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Neuro Pathology > Degenerative Disease of the CNS > Flashcards

Degenerative Disease of the CNS Flashcards

1
Q

Definition of dementia

A

Progressive impairment of multiple domains of cognitive function in an alert patient leading to loss of acquired skills and interference in occupational and social role

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2
Q

Parkinsonism is a clinical syndrome with >2 of;

A

Bradykinesia
Rigidity
Tremor
Postural instability

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3
Q

Definition of bradykinesia

A

Slowness of movement

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4
Q

Where is the pathology in parkinsons?

A

Basal ganglia

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5
Q

What is predominately lost is the basal ganglia in parkinsons?

A

Dopamine

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6
Q

What is the 2nd most common neurodegenerative disease?

A

Parkinsons

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7
Q

Types of late onset (65 +) dementia

A

Alzheimer’s (55%)
Vascular (20%)
Lewy body (20%)
Others (5%)

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8
Q

Types of young onset (<65 years old) dementia

A 
Alzheimers (33%)
Vascular (15%)
Frontotemporal (15%)
Other (33%)
- toxic (alcohol) 
- genetic (huntingtons)
- infection (HIV, CJD)
- inflammatory (MS)
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9
Q

Treatment causes of dementia

A

Vitamin deficiency - B12
Thyroid disease
HIV
Syphillis

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10
Q

What conditions mimic dementia?

A

Hydrocephalus
Tumour
Depression (pseudodementia)

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11
Q

Types of parkinsonism

A 
Idiopathic parkinsons disease (IPD)
Lewy body dementia (LBD)
Drug induced (e.g. dopamine antagonists)
Vascular parkinsons (lower half)
Parkinsons plus syndromes
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12
Q

What are some parkinsons plus syndromes?

A

Multiple system atrophy
Progressive supranuclear palsy
Corticobasal degeneration

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13
Q

What is examined when looking at cognitive function?

A 
Memory 
Attention 
Language 
Visuospatial 
Behaviour
Emotion 
Executive function 
Apraxia 
Agnosias
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14
Q

What is ataxia?

A

A motor disorder in which the individual has difficulty with the motor planning to perform tasks or movements when asked, provided that the request or command is understand and he/she is willing to perform the task

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15
Q

Definition of agnosia

A

Inability to process sensory information

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16
Q

What screening tests are done for cognitive function?

A 
Mini mental (MMSE)
Montreal (MOCA)
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17
Q

What type of dementia has a stepwise progression?

A

Vascular

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18
Q

What would dementia with abnormal movements indicate?

A

Huntington’s

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19
Q

What type of dementia also has parkinsonism?

A

Lewy body dementia

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20
Q

What type of dementia comes with myoclonus?

A

CJD

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21
Q

How do you get a definitive diagnosis of parkinsonism?

A

Post morteom

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22
Q

Presentation of IPD

A 
BRADYKINESIA 
\+ At least one of the following 
-  tremor
- rigidity
- postural instability
Slowly progressive (>5-10 years)
Asymmetric rest tremor
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23
Q

Does parkinsons have a good response to dopamine replacement treatment?

A

Yes

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24
Q

Treatment of PD

A

Dopamine replacement treatment

  • Levodopa (L-dopa) = CO-CARLEDOPA OR CO-BENELDOPA
  • dopamine agonists = ROPIRINOLE OR PRAMIPEXOLE OR ROTIGOTINE PATCH
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25
Q

Treatment of Alzheimer’s (+/- Lewy body dementia)

A 
Cholinesterase inhibitors (cholinergic deficit) 
- donepezil
- rivastigimine 
- galantamine
NMDA antagonist 
- memantine
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26
Q

Treatment for frontotemporal dementia

A

None

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27
Q

Complications for parkinsonism

A

Drug induced
- motor fluctuations - levodopa wears off
- dyskinesias - involuntary movements (levodopa)
- psychiatric - hallucinations, impulse control
Non drug induced especially non motor
- depression
- dementia
- autonomic; BP, bladder, bowel
- speech, swallow
- balance

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28
Q

Late treatment of parkinsonism

A 
Prolong levodopa half life 
- MAO-B inhibitors
- COMT inhibitor
- Slow release levodopas 
Add oral dopamine agonist 
Continous infusion 
- apomorphine 
- duodopa
Functional neurosurgery (Deep brain stimulation)
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29
Q

What is the commonest neurodegenerative condition?

A

Alzheimer’s disease

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30
Q

Mean onset age of Alzheimer’s?

A

70 y/o

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31
Q

Presentation of temporo-pareital dementia

A

Early memory disturbance
Language and visuospatial problems
Personality preserved until later

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32
Q

Presentation of frontotemporal dementia

A

Early change in personality/behaviour
Often change in eating habits
Early dysphagia
Memory/visuospatial relatively preserved

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33
Q

Presentation of vascular dementia

A

Mixed picture

Stepwise decline

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34
Q

What are the degenerative causes of parkinsonism?

A

IPD
LBD
Parkinsons plus syndromes

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35
Q

What are the 2ndry causes of parkinsonism?

A

Vascular

Drug induced

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36
Q

What is IPD responsive to?

A

Levodopa

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37
Q

Exclusion criteria for PD

A

History of
- repeated strokes with stepwise progression of parkinsonism features
- repeated head injury
- definite encephalitis
Oculogyric crises
Neuroleptic treatment at onset of symptoms
More than one affected relative
Sustained remission
Strictly unilateral features after 3 years
Supranuclear gaze palsy
Cerebellar signs
Early severe autonomic involvement
Early severe dementia with disturbance of memory, language and praxis
Babinskis sign
Prescence of cerebral tumour or communicating hydrocephalus on imaging study
Negative response to large doses of levodopa in absence of malabsorption
MPTP exposure

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38
Q

Supportive prospective criteria for PD

A 
Unilateral onset
Rest tremor present 
Progressive 
Persistent asymmetry affecting side of onset most
Excellent response (70-100%) to levodopa
Severe levodopa induced chorea
Levodopa response for 5 years or more
Clinical course of 10 years or more
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39
Q

Pathological changes of IPD

A

Deposits of Lewy bodies
Loss of pigmented dopaminergic neurones in brainstem nuclei
Alpha synuclein changes thought key
Over time changes spread - involving more of brainstem, then cortex etc

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40
Q

What is the most common cause of parkinsonism?

A

IPD

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41
Q

What cause of parkinsonism is more likely to have cognitive problems than parkinsonism?

A

LBD

42
Q

What presymptomatic changes can people get in PD before they get their symptoms?

A

Anosmia

43
Q

What can be used to assess bradykinesia?

A

Finger taps

44
Q

What can indicate rigidity?

A

Loss of arm swing whilst walking

45
Q

What type of signs to start off with indicate IPD?

A

Unilateral

46
Q

Assessment of parkinsonism

A

Finger tap

Full neurological assessment

47
Q

What happens to a finger tap in PD?

A

Do for 20 seconds, gets slower as time goes on and gets smaller
Worse on DOMINANT SIDE

48
Q

Extra indications of PD

A

Loss of facial expression

Micrographia

49
Q

What is micrographia?

A

Small and tremulous writing

50
Q

Diagnosis of PD

A

Clinical

51
Q

What must also be asked about in PD?

A

Non motor symptoms

52
Q

What are the non motor symptoms that may occur in PD?

A 
Cognition 
Cramps
Restless legs
Constipation 
Speech 
Mood changes
53
Q

When is a Dat-SPECT scan used?

A

To differentiate essential tremor from parkinsonian tremor

54
Q

When is Dat-SPECT scan +ve?

A

Degenerative parkinsonian conditions, but DOES NOT tell you which one

55
Q

Drug interactions of Dat-SPECT that decreases the binding

A 
Cocaine
Amphetamines
Methylphenidate 
Ephedrine 
Fentanyl
56
Q

Drug interactions of Dat-SPECT that increase the binding

A

SSRIs

57
Q

Why give levodopa in PD?

A

Replaces the chemical

58
Q

Why give dopamine agonists in PD?

A

Boots any chemical left over in the brain

59
Q

What are some other Parkinson therapies?

A

Apomorphine

Duodopa

60
Q

What is apomorphine used to treat?

A

Disabling motor fluctuations - if off spells

61
Q

How is apomorphine given?

A

SC infusion with pump or pen injection

62
Q

What is depression thought to be in PD?

A

2ndry to the disease

63
Q

Why may someone with PD need a dietician?

A

Due to loss of calories due to constant trembling

64
Q

What is duodopa?

A

Gel for continuous administration into the duodenum or upper jejunum via a pump and tube

65
Q

Triggers of freezing of gait

A

Different terrain
Doorways
Busy / loud environment

66
Q

When does freezing of gait occur in PD?

A

Advanced disease

67
Q

What is common in freezing of gait?

A

On and off spells

68
Q

What make freezing of gait better?

A

Cueing techniques

  • visual
  • rhythmic
69
Q

A slow shuffling turn then improves with what?

A

Walking

70
Q

Features of essential tremor

A 
FH 
Head can be involved 
Action tremor
May have had for many years 
Mobility issues/falls/constipation/speech/swallow should NOT be affected
71
Q

What may help an essential tremor?

A

Alcohol

72
Q

What is commonly used to treat an essential tremor?

A

Propanolol

73
Q

What is drug induced parkinsonism difficult to differentiate from?

A

IPD

74
Q

Can you still get drug induced parkinsonism if off the meds?

A

Yes, still may have symptoms for months after

75
Q

DAT SPECT Scan result in drug induced parkinsonism

A

Negative

76
Q

Can you fully recover after drug induced parkinsonism if stop meds?

A

Will not progress any further but will not fully recover

77
Q

Top 3 drugs causing drug induced PD

A

Metoclopradmide (anti emetic)
Prochlorperazine (anti emetic)
Haloperidol (antipsychotic)

78
Q

Causes of drug induced PD

A 
Metoclopramide (anti emetic)
Procholrperazine (anti emetic)
Haloperidol 
Antipsychotics 
Lithium 
Amoidarone 
Valproate
Cinnarizine
79
Q

Features of vascular parkinsonism

A 
Legs > arms
Stepwise progression 
Sudden onset 
Gait affected EARLY 
Cognitive impairment EARLY
80
Q

Levodopa response in vascular parkinsonism

A

Poor

81
Q

2 predominant types of multiple system atrophy

A
  1. MSA-P = predominant Parkinson features

2. MSA-C = predominant cerebellar features

82
Q

Features of multiple system atrophy

A 
Parkinsonism 
Autonomic disturbance 
- erectile dysfunction (usually early feature)
- postural hypotension 
- atonic bladder
Cerebellar signs
83
Q

What is the antiemetic choice in PD?

A

Domperidone

84
Q

Features of PD tremor

A

Unilateral

Improves with voluntary movements

85
Q

Parkinsonism with autonomic disturbance points towards what diagnosis?

A

Multiple system atrophy

86
Q

What can parkinsons lead to and why?

A

Postural hypotension

Due to autonomic failure

87
Q

What neurodegenerative condition is associated with MND?

A

Frontotemporal dementia

88
Q

As well as hands and fingers, what can an essential tremor also affect?

A

Vocal cords

89
Q

Presentation of progressive supranuclear palsy

A 
Impairment of vertical gaze 
- down gaze worse than up gaze 
- may complain of difficulty reading or climbing stairs
Parkinsonism 
Falls
Slurring of speech 
Cognitive impairment
90
Q

Which treatment of parkinsons has been linked to impulse control disorders?

A

Dopamine receptor agonists

91
Q

Treatment of vomiting caused by radio / chemotherapy

A

Ondansteron

92
Q

Treatment of vomiting caused by intracranial causes e.g. raised ICP, direct effect of a tumour

A

Haloperidol

93
Q

Treatment of vomiting caused by vestibular causes

A

Prochloperazine

94
Q

Treatment of vomiting caused by GI problems

A

Metoclopramide

95
Q

What drugs can cause neuroleptic malignant syndrome?

A

Antipsychotics

If dopaminergic drugs are stopped or have their dose reduced

96
Q

When does neuroleptic malignant syndrome start?

A

Within hours to days of starting an antipsychotic

97
Q

Presentation of neuroleptic malignant syndrome

A 
Pyrexia
Muscle rigidity 
Autonomic lability 
- HTN
- tachycardia
- tachypnoea
Agitated delirium with confusion
98
Q

What is often raised in neuroleptic malignant syndrome?

A

Creatinine kinase

99
Q

What may develop in neuroleptic malignant syndrome secondary to raised creatinine kinase?

A

AKI due to rhabdomyolysis

100
Q

Treatment of neuroleptic malignant syndrome

A

Stop drug
IV fluids to prevent renal failure
Dantrolene
Bromocriptine (dopamine agonist)

101
Q

What psychiatric condition is the most common in PD patients and what % of them have it?

A

40%

102
Q

Describe ataxic gait

A

A wide based gait with loss of heel to toe walking

Neuro Pathology (21 decks)

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