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CP3 Medicine > NEURO - MND/Dementia/Neuropathy/Myopathy > Flashcards

NEURO - MND/Dementia/Neuropathy/Myopathy Flashcards

1
Q

What is motor neurone disease?

A

Degenerative disease of the UMN/LMN of SC/CN + cortex

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2
Q

Which aspect is NOT involved in motor neurone disease?

A

Sensory

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3
Q

What are the 4 types of MND

A

ALS - Amlyotrophic lateral sclerosis
Progressive muscular atrophy
Primary lateral sclerosis
Bulbar

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4
Q

Which is the most common type of MND?

A

ALS

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5
Q

What parts of the nervous system are affected in ALS

A

Loss of Spinal and brainstem LMN

Loss of cortical UMN

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6
Q

Sx ALS (3)

A

LMN weakness - starting in hands + progressing to arms/legs
UMN spastic weakness - starts in legs –> arms
+/- Bulbar/pseudobulbar palsy

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7
Q

O/E ALS (4)

A

Mm wasting
Fasciculations
Brisk reflexes
Increased plantars

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8
Q

Features progressive muscular atrophy MND (3)

A

Loss = RESTRICTED TO SPINAL LMN
Hence
LMN signs only
wasting + fasciculations

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9
Q

Features - primary lateral sclerosis MND (2)

A

Loss RESTRICTED TO CORTICAL UMN

Hence only UMN signs

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10
Q

Features - bulbar MND

A

Bulbar Sx w/ PRESERVATION LIMB FCT

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11
Q

Why is the prognosis for bulbar MND so poor?

A

Early respiratory involvement

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12
Q

Causes of death MND (2)

A

Pneumonia

Ventilatory failure

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13
Q

Dementia - criteria for diagnosis

A

Impairment memory in 1+ of:
Language impairment
Apraxia (motor)
Agnosis (can’t interpret senses)

+
Impairment of fct
Present for at least 6 months
No other medical/physical causes

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14
Q

Modifiable RF dementia (6)

A 
Smoking 
Atherosclerosis 
Alcohol
Cholesterol 
Obesity
Low education
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15
Q

Non-modibiable RF dementia (3)

A

Genetics - APP/PSEN 1/2
AGE
MCI

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16
Q

macroscopic changes - Alzheimers (2)

A

Atrophy of the hippocampus

Enlarged ventricles

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17
Q

Microscopic changes ALzheimers (2)

A

Decr NT function

Abnormal protein depositon –> tangles + plaques

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18
Q

Which 3 genes have been associated with early onset alzheimers

A

APP
PSEN-1
PSEN-2

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19
Q

Which gene has been associated with late onset Alzheimers

A

APOE

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20
Q

Subtypes of vascular dementia (3)

A

Multi-infarct
Subcortical vascular dementia
Post stroke

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21
Q

What is multi-infarct vascular dementia

A

Cortical vascular dementia from small strokes in the cortex

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22
Q

What is subcortical vascular dementia

A

Small vessel deep damage

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23
Q

RF vascular dementia (4)

A

Smoking
High fat diet
Diabetes
HTN

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24
Q

LB Dementia pathology

A

Lewy bodies build up in the cortex and basal ganglia -> disruption NT function

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25
Q

Sx LB dementia (3)

A

Fluctuations in cognition
Visual hallucinations
Parkinsonism

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26
Q

Difference between Parkinsons dementia and LB dementia

A

LBD - cognitive dysfunction within 2 years of motor Sx

PDD - presents later

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27
Q

What % of frontotemporal dementia is though to be inherited

A

50%

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28
Q

Frontotemporal dementia Sx

A

Changes in behaviour emotion and language

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29
Q

MCI

A

Impairment of memory more than that of expected age but not enough to affect function

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30
Q

Does having MCI put you at greater risk of developing dementia?

A

Yes

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31
Q

What is dysphasia

A

Cant find words

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32
Q

What is agnosia

A

Difficulty recognising faces

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33
Q

Early Dementia Sx (3)

A

Memory loss
Slow to grasp new ideas
Errors of judgement

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34
Q

Mild-Mod dementia Sx (3)

A

Difficulty w/ daily tasks
Decline in language
Behaviour change

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35
Q

End stage dementia Sx (4)

A

Increased need of care
COmmunication impaired
Incontinence
Swallowing difficulty

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36
Q

Ix dementia

A 
FBC
B12/folate 
ESR
LFT
U+E
TFT
Glucose 
Lipids
ECG
CT = 1st line 
2nd line = MRI or HMPaO-SPECT
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37
Q

Drugs for Tx alzheimers dementia

A

ACEase inhibitors - rivastigmine

NDMA antagoists - memantine

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38
Q

Can you Tx vascular dementia w/ alzheimers meds

A

No

39
Q

Use of antipsychotics in dementia

A

FOr behaviour and psychological Sx

40
Q

Radiculopathy

A

Process affecting nn roots

41
Q

What is a neuropathy

A

Peripheral nn affected

42
Q

What is mononeuritis multiplex

A

Many single nn affected

43
Q

4 most common causes of neuropathies (4)

A

DM
Carcinomatos neuropathy
Vit B defic
Drugs

44
Q

Other caused neuropathies

A 
Folate/thiamine deficiency 
Uraemia in CKD
Alcohol/Pb 
RA/CT or myxoedema 
G-B, CIPD
Syphillis/ HIV (infective)
Vascular 
Neoplasm
45
Q

Drugs causing neuropathies (3)

A

Amiodarone
Statins
Hydralazine

46
Q

Inherited neuropathies (2)

A

Charcot Marie Tooth

Freidrich’s ataxia

47
Q

Charcot Marie tooth

A

Before 20
W/ progresive neuropathy
Typically affecting hands + feet 1st

48
Q

Features of Freidrich’s ataxia

A

Cerebellar ataxia in the 1st decade
UMN limb signs
Peripehral neuropathy

49
Q

How are demyelinating vs axonal neuropathies differentiated?

A

Nn conduction studies

50
Q

Demyelinating neuropathie

A

Damages spare axons
Affects swann cells
–> decreased conduction velocity

51
Q

E.g. demyelinating neuropathy

A

GBS

Common in immune-mediatedidsease

52
Q

Prognosis demyelinating neuropathy

A

Schwann cells can regrow hence improve w/ Tx

53
Q

Axonal neuropathy

A

.Nn cell bodsies untable to maintain long axonal processes
Degenerating starts in the periphery ==> neuronal cells
Leading to decrease amplitude of impulses

54
Q

Prognosis of axonal neuropathy

A

Axons cant regrow

Hence poor outcome

55
Q

Wallerian degeneration

A

Aftect nerve section or microinfarction

56
Q

Compression neuropathy

A

Leads to focal demyelination

57
Q

Nn infiltration

A

Malignant or granulomatous infilatration

58
Q

Ix neuropathies (7_

A 
FBC
U+E
LFT
Glucose
B12/folate
Autoantibodies
Nn covnduction studies
LP - incr GBS/CIDP 
Peripheral nn biopsy
59
Q

What is the most common acute polyneuropathy

A

GBS

60
Q

Is GBS demyelinating or axonal

A

Demyelinating

61
Q

How long after 1’ infection does GBS occur

A

3 ish weeks

62
Q

PS GBS (4)

A

Ascending paralysis
Loss of reflexes
Affecting lower limbs
Progresses over several days to weeks

63
Q

Complications of GBS (3)

A

VTE
BP irregularities
Arrhythmias

64
Q

Mx GBS (5)

A 
Admit to ITU/HDU
Monitor FVC reg
Ventilatory support 
SC heparin + TEDS
High dose IVIG within 2w
65
Q

What % GBS make a complete recovery over months

A

90%

66
Q

Mortality rate in acute phase GBS

A

10%

67
Q

What is shingles

A

Reactivation of VXV within dosral root ganglion

68
Q

Which dermatomes are most commonly affected by shingles

A

Low thoracic

69
Q

What is Ramsay Hunt syndrome

A

infection of geniculate ganglion –? facial palsy, facial/ear pain + vesicules in ear canal/pinna + soft palate

70
Q

Mx Shingles

A

Aciclovir 5-7days

Paracetamol/amitriptylline - pain

71
Q

What is post-herpetic neuralgia

A

Pain in a previous shingles zone

72
Q

Mx post-herpetic neuralgia

A

Amitriptylline

73
Q

What is myasthenia gravis

A

Disorder of neuromuscular transmission resulting from autoantibodies to components of the NM junction

74
Q

Which receptor is most commonly affected in myasthenia gravis?

A

Acetylcholine receptor

75
Q

PS myasthenia gravis (6)

A

Mm weakness + FATIGABILITY - gets worse throughout day
Fluctuating proximal weakness (> upper limb)
Symmetrical diplopia + ptosis

Dysphagia + speech difficulties
Wasting + resp difficulties after many years

76
Q

What is preserved in myasthenia gravis

A

Tendon reflexes

Heart also not affected

77
Q

What are the 3 types of muscle disease

A

Muscular dystrophies
Myopathies
Neurogenic mm diseases

78
Q

DMD genetics

A

X linked recessive

mutation in dystriphin gene - mm breaks down

79
Q

Features DMD (3)

A

Global weakness
Calf pseudohypertrophy
Gowers sign

80
Q

Ix DMD

A

CK increased

81
Q

What is Becker Muscular dystrophy

A

Milder + better prog version of DMD

82
Q

Gene issue Becker Muscular dystrophy

A

Partially functioning dystrophin

83
Q

Prognosis Becker Muscular dystrophy

A

40s

84
Q

Myotonic dystrophy genetics

A

AD

85
Q

What is the issue with in Myotonic dystrophy

A

Cl channelopathy

86
Q

PS Myotonic dystrophy

A

Mm weakness

Myotonia - can’t relax

87
Q

Ix mm disease

A

CK
Electromyography
Mm biopsy

88
Q

Spinothalamic tract - test (2)

A

Pain w/ neurotip

Temp - cool and warm object

89
Q

Dorsal column - test (3)

A

Vibration - tuning fork 128hz
Joint proprioception - joint movement
Light touch - cotton wool

90
Q

Pyramidal pathway - anatomy

A

Pathways from cerebral cortex to mm

91
Q

Physiological movement - pyramidal vs extrapyramidal

A 
pyramidal = voluntary
extrapyramidal = involuntary
92
Q

Pathological movements in pyramidal pathology (4)

A

Paralysis
Hyperreflexia
Spasticity
Paresis

93
Q

Pathological movements in extrapyramidal pathology (5)

A 
Chorea
Dystonia
Myoclonus 
Tremor 
Akathisia

CP3 Medicine (29 decks)

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