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Y2 Neurology > Y4 Myopathy > Flashcards

Y4 Myopathy Flashcards

1
Q

what are features of myopathy?

A

Gradual onset
􏰀 Symmetrical, proximal weakness: difficulty combing
hair, climbing stairs, getting up from chairs

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2
Q

what are the reflexes of myopathy?

A

preserved - normal

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3
Q

what is the difference in locations affected between myopathy and neuropathy ?

A

neuropathy - distal

myopathy - proximal

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4
Q

what two conditions have fatiguablity ?

A

MG, LEMS

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5
Q

what type of myopathy would be indicated by pain on exercise ?

A

ischaemia or metabolic myopathy

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6
Q

what type of myopathy would be indicated by pain at rest ?

A

inflammyopathy

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7
Q

what investigations should be done for myopathies?

A

ESR, CK, AST, LDH

􏰀 EMG

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8
Q

state two inflammatory myopathies ?

A

Polymyositis

􏰀 Dermatomyositis

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9
Q

what metabolic conditions can cause myopathies ?

A

Hyperthyroidism, Cushing’s, ↑/↓Ca2+

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10
Q

what medication can cause myopathies ?

A

steroids, statins, EtOH

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11
Q

state the two common types of muscular dystrophies ?

A

Duchennes

Beckers

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12
Q

what is the genetic inheritance of muscular dystrophies ?

A

X linked

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13
Q

what age does duchennes present ?

A

4years

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14
Q

what are the features of duchennes?

A

Difficulty standing
􏰁 Calf pseudohypertrophy 􏰁
Respiratory failure

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15
Q

what one investigation apart from genetics can confirm duchennes?

A

↑↑CK

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16
Q

is duchennes or beckers more severe?

A

duchennes

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17
Q

what muscular dystrophies has Difficulty puffing cheeks and raising arms above head?

A

Facioscapulohumeral MD

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18
Q

what is the genetic inheritance of Facioscapulohumeral MD ?

A

Autosomal dominant

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19
Q

what are three signs of Facioscapulohumeral MD ?

A

Weakness of face, shoulders and upper arms
(often asymmetric ̄c deltoids spared)
􏰁 Winging of scapula
􏰁 Foot drop.

20
Q

what is the cause of Myotonic Dystrophy?

A

Cl- channelopathy

21
Q

what does the face look like with myotonic dystrophy ?

A

Myopathic facies: long, thin, expressionless 􏰁
Wasting of facial muscles and SCM
􏰁 Bilateral ptosis
􏰁 Dysarthria: myotonia of tongue and pharynx

22
Q

what is myotonia ?

A

Myotonia: slow relaxation

23
Q

what do the hands look like in myotonic dystrophy?

A

Myotonia: slow relaxation
􏰂 E.g. inability to release hand after shake
􏰁 Wasting and weakness of distal muscles + areflexia → wrist drop
􏰁 Percussion myotonia: percuss thenar eminence → involuntary thumb flexion

24
Q

state some other features of myotonic dystrophy?

A 
􏰁 Frontal balding
􏰁 Cataracts
􏰁 DM
􏰁 Cardiomyopathy, tachy- / brad-arrhythmias
􏰁 Dysphagia
􏰁 Testicular atrophy
25
Q

what medication can improve myotonia during myotonic dystrophy?

A

phenytoin

26
Q

what is the pathophysiology of myasthenia gravis ?

A

Autoimmune disease mediated by Abs vs. nicotinic Ach receptors.

27
Q

what are the reflexes like with myasthenia gravis?

A

normal

28
Q

state the presenattion of myasthenia gravis ?

  • extra ocular
  • bulbar
  • face
  • neck
  • limb
A

Extra-ocular: bilateral ptosis, diplopia
􏰁 Bulbar: voice deteriorates on counting to 50
􏰁 Face: myasthenic snarl on attempting to smile 􏰁
Neck: head droop
􏰁 Limb: asymmetric, prox. weakness

29
Q

what two abs are present in myasthenia gravis ?

A

Anti-AChR Abs

MuSK abs

30
Q

what investigations should be done for myasthenia gravis ?

A 
Tensilon Test
􏰁 Give edrophonium IV
􏰁 +ve if power improves w/i 1min
􏰀 Anti-AChR Abs
􏰀 EMG
31
Q

what gender is myasthenia gravis associated with <50 and >50yrs ?

A 
<50 = women
>50 = males
32
Q

what condition is myasthenia gravis associated with <50yrs ?

A

thymic hyperplasia.

33
Q

what condition is myasthenia gravis associated with >50yrs ?

A

thyme tumour/atrophy

34
Q

what medication can be given for myasthenia gravis?

A

Anticholinesterase: e.g. pyridostigmine.

35
Q

what is the management of myasthenia gravis ?

A 
steroids 
anti cholinesterase 
immunosuppression (methotrexate) 
IV Ig
plasma exchange (plasmapheresis)
thymectomy
36
Q

what is a Myasthenic Crisis?

A

Weakness of respiratory muscles during relapse may be lethal.

37
Q

how is a myasthenic crisis monitored ?

A

Monitor FVC: vent support if <20ml/kg

38
Q

what does LEMS stand for

A

Lambert-Eaton Myasthenic Syndrome

39
Q

what is the pathophysiology of LEMS ?

A

Abs to VGCC ↓ influx of Ca2+ during presynaptic excitation → ↓ presynaptic ACh-vesicle fusion.

therefore less ACh being released and binding to receptors

40
Q

what does VGCC stand for in LEMS ?

A

voltage-gated calcium channels

41
Q

what are two common causes of LEMS ?

A

Paraneoplastic: e.g SCLC 􏰀

Autoimmune

42
Q

what abs are present in LEMS ?

A

anti VGCC abs

43
Q

what is the presentation of LEMS ?

A 
same as myasthenia gravis +
􏰁
Leg weakness early (before eyes) 􏰁 
Extra: Autonomic and areflexia
􏰁Movement improves symptoms
􏰁Small response to edrophonium
44
Q

what treatment is available for LEMS?

A

3,4-diaminopyridine or IVIg

45
Q

how does botulism toxin work?

A

prevents ACh vesicle release

46
Q

what is the presentation of botulism ?

A

Descending flaccid paralysis ̄c no sensory signs

47
Q

what is the treatment for botulism ?

A

benpen + antiserum

Y2 Neurology (52 decks)

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