3. Neuromuscular Junction Disorders Flashcards

1
Q

Botulism- Presynaptic!!!!

A

Found in improperly preserved or canned foods & contaminated wounds
Neurotoxin produced by clostridium botulinum
Anaeobic, gram positive rod

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2
Q

Classification/ aquistion

A
Food bourne (HONEY)
Wound
Unclassified
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3
Q

Mechanism

A

Botulinum toxin enters presynaptic terminals
Blocks the fusion of ACh vesicles with PRESYNAPTIC membrane (myoneural junction)
Inhibit ACh release into neuromuscular junction
Nerve impulse fails to transmit across the neuromuscular junction
Muscle paralysis

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4
Q

Botulism- Timeframe

A

12-36 hrs after ingestion of contaminated food

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5
Q

Botulism Signs and Symptoms

A

Flaccid symmetrical paralysis
Blurred & double vision, photophobia, ptosis(drooping of eyelid)
Dry mouth, nausea, & vomiting
Lethargy
Difficulty in swallowing (dysphagia) & speech (dysarthria)

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6
Q

Botulism- sensory

A

NO SENSORY INVOLVEMENT

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7
Q

Prevention

A

Boiling food 10 min
Avoid honey for children <1
Wound care and sterile technique

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8
Q

Botulism Recovery

A

sprouting of new terminal nerve filaments and formation of new synapses

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9
Q

BoTox as a treatment for Spasticity

A

SCI
MS
CVA
TBI

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10
Q

BoTox Mechanism

A

Prevent the release of acetylcholine from the PRE-SYNAPTIC nerve terminal, thus blocking peripheral cholinergic transmission at the neuromuscular junction

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11
Q

BoTox active time

A

3-4 months

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12
Q

BoTox taking effect

A

Taken up at NMJ within 12 hr

Effect ovvurs over 4-7 days

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13
Q

Plan of care after botox

A

ACTIVE STRETCHING- Reciprocal inhibition
Strengthening of opposite muscles
Dynasplint or some sort of passive stretching can help
Don’t have a lot of time before wears off

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14
Q

Post injection

A

PT MANDATORY
Splinting
Dont overstretch
Increase strength of antagonist

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15
Q

BoTox Side effects

A

Swallowing
Speaking
Breathing
Drooping eyelids

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16
Q

Myasthenia gravis- POSTSYNAPTIC

A

large muscle weakness

Post synaptic disease at NMJ

17
Q

Myasthenia gravis Mechanism

A

Widened synaptic cleft
Loss of folds – muscle endplate membrane
Reduction in number and density of ACh receptors
Results in weakness or paresis
*****ACH neurotransmitter is less likely to find a receptor before it is hydrolyzed by ACHesterase

18
Q

Mysathenia gravis drug treatment

A

target the breakdown of neurotransmitter to slow it down providing more time to find a receptor

19
Q

Myasthenia gravis- THYMUS

A

acquired autoimmune
viral infection
hyperplasia of thymus (70)
tumors of thymus (10)

20
Q

Myasthenia gravis prevalence

A

BIMODAL
Female peak young (15-30)
Male peak (60-75)
Greater in females

21
Q

Myasthenia gravis disease progression

PROXIMAL TO DISTAL

A

maximal weakness within a year in 2/3 cases

after 15-20 years, weakness is fixed

22
Q

myasthenia gravis remission

A

25%

23
Q

classifications of myasthenia gravis

A

Ocular (10-15)

Generalized (85)

24
Q

Myasthenic crisis

A

respiratory failure

25
Q

Byasthenia gravis Signs and Symptoms

A
Ptosis
Diploplia
facial weakness (CNVII)
Oropharyngal weakness
Swalling difficulties
26
Q

Therapy for myasthenia gravis

A

Schedule therapy in A.M. to get the most out of therapy, want to see patients in P.M. to understand how severe their condition gets
Less aggressive physical activity toward the end of the treatment or HEP instruction, do harder activities first when they’re in peak
PRIMARILY MUSCULAR DISEASE (neuromuscular junction), rarely have sensory dysfunction
Have patients swallow with chin tucked to avoid aspiration
Don’t speak with food in mouth
Monitor tidal volume, vital capacity, and inspiratory force during PT

27
Q

Myasthenia gravis weakness and impportant info

A

BUE or BLE (proximal > distal)

Respiratory muscles

Better in A.M., declines as the day progresses (or declines during exercise)

28
Q

Myasthenia gravis diagnosis

A

presence of circulating antibodies to ACH receptors
Muscle biopsy to count ACH receptors
Decremental response to repeated stim

29
Q

Myasthenia gravis diagnosis

TENSILON TEST``

A

Give tensilon
Compare performanc on/off/placebo
IF STRENGTH/ENDURANCE IMPROVE ON THEN ITS MG

30
Q

Tensilon MOI

A

Mainly diagnostic

hydrolyzes ACh so it stays in the synapse longer

31
Q

Myasthenia gravis intervention drug treatment

A
Anticholinesterae
Immunosuppressives
IVIG
Plasmaphoresis
Thymectomy- adults