked’ facial expression

A

bradykinesia - decreased muscle power = facial muscles weak

66
Q

what is the tone like in parkinsons

give an example

A

rigidity - hypertonia

eg cogwheel rigidity in hands

67
Q

what is the handwriting like in parkinsons

A

small - hypographia

68
Q

what is gait like in Parkinson’s

A

shuffling gait

69
Q

what sensory function is often lost in parkinosns

A

smell - anosmia

70
Q

what sleep problems occur in parkinsons

A

REM sleep behaviors - talking in sleep, sitting up in bed etc

71
Q

what psychiatric condition often occurs in parkinsons

A

depression

72
Q

what type of dementia is associated with parkisons

A

lewy body dementia

73
Q

diagnosis of parkinsons

A

based on history and examination

74
Q

investigations for parkinsons if not convinced on diagnosis (2)

A

levodopa responsiveness challenge (do symptoms improve with levodopa)
brain imaging - to exclude other causes

75
Q

first line treatment for parkinsons

A

levodopa

76
Q

side effect of levodopa

how do you treat

A

nausea vomiting

give domperidone (is a dopamine antagonist but doesn’t cross BBB, others do = would make parkinsons worse)

77
Q

how does levodopa work

A

dopamine agonist - acts as a dopamine building block = increases dopamine conc

78
Q

what alterative treatment can be given in parkinsons before levodopa if disease is early stage

A

dopamine agonist eg ropinirole

79
Q

what is it a sign of if levodopa doesn’t work in parkinsons

A

late stage parkinsons
Or
parkinsonism caused by vascular problem

80
Q

why is dietician crucial in treating parkinsons patient

A
likely to be malnourished 
increased movement (form tremor) = increased expenditure
also memory loss = likely to forget meals
81
Q

what GI problem is common in parkinsons

A

constipation

82
Q

what genetic condition presents similar to parkinsons

A

fragile X tremor ataxia syndrome (FXTAS)

83
Q

what is the inheritance pattern of huntingtons

A

autosomal dominant

84
Q

chance of your child getting huntingtons if you have it

A

50% bc autosomal dominant inheritance

85
Q

what genetic sequence causes huntingtons

A

run of CAG (glutamine) >32 in a row

86
Q

which part of the basal ganglia is affected in huntingtons

A

caudate nucleus

87
Q

which pathway (indirect or direct) is affected by neurodegeneration in huntingtons

A

indirect pathway

88
Q

what age of presentation is common in huntingtons

A

35-50

89
Q

classic presentation of huntingtons (3)

A

emotional disturbance - depression, anxiety etc
cognitive problems
motor disturbance = chorea - writhing/dance like movements

90
Q

investigations for huntingtons

A

genetic testing

MRI

91
Q

treatment of huntingtons

A

nothing

symptomatic;
mood stabilisers
radiotherapy for chorea

92
Q

where is the neurodegeneration in motor neurone disease (MND)

A

in upper and lower motor neurons (UMN and LMN)

93
Q

is there sensory symptoms in MND

A

no

just motor

94
Q

how do you differentiate between MS and MND

A

MS has motor and sensory problems

MND has motor symptoms only

95
Q

LMN symptoms in MND

A

muscle wasting/weakness

absent reflexes

96
Q

is there eye involvement in MND

A

no

97
Q

UMN symptoms in MND

A

positive babinskis sign (hyperreflexia)

increased tone

98
Q

what type of dementia is associated with MND

which type of MND specifically

A

frontotemporal lobe dementia

amyotrophic lateral sclerosis

99
Q

which type of MND presents with UMn features only

good or bad prognosis

A

primary lateral sclerosis

good prognosis (>5y)

100
Q

which type of MND presents with UMN and LMN features

good or bad prognosis

A

amyotrophic lateral sclerosis

bad prognosis (3-5y survival)

101
Q

is amyotrophic lateral sclerosis or primary lateral sclerosis more common type of MND

A

amyotrophic lateral sclerosis is more common (85%)

102
Q

is there curative treatment for MND

A

no just symptomatic

103
Q

tremor treatment (2)

A

beta blocker

primidone

104
Q

resting tremor

A

parkinsons/parkinsonism

105
Q

intention tremor (occurs during movement)

A

cerebellar disease (MS or stroke), not parkinsons

106
Q

treatment of tics

A

clonidine

107
Q

what is a brief irregular purposeless movement that flits and flows from one body part to another

how might they disguise it

what does it occur in

A

chorea

disguise it by flicking hair+, sorting glasses etc

huntingtons
parkinsons
wilsons
loads of things!

108
Q

eosinophilic inclusions

A

= lewy bodies

so lewy body dementia or parkinsons