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J Neuro > NMJ disorders > Flashcards

NMJ disorders Flashcards

1
Q

what are the 4 neuromuscular junction disorders

A

myasthenia gravis
lambert eaton myasthenia syndrome
botulism
neuromyotonia (NMT aka isaacs disease)

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2
Q

which NMJ is most common (still rare tho lol)

A

myasthenia gravis

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3
Q

what type of condition is myasthenia gravis

A

autoimmune

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4
Q

what are the antibodies in myasthenia gravis produced in response to

A

in respone to ACh receptors in the post synaptic NMJs

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5
Q

what symptom does reduced ACh receptors in NMJ cause

A

weakness and fatigue (bc the muscles have less effective channels)

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6
Q

what NMJ disorder is associated with thymoma

A

myasthenia gravis

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7
Q

who gets myasthenia gravis (2 groups of people)

A

30yo females

70yo males

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8
Q

which NMJ disorder presents with ocular involvement, worse as day goes on

A

myasthenia gravis

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9
Q

presentation of myasthenia gravis

A

ocular involvement (diplopia, ptosis), worse as day goes on
weakness of proximal muscles
bilateral facial weakness, cant chew at end of meal
fatigue, worse as day goes on

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10
Q

what are reflexes like in myasthenia gravis

A

normal

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11
Q

do symptoms get better or worse as day goes on in myasthenia gravis

A

worse - more tired

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12
Q

investigations for myasthenia gravis

A

anti-ACh antibodies
anti-muscle specific kinase (MUSK) antibody
CT thorax - for thymoma

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13
Q

treatment of myasthenia gravis

A

anticholinesterase eg pyridostigmine

immunosuppressants - steroid, azathioprine

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14
Q

what type of condition is lambert eaton

A

autoimmune

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15
Q

what are the autoantibodies in response to in lambert eaton

what does this cause

A

Ca+ channels in NMJ = decreased vesicle release = muscle weakness

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16
Q

how does the muscle weakness differ in lambert eaton in comparison to myasthenia gravis

A

myasthenia gravis - worse as day goes on

lambert eaton - better after exercise

17
Q

what cancer is lambert eaton associated wit h

A

small cell lung cnacer

18
Q

treatment of lambert eaton

A

anticholinesterase eg pyridostigmine
K+ channel blockers eg diaminopyridine
IVIg
CXR - for lung SCC

19
Q

causes of botulism

A

toxin - food poisoning, infections,

20
Q

what does the botulinum toxin do to NMJ

A

cleaves pre synaptic proteins = irreversibly inhibits ACh release

21
Q

how does botulism present

A 
paralysis 
difficulty swallowing 
drooping eyelids 
NO sensory loss
rapid change
22
Q

can you treat botulin

A

probs nah, most die

23
Q

what is wrong in neuromyotonia (isaacs syndrome)

A

action potential is longer than normal in NMJ

24
Q

presentation of neuromyotonia (isaacs syndrome)

A

cramps
stiffness
muscle twitches (fasciculations)

25
Q

treatment of neuromyotonia (isaacs syndrome)

A

anti convulsants eg carbamazepine

J Neuro (32 decks)

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