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Neuromuscular Disease Flashcards

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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
what is acquired myasthenia gravis caused by?
immune mediated
26
what happens during aquired myasthenia gravis to produce symptoms?
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
27
how do myasthenia gravis patients present?
muscle weakness or fatigue which is usually more obvious when the patient is exercising regurgitation commonly seen
28
what are the 3 possible types of myasthenia gravis?
focal generalise acute fulminating
29
what type of myasthenia gravis is most severe?
acute fulminating
30
what is focal myasthenia gravis?
affects one muscle group only
31
what is generalised myasthenia gravis?
muscles all over the body affected
32
why is regurgitation commonly seen in myasthenia gravis patients?
due to oesophageal dilation/weakness
33
what is typical behaviour while walking of a patient with myasthenia gravis?
will walk in a slow/stilted manner for a short period and then need to stop to rest
34
how is myasthenia gravis diagnosed?
presumptive based on history and presentation thoracic radiographs to show megaoesophagus edrophonium / tensilon test
35
are electrodiagnostics usually used to test for myasthenia gravis?
not usually as they are not specific - will be able to rule out some other diseases but not pinpoint myasthenia gravis
36
what is a edrophonium / tensilon test?
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
37
what is a positive result in edrophonium / tensilon tests?
animal is able to walk forward normally after IV injection
38
how is myasthenia gravis treated?
anticholinesterase therapy corticosteroids at immunosuppressive doses alongside intensive nursing care and support
39
how do anticholinesterases help patients with myasthenia gravis?
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
what are the side effects of anticholinesterase therapy?
GI signs, excessive salivation, lacrimation
41
what condition are myasthenia gravis patients at great risk of?
aspiration pneumonia
42
why are myasthenia gravis patients at risk of aspiration pneumonia?
increase in muscle weakness increases likelihood of reflux
43
what is polymyositis?
immune mediated inflammatory myopathy
44
what causes polymyositis?
infiltration of inflammatory cells into the skeletal muscle so muscle doesn't function as well
45
what is polymyositis caused by?
idiopathic but can be associated with systemic disease
46
what are the 2 types of polymyositis?
focal | diffuse
47
what is focal polymyositis?
one muscle group affected
48
what is diffuse polymyositis?
large muscles or lots of muscle groups affected
49
how do polymyositis patients present?
``` exercise intolerance and stiffened gait as the muscles cannot work as normal muscle weakness muscle atrophy dysphonia dysphagia regurgitation ```
50
are signs of polymyositis consistent in the beginning of the disease?
no - often wax and wane
51
how is polymyositis diagnosed?
``` criteria not well defined diagnosis of exclusion as there are no specific tests clinical history biochemistry electrodiagnostic testing muscle biopsy ```
52
what biochemisty parameter is likely to be elevated in polymyositis patients?
creatinine kinase - found during muscle breakdown
53
how is polymyositis treated?
corticosteroids at immunosuppressive doses | intensive nursing care and support are vital
54
what drug may be used alongside steroids to treat polymyositis?
Azothrioprine
55
why may Azothrioprine be needed alongside steroids in treatment of polymyositis?
if patients cannot tolerate steroid side effects as a lower steroid dose can be given
56
what are the side effects of corticosteroids?
polyphagia polydipsia oedema weight gain
57
what is the risk of aspiration pneumonia in polymyositis patients?
high due to megaoesophagus
58
what is the pressure sore risk like for polymyositis patients?
medium due to loss of muscle coverage
59
what is the contracture risk like in polymyositis patients?
low
60
what is the aspiration pneumonia risk like for polyradiculoneuritis patients?
low as autonomic function remains
61
what is the pressure sore risk like for polyradiculoneuritis patients?
high
62
what is the contracture risk like for polyradiculoneuritis patients?
high in the young and medium in adults due to recumbancy
63
what is the aspiration pneumonia risk like for myasthenia gravis patients?
high due to mega oesophagus
64
what is the pressure sore risk like for myasthenia gravis patients?
low
65
what is the contracture risk like for myasthenia gravis patients?
none
66
who is the team involved in the care of the neuromuscular patient?
patient owner nurses vet
67
what is aspiration pneumonia?
inhalation of GI contents into the lungs usually due to regurgitation
68
what is the result of aspiration on the lungs?
pulmonary damage | inflammatory response
69
what are the clinical signs of aspiration pneumonia?
coughing tachypnoea harsh lung sounds crackles on auscultation
70
what is the most common complication in patients with myasthenia gravis?
aspiration pneumonia
71
how is aspiration pneumonia treated?
``` 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
is respiratory physiotherapy of use in aspiration pneumonia patients?
evidence varies - depends on clinician
73
how often should aspiration pneumonia patients be turned?
every 4-6 hours
74
what should animals with aspiration pneumonia be fed?
no liquid or dry food due to aspiration / irritation risk balls of soft food fed at height keep raised after eating
75
what may severe aspiration pneumonia cases need?
mechanical ventilation as unable to oxygenate properly
76
what is a common complication for recumbent patients?
pressure sores
77
what does recumbancy lead to?
increased pressure over bony prominences which leads to ischemia and necrosis
78
what are the stages of pressure sores?
stage 1 - 4
79
describe a stage 1 pressure sore
lightly pigmented | skin intact
80
describe a stage 2 pressure sore
skin is broken
81
describe a stage 3 pressure sore
larger and deeper wound | has broken through fat below the skin
82
describe a stage 4 pressure sore
large and deep through the muscle to bone necrosis infection
83
what areas of the animals body are at particular risk of pressure sores?
``` elbows hips stifle carpus tarusu ```
84
how can pressure sores be prevented?
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
how are pressure sores treated?
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
what is muscle contracture?
adaptive shortening of muscle adaptive shortening of soft tissues inelasticity of soft tissues
87
what leads to muscle contracture?
recumbancy and immobilisation
88
how is muscle contracture treated?
intensive physiotherapy
89
what is involved in intensive physiotherapy to treat muscle contracture?
massage PROM - passive range of motion properoceptive exercises neuromuscular stimulator
90
what must be considered when treating neuromuscular patients?
``` temperament client expectations client limits disease processes previous injuries / surgeries other medical conditions ```
91
what respiratory techniques may be used on aspiration pneumonia patients?
vibration - shaking patients sides | coupage - cupped hands tapping on the chest from caudal to cranial

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