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MedEd GKP/AB/VP > Neuro 3 > Flashcards

Neuro 3 Flashcards

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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
Lewy Body Dementia - Who?
Middle age to elderly
26
Lewy Body Dementia - RF
-
27
Lewy Body Dementia - Pathophysiology
Lewy Bodies: Eosinohilic intracytoplasmic neuronal inclusions composed of apha-synuclein and ubiquitin
28
Lewy Body Dementia - Onset/Progression
Fluctuating
29
Lewy Body Dementia - Presentation
Classic Presentation (2+ needed for Dx): ``` Fluctuating confusion and consciousness Visual hallucinations (people, animals) New onset Parkinsonism +/- Repeated falls +/- Syncope ```
30
Parkinsonism classic presentation
Classic Presentation: Bradykinesia Resting tremor (4-6Hz) Rigidity (increased tone) +/- postural instability
31
Parkinsonism - what else?
6 M's: ``` Monotonous, hypotonic speech Micrographia hypomiMesis (expressionless face) March a petit pas (classic gait) Misery (depression) Memory loss (dementia) ```
32
Parkinsonism - Causes?
Idiopathic Parkinson's Disease: Asymmetric signs of Parkinsonism Drug induced Parkinson's: Bilateral, symmetrical signs Parkinson's Plus: Additional features present
33
Idiopathic Parkinson's Disease - Pathophysiology
Decreased Dopamine - Degeneration of dopaminergic neurons in the substantia nigra
34
Parkinsonism - Signs
Cogwheel rigidity (Lead-pipe rigidity and tremor) March a petit pas (Gait with little steps - as if chasing one's centre of gravity)
35
Which of the following is not classically a feature of idiopathic Parkinson's disease? ``` Athetosis Hypomimia March a petit pas Depression Micrographia ```
Athetosis
36
Parkinson's Plus
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.
37
Huntington's Chorea - Who?
About 40 yrs old - anticipated.
38
Huntington's Chorea - Inheritance
Autosomal Dominant
39
Huntington's Chorea - Genetics
HTT gene on Chr. 4 | Anticipation: Age of onset links to number of CAG repeats.
40
Huntington's Chorea - Presentation
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
Huntington's Chorea - Prognosis
No cure No Rx slows progression Death within 15 yrs of Dx
42
Huntington's Chorea - Pathophysiology
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
Motor Neuron Disease | ALS - Motor Nerves Affected
Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease) UMN and LMN
44
Motor Neuron Disease | ALS - Presentation
UMN + LMN signs
45
Motor Neuron Disease | Progressive Muscular Atrophy Variant - Motor Nerves Affected
LMN only
46
Motor Neuron Disease | Progressive Muscular Atrophy Variant - Presentation
LMN signs only - e.g. flail arm/flail foot syndrome
47
Motor Neuron Disease | Progressive bulbar palsy variant - Motor Nerves Affected
Cranial Nerves XI - XII
48
Motor Neuron Disease | Progressive bulbar palsy variant - Presentation
Tongue: Flaccid, Fasciculating tongue Jaw: Absent Jaw-Jerk Voice: Nasal 'Donald Duck' Voice
49
Motor Neuron Disease Types
Amyotrophic Lateral Sclerosis [ALS] "Lou Gehrig's" Progressive Muscular Atrophy Variant Progressive Bulbar Palsy Variant Pseudobulbar Palsy Primary Lateral Sclerosis Variant
50
Motor Neuron Disease | Pseudobulbar Palsy - Motor Nerves Affected
UMN lesion of cranial nerves IX - XII
51
Motor Neuron Disease | Pseudobulbar Palsy - Presentation
Tongue: Slow movements Jaw: Brisk Jaw jerk Voice: 'Hot Potato' Speech
52
Motor Neuron Disease | Primary Lateral Sclerosis Variant - Motor Nerves Affected
Loss of Betz cells in motor cortex - mainly UMN
53
Motor Neuron Disease | Primary Lateral Sclerosis Variant - Presentation
UMN pattern of weakness Brisk reflexes Extensor plantar response No LMN signs
54
Multiple Sclerosis - Definition
Multiple lesions separated in time and space | Demyelinating disease of the CNS
55
Multiple Sclerosis - Pathophysiology
Type IV hypersensitivity reaction
56
Multiple Sclerosis - Who?
Female 20-40 White
57
Multiple Sclerosis - Presentation
PC of one neurological problem PMH of unrelated neurological problem +/- symptoms triggered/exacerbated by hot bath/shower - triggers demyelination
58
Multiple Sclerosis - O/E
Early signs - papillitis
59
Multiple Sclerosis - Types
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
Papilloedema vs Papillitis
``` 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
Guillain-Barre Syndrome - RF
Recent URTI or Gastroenteritis (Campylobacter jejuni)
62
Guillain-Barre Syndrome - Pathophysiology
Autoimmune
63
Guillain-Barre Syndrome - Classic Presentation
Sudden onset Ascending symmetrical muscle weakness and decreased sensation LL > UL Distal muscles > Proximal muscles
64
Guillain-Barre Syndrome - O/E
Flaccid paralysis | Absent reflexes
65
Guillain-Barre Syndrome - Complications
Respiratory failure (1/4)
66
Guillain-Barre Syndrome - Miller-Fisher Variant
(rare) Opthalmoplegia Ataxia Arreflexia
67
Myasthenia Gravis - Antibody
AChR
68
Myasthenia Gravis - Key feature
Muscles fatigue with use
69
Myasthenia Gravis - Presentation
Drooping eyelids, slurred speech, swallowing difficulties | +/- SOB
70
Myasthenia Gravis - Reflexes
Normal
71
Myasthenia Gravis - Association
``` Thymic hyperplasia (70%) Thyoma (10%) ```
72
Neurofibromatosis Type 1 - Inheritance
Autosomal Dominant
73
Neurofibromatosis Type 1 - Gene
NF1 | Chr.17
74
Neurofibromatosis Type 1 - Commoner?
Commoner
75
Neurofibromatosis Type 1 - Presentation
``` Cafe-au-lait spots Freckling in skin folds (axilla, groin, neck base, under breast) Neurofibromas Lisch nodules Spinal scoliosis ```
76
Neurofibromatosis Type 1 - Other features
Short stature | Mild learning difficulty
77
Cafe au lait spots Dx
Pre-pubertal: > 5 spots > 5mm each | Post-pubertal: > 5 sports > 15mm each
78
Neurofibromatosis Type 2 - Inheritance
Autosomal Dominant
79
Neurofibromatosis Type 2 - Gene
NF2 | Chr.22
80
Neurofibromatosis Type 2 - Commoner?
Rarer
81
Neurofibromatosis Type 2 - Presentation
Sensorineural hearing loss: Bilateral vestibular Schwannomas (acoustic neruomas), becoming symptomatic by age 20
82
Neurofibromatosis Type 2 - Other features
No or fewer Cafe au lait spots than NF1 +/- Tinnitus +/- Vertigo
83
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 ```
Auditory neuromas
84
Wernicke-Korsakoff Syndrome - Types
Wernicke's encephalopathy | Korsakoff's psychosis
85
Wernicke's Encephalopathy - Cause
Thiamine Deficiency
86
Wernicke's Encephalopathy - Who?
Alcoholics ! Malnourished Elderly
87
Wernicke's Encephalopathy - Acute/Chronic?
Acute
88
Wernicke's Encephalopathy - Reversible?
Yes
89
Wernicke's Encephalopathy - Presentation
Classic Presentation: ACE Ataxia Confusion Eye signs: opthalmoplegia nystagmus
90
Korsakoff's Psychosis - Cause
Thiamine deficiency
91
Korsakoff's Psychosis - Who?
Alcoholics ! Malnourished Elderly
92
Korsakoff's Psychosis - Acute/Chronic?
Chronic
93
Korsakoff's Psychosis - Reversible?
No (...?)
94
Korsakoff's Psychosis - Presentation
Memory Loss | Confabulation
95
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 ```
Wernicke's

MedEd GKP/AB/VP (28 decks)

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