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Binocular Vision > Myogenic Palsies - Miriam > Flashcards

Myogenic Palsies - Miriam Flashcards

1
Q

What is myasthenia Gravis?

A

This is an autoimmune condition, Life threatening disease in certain cases

It is when the muscles are very weak.

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2
Q

Adult MG?

A

Ocular Myasthenia: Limited to the eye. If still only eye muscles then unlikely to become generalized.

Mild to moderate : Often slow onset of symptoms eyes usually first.
Acute fulminating : RAPID ONSET - AFFECTS RESPIRATORY MUSCLES
Late severe : develops after 2 years of initial muscle weakness

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3
Q

What are the three paediatric groups of children with MG?

A

1) Neonatal
2) Congenital
3) Juvenile

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4
Q

What does neonatal mean?

A

Born to a myasthenic mother, self limits in 6 weeks

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5
Q

What does congenital MG mean?

A

Infants born to non-myasthenic mothers

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6
Q

What does juvenile MG mean?

A

Develops in infancy, spontaneous remission

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7
Q

What are four types of adult MG?

A

1) Ocular myasthenia
2) Mild to moderate
3) Acute fulminating
4) Late severe

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8
Q

What is ocular MG?

A

Limited to eye muscles only

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9
Q

If a px has ocular MG for 2 years, is it likely for it go on to other muscles of the body and become generalised?

A

No

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10
Q

What is mild to moderate mg?

A

Generalised Slow onset of sx + respiratory muscles not affected

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11
Q

What is acute fulminating mg?

A

Generalised, rapid onset, affects respiratory muscles- THEY CAN DIE

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12
Q

What is late severe mg?

A

Generalised, start with mild or ocular but after 2 years they start to develop severe form

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13
Q

What is drug-related MG?

A

MG is induced by D-pencillamine

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14
Q

What is D-pencillamine used to treat?

A

Rheumatoid arthritis and systemic lupus

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15
Q

why is anisha so bent

A

becasue she wa sborn that way

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16
Q

Why does muscles fatigue in MG?

A
  • antibodies prevent ACh binding at synaptic gap= reduction of ACh uptake
  • more ACh produce so muscle contraction can occur
  • body runs out of ACh= muscles tired
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17
Q

Which muscle types are affected ?

A

Striated muscles

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18
Q

What are some general sx an mg px may experience?

A

General fatigue and lack of energy

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19
Q

What are some limb related sx a mg px may experience?

A

Difficulty getting out a chair or climbing downstairs

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20
Q

What are some facial issues a px with mg may experience ?

A

Lack of facial expression

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21
Q

What other sx may they complain of?

A

Problems with speech, breathing and swallowing

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22
Q

What two ocular symptoms marks the onset of 70% of mg cases?

A

Ptosis and diplopia

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23
Q

Is Ptosis often bilateral or unilateral?

A

Bilateral but asymmetric

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24
Q

What happens to the ptosis with increase of fatigue?

A

Ptosis gets worse

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25
Q

What is often seen in an attempt to elevate an mg eye?

A

Frontalis o/a

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26
Q

To try to lift the ptotic eye, what may happen to the contralateral eye?

A

Lid retraction

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27
Q

If you detect ptosis, what is a big question you must ask in Relation to mg?

A

Does it get worse throughout the day or when you do exercise

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28
Q

What can you do in practice to investigate if ptosis is related to mg?

A

Muscle fatigue-
Ask the px to elevate their eyes and look at a pen torch for a minute, measure the ptosis before and after to see if it increases in size

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29
Q

What do you do to see if a px has Cogans lid twitch when trying to investigate ptosis?

A
  • Depress eyes for 15 seconds by looking at a pen torch and return eyes to pp
  • Lid initially moves above ptotic level and the comes back down

E.g the eye lid overshoots

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30
Q

What do you do to see if a px has Gorelick’s sign when investigating Ptosis?

A
  • Hold up more ptotic lid
  • The ptosis on the less effects side starts to increase
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31
Q

What is the ice pack test when investigating ptosis

A

An ice pack place on both lids for 2 mins and if px ptosis improves = strong indication of MG

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32
Q

Will a px with MG have vertical or horizontal dipl?

A

It varies

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33
Q

What question is good to ask a px who is complaining of dipl and you may suspect mg?

A

Does the dipl increase throughout thr day or with exercise

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34
Q

If using a Hess chart what should you write on it ?

A

Date but also the time performed

35
Q

What may you find if a px has mg and you have done multiple Hess charts ?

A

Different palsies and weaknesses on each time done as different muscles are more weak on different occasions

36
Q

How can you check for obicularis weakness (when you scrunch up your eyes)?

A

Cannot squeeze their eyes tight enough for eyelashes to be buried + when a clinician tries to open their eyes, it only requires gentle pressure

37
Q

What tests should you do with a suspect MG px?

A
  • Ocular motility
  • PCT
  • Convergence
  • PFR
  • Hess chart
38
Q

What are some good questions to ask a suspect MG px?

A
  • Do sx increase with fatigue?
  • Do they get worse after exercise?

(Remember early stages may be intermittent weaknes)

39
Q

How can MG be diagnosed for general MG?

A

With a blood test which detects those antibodies that prevent bind of Acetylcholine
*this isn’t amazing for those px who only have ocular MG

40
Q

How can mg be detected by looking at an mg px’s thymus?

A

Large thymus + tumours in the thymus which can be seen with a CT or MRI scan

41
Q

What is the Tensilon test ?

A

Test used to detect MG by:
- Being injected with anticholinesterase
- Then watch fatigue muscles recover
- Lasts 5 mins
-May want to do an EMG before and after

42
Q

What is bad about Tensilon test?

A

Adverse reactions such as cardiac arrhythmias

43
Q

What is the sleep test?

A

Ask the px to rest for 30 mins and see of ptosis improves

44
Q

What is strongly associated with TED?

A

MG

45
Q

Name the 5 tests to detect MG (not optom related)

A

1) Blood test
2) tensilon test
3) Enlarged thymus on MRI/CT scan
4) Sleep test
5) Noting ocular fatigue when carrying out tests such as OM

46
Q

What 4 systemic treatments of MG?

A
  • Pyridostigmine
  • Immunisuppresive systemic steroids
  • Plasmaphoresis
  • Thymectomy
47
Q

What is Pyridostigmine?

A

Longer acting anticholinesterase

48
Q

What does Plasmaphoresis do?

A

“Clean the blood“ which Reduces level of circulating antibody or circulating immunoglobulin

49
Q

What are three ocular treatments for mg?

A
  • Ptosis prop (glasses or a cls)
  • occlusion - for dipl
  • Surgery
50
Q

What are three occlusive ocular treatments for mg?

A

Blenderm (surgical tape) on glasses
Frosted lenses
Occlusive CLs

51
Q

Why is surgery contradicted for mg pxs?

A

Only allowed to be carried out if stable for a long term

52
Q

What is Lambert-Eaton Rooke syndrome? (LERS)

A

Issue is presynaptic, proximal limb weakness

53
Q

What is the key difference between MG and LERS?

A

MG is an issue POST-synaptic and LERS is an issue PRE-synaptic

54
Q

What do you have an increased risk of if you have LERS?

A

Small cell carcinoma (this is a cancer) - risk reduces after having it after 2 years

55
Q

What are 4 clinical characteristics of LERS ?

A
  • Limb weakness
  • Resting action potential of muscles reduced
  • Muscle’s action potential increases after repetitive stimulation
  • dry mouth
56
Q

What are some ocular characteristics of LERS?

A

50% have ptosis
EOM involvement unusual
Response to Tensilon unpredictable

57
Q

What are the three managements of LERS?

A

1) Exclude malignancy (small cell carcinoma)
2) Ptosis props
3) Guanidine hydrochloride drops

58
Q

What is guanidine used to treat ?

A

Muscle weaknesses

59
Q

What does CPEO stand for ?

A

Chronic progressive external ophthalmoplegia

60
Q

What are 6 characteristics of CPEO?

A
  • Progressive symmetrical loss of OM
  • Progressive symmetrical bilateral ptosis
  • Loss of Bell’s reflex
  • May not complain of dipl
  • During early stages due to the asymmetry dipl may be complained of
  • Orbicualris weakness
61
Q

Here is a girl with CPEO, you can see her bilateral ptosis getting progressively worse

A
62
Q

Why are pupil responses not affected by CPEO?

A

Only striated muscles are affected and pupillary response are conducted by smooth muscle

63
Q

What describes the term of end stage of CPEO?

A

“FROZEN EYE”- they have very little eye movement

64
Q

What is the aetiology of CPEO?

A

Mitochondrial myopathy

65
Q

What syndrome is associated with CPEO?

A

Kearne-Sayre-Daron syndrome

66
Q

What are some features of Kearne-Sayre-Daron syndrome?

A
  • Onset before 20 years old
  • Fine pigmentary retinopathy
    -Heart conduction block
67
Q

Is CPEO the most common mitochondrial myopathy ?

A

Yes

68
Q

What is the management of CPEO if they complain of diplopia?

A

Prisms
Occlusion (cls, blenderm, frosted glasses)
Surgery

69
Q

Why would you do surgery for CPEO?

A

If the eyes are grossly fixated eccentrically

70
Q

What is orbital myositis?

A

Inflammation of one or more of the EOM

71
Q

Is orbital myositis normally uni or bi lateral?

A

Unilateral

72
Q

What are 6 signs and symptoms someone with orbital myositis may have?

A

1) Proptosis
2) Pain
3) Lid oedema
4) Photophobia
5) Conjunctivitis
6) limitation of EOM

*remember this is because the muscles are very inflamed

73
Q

What is associated with orbital myositis ?

A

Autoimmune conditions

74
Q

What are 5 DD of orbital myositis?

A

Orbital cellulitis
Orbital abscess
Hematoma
Graves’ disease TED
Tumours

75
Q

What is a hematoma ?

A

an area of blood that collects outside of the larger blood vessels- like a bad bruise
(Priyesh gives hematomas)

76
Q

What instrument is used to measure proptosis?

A

Exophthalmometer - measures by how much the eye is protruding, measure from the side

77
Q

What does this CT scan show?

A

Px with orbital myositis (look at those inflamed EOM + proptosis)

78
Q

How is orbital myositis managed?

A

Systemic steroids but can be self limiting

79
Q

What is rhabdomyosarcoma?

A

Highly malignant tumour of the striated muscles

80
Q

Does rhabdomyosarcoma Normally occur in children or adults?

A

Children - in their first decade of life I

81
Q

Are rhabdomyosarcomas more prevalent in boys or girls ?

A

Boys

82
Q

As there is a VERY rapid growth of this tumour with rhabdomyosarcoma, what will you see?

A

Proptosis

83
Q

What are some early signs of rhabdomyosarcoma?

A

Tropia and limitation of eye movements

84
Q

What is the treatment rhabdomyosarcoma?

A

Radiotherapy or chemotherapy

Binocular Vision (30 decks)

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