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1. Clin Med III > 3- Myasthenia Gravis & MS > Flashcards

3- Myasthenia Gravis & MS Flashcards

1
Q

What is the most common disorder of neuromuscular transmission?

A

Myasthenia gravis

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2
Q

Who is affected by myasthenia gravis?

A

Bimodal

20s-30s, 60s-80s

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3
Q

What are the 2 types of myasthenia gravis?

A

Ocular, generalized

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4
Q

What is myasthenia gravis?

A

Autoimmune attack to ACh receptors at the NMJ leading to muscle fatiguability and weakness

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5
Q

What is the hallmark of myasthenia gravis?

A

Fluctuating weakness/ fatigue of:

Ocular- most common

Bulbar- speech, chewing, swallowing

Facial muscle weakness- “myasthenic sneer”

Neck- “dropped head syndrome”

Limbs- UE > LE

Respiratory muscles- “myasthenia crisis”

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6
Q

When is the weakness and fatigue a/w myasthenia gravis worse?

A

End of day, after exercise

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7
Q

What triggers may worsen myasthenia gravis?

A

Anything that causes stress on the body

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8
Q

What drugs should be avoided in pts with myasthenia gravis?

A

Fluoroquinolones

Beta blockers

Hydroxychloroquine

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9
Q

What tests (although not confirmatory) performed on PE may indicate MG?

A

Upward gaze x 1 min = ptosis

Ice pack test w/ ptosis = improved sxs

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10
Q

What will electrophysiologic studies (NCS and EMG) show for a pt with MG?

A

Decreased response on repetitive nerve stimulation

Fatiguability

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11
Q

How is MG diagnosed?

A

Serologic tests- AChR Ab, MuSK Ab

CT/ MRI chest- thymic hyperplasia/ thymoma

(Edrophonium/ Tensilon test = immediate increase in muscle strength, no longer used)

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12
Q

How would you be able to distinguish between MG and ALS?

A

ALS does not have ptosis or diplopia

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13
Q

Lambert-Eaton myasthenic syndrome is an AI disease often a/w malignany and due to reduced ACh release in the NMJ. How would you distinguish these sxs from those of MG?

A

Proximal muscle weakness

Sxs worse in am and improve with exercise

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14
Q

What is used for symptomatic tx of MG?

A

Pyridostigmine- AChE inhibitor

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15
Q

What meds are used for chronic immunotherapies in the tx of MG?

A

Glucocorticoids, Azathioprine- immunomodulating agents

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16
Q

What meds are used for rapid (short acting) immunomodulating therapies in the tx of MG?

A

Plasma exchange, IVIG immune globulin

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17
Q

What is the surgical therapy for MG?

A

Thymectomy

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18
Q

What MG treatment has rapid onset and improves strength (limb/ bulbar > ocular), and what is the caution with use?

A

Pyridostigmine

Cholinergic SEs

Not sufficient monotherapy (add immunotherapy)

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19
Q

What is the 2nd leading cause of permanent disability in young adults?

A

MS

20
Q

MS is an immune mediated inflammatory demyelinating disease of CNS that leads to what?

A

Plaques/ demyelination → affects nerve transmission

21
Q

What is the imaging test of choice for MS?

A

MRI w/ and w/o contrast

(shows plaques +/- black holes/ brain atrophy)

22
Q

What is the hallmark of MS?

A

Symptomatic episodes occur months- years apart and affect different anatomic locations (separation of time and space)

23
Q

What is the most common type of MS?

A

Relapsing-remitting disease (RRMS)

24
Q

What is the 1st attack of MS?

A

Clinically isolated syndrome (CIS)

25
Q

What type of MS is defined as clearly defined relapses with full recovery or some deficit?

A

Relapsing-remitting disease (RRMS)

26
Q

What type of MS is defined as initially RRMS w/ gradual worsening and typically occurs 10-20 yrs after MS dx?

A

Secondary progressive (SPMS)

27
Q

What type of MS is defined as progressive sx increase from disease onset with no remission and is the most aggressive type?

A

Primary-progressive (PPMS)

28
Q

What is defined as transient worsening of MS due to elevated body temp?

A

Uhthoff phenomenon

29
Q

Most common clinical presentation of RRMS is a young adult with episodes of what sxs that occur over hours- days and resolve over weeks to months?

A

Paresthesia-sensory (most common initial feature)

Optic neuritis

Weakness

30
Q

On PE pt notes an electrical shock sensation down back/ limbs with neck flexion. What is this called and what condition is it a/w?

A

Lhermitte sign, MS

31
Q

On PE pt notes a defective pupillary reaction to light which you believe is due to optic nerve dysfunction. What is this called and what condition is it a/w?

A

Marcus Gunn pupil (affected pupil does not constrict in response to bright light)

MS

32
Q

Nystagmus, incoordination, and tremor are signs of cerebellar involvement with what condition?

A

MS

33
Q

Hyperreflexia, (+) Babinski sign, and clonus are UMN signs a/w what condition?

A

MS

34
Q

How is MS diagnosed?

A

CNS lesion dissemination in time and space

Clinical findings + MRI findings (plaques)

35
Q

What is the key principle of the McDonald criteria used in the diagnosis of MS?

A

Demonstration of CNS lesions disseminated in space and time

Damage in more than one place in CNS

Damage has occured more than once

36
Q

Additional tests used to dx MS might include analysis of CSF. What would you expect to see?

A

Oligoclonal bands

(appearance/ pressure N, total WBC N in 2/3 of pts)

37
Q

Evoked potential studies such as electrical agents generated in CNS by peripheral stimulation of sensory organ and tools used to measure time a stimulus takes to reach the cerebral cortex are used to detect abn CNS function and can contribute to what dx?

A

MS

38
Q

Can abn MRI changes indicative of MS be found in clinically healthy patients?

A

YES, but should still refer for workup

39
Q

MS tx varies based on type. What is first line tx for relapsing-remitting MS (RRMS)?

A

Disease modifying therapy

(primary and secondary progressive MS types are more difficult to treat and have fewer effective tx options)

40
Q

What is the role of disease modifying therapies (immune modulating meds) in the management of MS?

A

Not a cure

Decrease relapse rate, slower accumulation of MRI lesions, continuted indefinitely

41
Q

What should first be ruled out in the tx of an acute exacerbation of MS?

A

Infection

42
Q

What is the goal of tx of an acute exacerbation of MS and what is the tx?

A

Speed recovery time from attack

Glucocorticoids (IV methylprednisone) = 1st line

+/- ACTH (steroid alternative), plasma exchange/ plasmapheresis (if poor response to steroids)

43
Q

When should a pt with MS be admitted?

A

Unable to manage at home, severe relapses

44
Q

What MS pts should be referred?

A

All pts with MS should be followed by a neurologist

45
Q

Why is a multidisciplinary approach for sx management in patients with MS critical?

A

At risk for developing many other sxs

(ex. depression, spasticity, bladder dysfunction, gait impairment)

46
Q

Eventual disability is common with MS dx, how much is life expectancy typically reduced by?

A

7-14 years

1. Clin Med III (29 decks)

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