Neuromuscular Disease Flashcards

1
Q

what does neuromuscular disease affect?

A

the peripheral nervous syste,

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2
Q

what specific area of the peripheral nervous system is affected by neuromuscular disease?

A

lower motor neurones that innervate skeletal muscle

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3
Q

what is the effect of neuromuscular disease on muscle function?

A

causes a reduction in ability to function properly

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4
Q

where along the neuromuscular pathway can be affected by neuromuscular disease?

A

anywhere along neuromuscular pathway

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5
Q

how do patients with neuromuscular disease present?

A

weakness and lethargy

muscles are unable to contract properly

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6
Q

describe what happens at the neuromuscular junction

A

depolarisation of end of neuron at synapse causes opening of Ca2+ channels
Ca2+ fuses with vesicles containing Ach and these then fuse with the pre-synaptic membrane releasing neurotransmitter into the synaptic cleft
Ach crosses the synapse and binds to Na+ channels causing them to open and the post snaptic cell depolarises

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7
Q

what sort of disease is polyradiculoneuritis?

A

immune mediated disease

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8
Q

what does polyradiculoneuritis affect?

A

myelin and/or axons

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9
Q

what is the name for diseases which affect myelin or axons?

A

axonopathy

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10
Q

what is the most common peripheral neuropathy in dogs?

A

polyradiculoneuritis

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11
Q

what is the presentation of a patient with polyradiculoneuritis?

A

short-strided gait that progresses to tetraparesis
ambulatory or non-ambulatory
dysphonia
autonomic function remains

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12
Q

are clinical signs of polyradiculoneuritis the same with all patients?

A

no, they vary depending on patient and severity

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13
Q

what function remains in polyradiculoneuritis patients?

A

autonomic (BAR, DUDE is normal)

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14
Q

what is tetraparesis?

A

all 4 limbs become weak and the patient is unable to walk properly / at all

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15
Q

what is dysphonia?

A

change in voice (bark)

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16
Q

when does recovery from polyradiculoneuritis occur?

A

1-4 months after signs stabilise

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17
Q

how is polyradiculoneuritis diagnosed?

A

accurate patient history
physical and neurological exam
electrodiagnostic testing
muscle and nerve biopsies

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18
Q

what tests are used in electrodiagnostic testing?

A

EMG - electromylogram

NCV - nerve conduction velocity

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19
Q

how is polyradiculoneuritis treated?

A

intensive nursing care and physiotherapy

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20
Q

is medical treatment for polyradiculoneuritis effective?

A

medical treatment with corticosteroids shown to have little to no effect

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21
Q

what is myasthenia gravis a disease of?

A

neuromuscular transmission which affects the neuromuscular junction

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22
Q

what is the name of diseases which affect the neuromuscular junction?

A

junctionopathy

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23
Q

what are the 2 ways myasthenia gravis may be aquired?

A

congenital

aquired

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24
Q

what is congenital myasthenia gravis caused by?

A

reduced number of Ach receptors

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25
Q

what is acquired myasthenia gravis caused by?

A

immune mediated

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26
Q

what happens during aquired myasthenia gravis to produce symptoms?

A

antibodies act on Ach receptor and alter it’s function by preventing or changing the binding of Ach to receptors so there are fewer receptors which are effective and so muscles cannot contract normally

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27
Q

how do myasthenia gravis patients present?

A

muscle weakness or fatigue which is usually more obvious when the patient is exercising
regurgitation commonly seen

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28
Q

what are the 3 possible types of myasthenia gravis?

A

focal
generalise
acute fulminating

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29
Q

what type of myasthenia gravis is most severe?

A

acute fulminating

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30
Q

what is focal myasthenia gravis?

A

affects one muscle group only

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31
Q

what is generalised myasthenia gravis?

A

muscles all over the body affected

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32
Q

why is regurgitation commonly seen in myasthenia gravis patients?

A

due to oesophageal dilation/weakness

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33
Q

what is typical behaviour while walking of a patient with myasthenia gravis?

A

will walk in a slow/stilted manner for a short period and then need to stop to rest

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34
Q

how is myasthenia gravis diagnosed?

A

presumptive based on history and presentation
thoracic radiographs to show megaoesophagus
edrophonium / tensilon test

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35
Q

are electrodiagnostics usually used to test for myasthenia gravis?

A

not usually as they are not specific - will be able to rule out some other diseases but not pinpoint myasthenia gravis

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36
Q

what is a edrophonium / tensilon test?

A

injection of IVdrug
wait for a few seconds and then walk animal forwards
if + the animals will be able to walk normally where they couldn’t before

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37
Q

what is a positive result in edrophonium / tensilon tests?

A

animal is able to walk forward normally after IV injection

38
Q

how is myasthenia gravis treated?

A

anticholinesterase therapy
corticosteroids at immunosuppressive doses alongside
intensive nursing care and support

39
Q

how do anticholinesterases help patients with myasthenia gravis?

A

act like tensilon and so increase the Ach available and time spent in synaptic cleft to improve attachment of Ach to receptors and allow normal muscle function

40
Q

what are the side effects of anticholinesterase therapy?

A

GI signs, excessive salivation, lacrimation

41
Q

what condition are myasthenia gravis patients at great risk of?

A

aspiration pneumonia

42
Q

why are myasthenia gravis patients at risk of aspiration pneumonia?

A

increase in muscle weakness increases likelihood of reflux

43
Q

what is polymyositis?

A

immune mediated inflammatory myopathy

44
Q

what causes polymyositis?

A

infiltration of inflammatory cells into the skeletal muscle so muscle doesn’t function as well

45
Q

what is polymyositis caused by?

A

idiopathic but can be associated with systemic disease

46
Q

what are the 2 types of polymyositis?

A

focal

diffuse

47
Q

what is focal polymyositis?

A

one muscle group affected

48
Q

what is diffuse polymyositis?

A

large muscles or lots of muscle groups affected

49
Q

how do polymyositis patients present?

A
exercise intolerance and stiffened gait as the muscles cannot work as normal
muscle weakness
muscle atrophy
dysphonia
dysphagia
regurgitation
50
Q

are signs of polymyositis consistent in the beginning of the disease?

A

no - often wax and wane

51
Q

how is polymyositis diagnosed?

A
criteria not well defined
diagnosis of exclusion as there are no specific tests
clinical history
biochemistry
electrodiagnostic testing
muscle biopsy
52
Q

what biochemisty parameter is likely to be elevated in polymyositis patients?

A

creatinine kinase - found during muscle breakdown

53
Q

how is polymyositis treated?

A

corticosteroids at immunosuppressive doses

intensive nursing care and support are vital

54
Q

what drug may be used alongside steroids to treat polymyositis?

A

Azothrioprine

55
Q

why may Azothrioprine be needed alongside steroids in treatment of polymyositis?

A

if patients cannot tolerate steroid side effects as a lower steroid dose can be given

56
Q

what are the side effects of corticosteroids?

A

polyphagia
polydipsia
oedema
weight gain

57
Q

what is the risk of aspiration pneumonia in polymyositis patients?

A

high due to megaoesophagus

58
Q

what is the pressure sore risk like for polymyositis patients?

A

medium due to loss of muscle coverage

59
Q

what is the contracture risk like in polymyositis patients?

A

low

60
Q

what is the aspiration pneumonia risk like for polyradiculoneuritis patients?

A

low as autonomic function remains

61
Q

what is the pressure sore risk like for polyradiculoneuritis patients?

A

high

62
Q

what is the contracture risk like for polyradiculoneuritis patients?

A

high in the young and medium in adults due to recumbancy

63
Q

what is the aspiration pneumonia risk like for myasthenia gravis patients?

A

high due to mega oesophagus

64
Q

what is the pressure sore risk like for myasthenia gravis patients?

A

low

65
Q

what is the contracture risk like for myasthenia gravis patients?

A

none

66
Q

who is the team involved in the care of the neuromuscular patient?

A

patient
owner
nurses
vet

67
Q

what is aspiration pneumonia?

A

inhalation of GI contents into the lungs usually due to regurgitation

68
Q

what is the result of aspiration on the lungs?

A

pulmonary damage

inflammatory response

69
Q

what are the clinical signs of aspiration pneumonia?

A

coughing
tachypnoea
harsh lung sounds
crackles on auscultation

70
Q

what is the most common complication in patients with myasthenia gravis?

A

aspiration pneumonia

71
Q

how is aspiration pneumonia treated?

A
careful and close monitoring of patients
early administration of antibiotics
IVFT
O2 therapy
supportive care
respiratory physiotherapy if clinician wishes
walking and turning patients regularly
feeding balls of food from height
severe cases may need to go onto mechanical ventilation
72
Q

is respiratory physiotherapy of use in aspiration pneumonia patients?

A

evidence varies - depends on clinician

73
Q

how often should aspiration pneumonia patients be turned?

A

every 4-6 hours

74
Q

what should animals with aspiration pneumonia be fed?

A

no liquid or dry food due to aspiration / irritation risk
balls of soft food
fed at height
keep raised after eating

75
Q

what may severe aspiration pneumonia cases need?

A

mechanical ventilation as unable to oxygenate properly

76
Q

what is a common complication for recumbent patients?

A

pressure sores

77
Q

what does recumbancy lead to?

A

increased pressure over bony prominences which leads to ischemia and necrosis

78
Q

what are the stages of pressure sores?

A

stage 1 - 4

79
Q

describe a stage 1 pressure sore

A

lightly pigmented

skin intact

80
Q

describe a stage 2 pressure sore

A

skin is broken

81
Q

describe a stage 3 pressure sore

A

larger and deeper wound

has broken through fat below the skin

82
Q

describe a stage 4 pressure sore

A

large and deep
through the muscle to bone
necrosis
infection

83
Q

what areas of the animals body are at particular risk of pressure sores?

A
elbows
hips
stifle
carpus
tarusu
84
Q

how can pressure sores be prevented?

A

thick padded bedding
turn every 2-4 hours
donut bandage on areas of concern to reduce pressure
porous bedding
incontinence pads to remove moisture
non-slip floors
physiotherapy
monitor patients closely
checklist to maintain treatment level across shifts
express / catheterise bladder to prevent urine scaulding

85
Q

how are pressure sores treated?

A

prevention better than cure
myasthenia gravis patients are especially prone
bandages and commercial boots may be useful
debride sores if necessary
use antibiotics if infection suspected

86
Q

what is muscle contracture?

A

adaptive shortening of muscle
adaptive shortening of soft tissues
inelasticity of soft tissues

87
Q

what leads to muscle contracture?

A

recumbancy and immobilisation

88
Q

how is muscle contracture treated?

A

intensive physiotherapy

89
Q

what is involved in intensive physiotherapy to treat muscle contracture?

A

massage
PROM - passive range of motion
properoceptive exercises
neuromuscular stimulator

90
Q

what must be considered when treating neuromuscular patients?

A
temperament
client expectations
client limits
disease processes
previous injuries / surgeries
other medical conditions
91
Q

what respiratory techniques may be used on aspiration pneumonia patients?

A

vibration - shaking patients sides

coupage - cupped hands tapping on the chest from caudal to cranial