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MedEd GKP/AB/VP > Neuro 3 > Flashcards

Neuro 3 Flashcards

1
Q

Types of Dementia

A

Alzheimer’s Dementia
Vascular Dementia
Lewy Body Dementia
Fronto-temporal Dementia (Pick’s disease)

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2
Q

Other Neurological Diseases

A 
Parkinsonism/ Parkinson's Disease
Huntington's Chorea
Motor Neuron Disease
Guillain-Barre Syndrome
Myasthenia Gravis
Neurofibromatosis
Wernicke-Korsakoff Syndrome
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3
Q

Alzheimer’s Dementia - who?

A

Elderly (although early-onset subtype)

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4
Q

Alzheimer’s Dementia - RF

A

Age
Genetics
Down’s Syndrome

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5
Q

Alzheimer’s Dementia - Pathophysiology

A

Beta amyloid plaques - disrupts signalling
Tau neurofibrillary tangles - disrupts microtubules in neurons.
Decrease in cortical ACh

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6
Q

Alzheimer’s Dementia - Onset/Progression

A

Gradual, Progressive

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7
Q

Alzheimer’s Dementia - Presentation

A

5 A’s
+/- Depression
+/- Paranoid delusions

5 A's:
Amnesia
Anomia
Apraxia
Agnosia
Aphasia
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8
Q

5 A’s of Alzheimer’s

A 
Amnesia - remembering
Anomia - naming
Apraxia - doing
Agnosia - recognizing
Aphasia - speaking
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9
Q

Alzheimer’s Dementia - Genetics

Early Onset

A

Younger than 65 yrs old
Presenilin 1
Presenilin 2
APP gene

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10
Q

Alzheimer’s Dementia - Genetics

Late Onset

A

Older than 65 yrs old

Apolipoprotein E4

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11
Q

Which of the following is not classically a feature of Alzheimer’s Disease?

Amnesia
Anomia
Apraxia
Ataxia
Depression
A

Ataxia

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12
Q

Down’s Syndrome is associated with which form of dementia?

Alzheimer's dementia
Pick's disease
Lewy body dementia
Frontotemporal dementia
Vascular dementia
A

Alzheimer’s Dementia

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13
Q

APP

A

Amyloid Precursor Protein

–> helps neurons grow and repair; gets used, broken down and recycled.

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14
Q

Vascular Dementia - Who?

A

Elderly

Vasculopaths

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15
Q

Vascular Dementia - RF

A

M>F
Elderly
CVD risk factors

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16
Q

Vascular Dementia - Pathophysiology

A

Infarction of small and medium sized vessels

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17
Q

Vascular Dementia - Onset/Progression

A

Sudden onset

Stepwise deterioration

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18
Q

Vascular Dementia - Presentation

A

+/- Like Alzheimer’s
Location-specific deficits
Emotional and personality changes
Focal neurology

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19
Q

Pick’s Disease - Who?

A

40-60yrs

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20
Q

Pick’s Disease - RF

A

+/- FHx (although most sporadic)

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21
Q

Pick’s Disease - Pathophysiology

A

Pick Bodies: Hyperphosphorylated tau protein

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22
Q

Pick’s Disease - Onset/Progression

A

Death within 5-10 yrs

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23
Q

Pick’s Disease - Presentation

A 
Personality change
Disinhibition
Emotional blunting
Relative preservation of memory
Overeating, preference for sweet foods

Silver staining Tau

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24
Q

An elderly gentleman is accompanied to the GPs with his daughter. She is distressed that ‘something’s happened to Dad, he’s changed …’. It transpires that he has started swearing at people in the street and flirting with all the women he meets. He scores well on an AMTS.

What is the likely Dx?

Pick’s disease
Lewy body dementia
Vascular dementia
Alzheimer’s dementia
CJD
A

Pick’s Disease

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25
Q

Lewy Body Dementia - Who?

A

Middle age to elderly

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26
Q

Lewy Body Dementia - RF

A

-

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27
Q

Lewy Body Dementia - Pathophysiology

A

Lewy Bodies: Eosinohilic intracytoplasmic neuronal inclusions composed of apha-synuclein and ubiquitin

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28
Q

Lewy Body Dementia - Onset/Progression

A

Fluctuating

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29
Q

Lewy Body Dementia - Presentation

A

Classic Presentation (2+ needed for Dx):

Fluctuating confusion and consciousness
Visual hallucinations (people, animals)
New onset Parkinsonism
\+/- Repeated falls
\+/- Syncope
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30
Q

Parkinsonism classic presentation

A

Classic Presentation:

Bradykinesia
Resting tremor (4-6Hz)
Rigidity (increased tone)
+/- postural instability

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31
Q

Parkinsonism - what else?

A

6 M’s:

Monotonous, hypotonic speech
Micrographia
hypomiMesis (expressionless face)
March a petit pas (classic gait)
Misery (depression)
Memory loss (dementia)
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32
Q

Parkinsonism - Causes?

A

Idiopathic Parkinson’s Disease:
Asymmetric signs of Parkinsonism

Drug induced Parkinson’s:
Bilateral, symmetrical signs

Parkinson’s Plus:
Additional features present

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33
Q

Idiopathic Parkinson’s Disease - Pathophysiology

A

Decreased Dopamine - Degeneration of dopaminergic neurons in the substantia nigra

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34
Q

Parkinsonism - Signs

A

Cogwheel rigidity
(Lead-pipe rigidity and tremor)

March a petit pas
(Gait with little steps - as if chasing one’s centre of gravity)

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35
Q

Which of the following is not classically a feature of idiopathic Parkinson’s disease?

Athetosis
Hypomimia
March a petit pas
Depression
Micrographia
A

Athetosis

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36
Q

Parkinson’s Plus

A

Multiple system atrophy [MSA] - AKA Sky-Dragar Syndrome: Early autonomic features.

Progressive Supranuclear Palsy [PSP] - AKA Steele-Richardson-Olszewski Syndrome: Early postural instability and vertical gaze palsy.

Lewy Body Dementia: Early dementia and visual hallucinations.

Corticobasal ganglionic degeneration [CBD]: Alien limb phenomenon.

Vascular Parkinson’s: Legs particularly affected; Gait worse than tremor.

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37
Q

Huntington’s Chorea - Who?

A

About 40 yrs old - anticipated.

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38
Q

Huntington’s Chorea - Inheritance

A

Autosomal Dominant

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39
Q

Huntington’s Chorea - Genetics

A

HTT gene on Chr. 4

Anticipation: Age of onset links to number of CAG repeats.

40
Q

Huntington’s Chorea - Presentation

A

Movement: Involuntary Movements. Chorea (purposeless, dance-like movements) and Athetosis (writhing movements of hands)

Cognitive: Dementia + Personality Changes

Mood: Depression

FHx: Increasingly early onset throughout generations

41
Q

Huntington’s Chorea - Prognosis

A

No cure
No Rx slows progression
Death within 15 yrs of Dx

42
Q

Huntington’s Chorea - Pathophysiology

A

Repeat Expansion:
Normal HTT gene = 10-35 CAG repeats
HD HTT gene = >36 CAG repeats

HTT –> Glutamine

Theory: Increased glutamine aggregates in caudate and putamen –> neuronal death.

43
Q

Motor Neuron Disease

ALS - Motor Nerves Affected

A

Amyotrophic Lateral Sclerosis
(Lou Gehrig’s Disease)

UMN and LMN

44
Q

Motor Neuron Disease

ALS - Presentation

A

UMN + LMN signs

45
Q

Motor Neuron Disease

Progressive Muscular Atrophy Variant - Motor Nerves Affected

A

LMN only

46
Q

Motor Neuron Disease

Progressive Muscular Atrophy Variant - Presentation

A

LMN signs only - e.g. flail arm/flail foot syndrome

47
Q

Motor Neuron Disease

Progressive bulbar palsy variant - Motor Nerves Affected

A

Cranial Nerves XI - XII

48
Q

Motor Neuron Disease

Progressive bulbar palsy variant - Presentation

A

Tongue: Flaccid, Fasciculating tongue
Jaw: Absent Jaw-Jerk
Voice: Nasal ‘Donald Duck’ Voice

49
Q

Motor Neuron Disease Types

A

Amyotrophic Lateral Sclerosis [ALS] “Lou Gehrig’s”

Progressive Muscular Atrophy Variant

Progressive Bulbar Palsy Variant

Pseudobulbar Palsy

Primary Lateral Sclerosis Variant

50
Q

Motor Neuron Disease

Pseudobulbar Palsy - Motor Nerves Affected

A

UMN lesion of cranial nerves IX - XII

51
Q

Motor Neuron Disease

Pseudobulbar Palsy - Presentation

A

Tongue: Slow movements
Jaw: Brisk Jaw jerk
Voice: ‘Hot Potato’ Speech

52
Q

Motor Neuron Disease

Primary Lateral Sclerosis Variant - Motor Nerves Affected

A

Loss of Betz cells in motor cortex - mainly UMN

53
Q

Motor Neuron Disease

Primary Lateral Sclerosis Variant - Presentation

A

UMN pattern of weakness
Brisk reflexes
Extensor plantar response
No LMN signs

54
Q

Multiple Sclerosis - Definition

A

Multiple lesions separated in time and space

Demyelinating disease of the CNS

55
Q

Multiple Sclerosis - Pathophysiology

A

Type IV hypersensitivity reaction

56
Q

Multiple Sclerosis - Who?

A

Female
20-40
White

57
Q

Multiple Sclerosis - Presentation

A

PC of one neurological problem
PMH of unrelated neurological problem
+/- symptoms triggered/exacerbated by hot bath/shower - triggers demyelination

58
Q

Multiple Sclerosis - O/E

A

Early signs - papillitis

59
Q

Multiple Sclerosis - Types

A

Primary progressive MS - Steady accumulation of disability with no clear relapsing/remitting pattern.

Relapsing-remitting MS - Unpredictable clinical attacks with complete recovery in between attacks.

Progressive-relapsing MS - Steady declines in onset, with attack superimposed.

Secondary progressive MS - Initially relapsing and remitting MS that suddenly begins to have decline without periods of remission.

Relapsing and remitting –> Progressive

60
Q

Papilloedema vs Papillitis

A 
Papilloedema...
Cause: Raised ICP
Appearance on Fundoscopy: Blurred optic disc margin
Vision: Normal
Eye movements: No pain
Bilateral

Papillitis…
Cause: MS (inflammation of optic nerve head)
Appearance on Fundoscopy: Blurred optic disc margin
Vision: Blurred
Eye movements: Pain on movement
Unilateral

61
Q

Guillain-Barre Syndrome - RF

A

Recent URTI or Gastroenteritis (Campylobacter jejuni)

62
Q

Guillain-Barre Syndrome - Pathophysiology

A

Autoimmune

63
Q

Guillain-Barre Syndrome - Classic Presentation

A

Sudden onset
Ascending symmetrical muscle weakness and decreased sensation
LL > UL
Distal muscles > Proximal muscles

64
Q

Guillain-Barre Syndrome - O/E

A

Flaccid paralysis

Absent reflexes

65
Q

Guillain-Barre Syndrome - Complications

A

Respiratory failure (1/4)

66
Q

Guillain-Barre Syndrome - Miller-Fisher Variant

A

(rare)
Opthalmoplegia
Ataxia
Arreflexia

67
Q

Myasthenia Gravis - Antibody

A

AChR

68
Q

Myasthenia Gravis - Key feature

A

Muscles fatigue with use

69
Q

Myasthenia Gravis - Presentation

A

Drooping eyelids, slurred speech, swallowing difficulties

+/- SOB

70
Q

Myasthenia Gravis - Reflexes

A

Normal

71
Q

Myasthenia Gravis - Association

A 
Thymic hyperplasia (70%)
Thyoma (10%)
72
Q

Neurofibromatosis Type 1 - Inheritance

A

Autosomal Dominant

73
Q

Neurofibromatosis Type 1 - Gene

A

NF1

Chr.17

74
Q

Neurofibromatosis Type 1 - Commoner?

A

Commoner

75
Q

Neurofibromatosis Type 1 - Presentation

A 
Cafe-au-lait spots
Freckling in skin folds (axilla, groin, neck base, under breast)
Neurofibromas
Lisch nodules
Spinal scoliosis
76
Q

Neurofibromatosis Type 1 - Other features

A

Short stature

Mild learning difficulty

77
Q

Cafe au lait spots Dx

A

Pre-pubertal: > 5 spots > 5mm each

Post-pubertal: > 5 sports > 15mm each

78
Q

Neurofibromatosis Type 2 - Inheritance

A

Autosomal Dominant

79
Q

Neurofibromatosis Type 2 - Gene

A

NF2

Chr.22

80
Q

Neurofibromatosis Type 2 - Commoner?

A

Rarer

81
Q

Neurofibromatosis Type 2 - Presentation

A

Sensorineural hearing loss: Bilateral vestibular Schwannomas (acoustic neruomas), becoming symptomatic by age 20

82
Q

Neurofibromatosis Type 2 - Other features

A

No or fewer Cafe au lait spots than NF1
+/- Tinnitus
+/- Vertigo

83
Q

Which of the following is not classically a feature of idiopathic neurofibromatosis type 1?

Cafe au lait spots
Neurofibromas
Auditory neuromas
Lisch nodules
Skin fold freckling
A

Auditory neuromas

84
Q

Wernicke-Korsakoff Syndrome - Types

A

Wernicke’s encephalopathy

Korsakoff’s psychosis

85
Q

Wernicke’s Encephalopathy - Cause

A

Thiamine Deficiency

86
Q

Wernicke’s Encephalopathy - Who?

A

Alcoholics !
Malnourished
Elderly

87
Q

Wernicke’s Encephalopathy - Acute/Chronic?

A

Acute

88
Q

Wernicke’s Encephalopathy - Reversible?

A

Yes

89
Q

Wernicke’s Encephalopathy - Presentation

A

Classic Presentation: ACE
Ataxia
Confusion
Eye signs: opthalmoplegia nystagmus

90
Q

Korsakoff’s Psychosis - Cause

A

Thiamine deficiency

91
Q

Korsakoff’s Psychosis - Who?

A

Alcoholics !
Malnourished
Elderly

92
Q

Korsakoff’s Psychosis - Acute/Chronic?

A

Chronic

93
Q

Korsakoff’s Psychosis - Reversible?

A

No (…?)

94
Q

Korsakoff’s Psychosis - Presentation

A

Memory Loss

Confabulation

95
Q

You are called to see a 40yo man in A&E. You try to take a Hx but the man in confused and unable to tell you much. O/E he has a cerebellar ataxia and nystagmus.

What is the likely Dx
Multiple sclerosis
Head trauma
Korsakoff’s
Wernicke’s
Motor neuron disease
A

Wernicke’s

MedEd GKP/AB/VP (28 decks)

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