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X Clin Med III Final Exam > Myasthenia Gravis and Multiple Sclerosis > Flashcards

Myasthenia Gravis and Multiple Sclerosis Flashcards

1
Q

Most common disorder of neuromuscular transmission

A

Myasthenia gravis

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2
Q

What is myasthenia gravis?

A

Autoimmune disorder of the acetylcholine receptors at the NMJ causing weakness and fatiguability of muscles

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3
Q

Distribution of myasthenia gravis

A

20-30s (more females)

60s-80s (more males)

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4
Q

Types of myasthenia gravis

A

Ocular (can progress to next)

Generalized

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5
Q

What might play a role in myasthenia gravis?

A

Thymus (maybe due to similar cells that are on skeletal muscles)-do CT or MRI of chest
Most pts have thymus hyperplasia or thymoma

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6
Q

Hallmark of myasthenia gravis

A

Fluctuating weakness in ocular, bulbar (speech, chewing, swallowing), limb or respiratory muscles

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7
Q

Sxs of myasthenia gravis

A

Fluctuating weakness and fatigue of specific muscle groups (worse at end of day or after exercise)

  • ptosis/diplopia
  • dysarthria, dysphagia, chewing difficulties
  • myasthenic sneer (facial muscle weakness)
  • Dropped head syndrome and limb weakness in arms more than legs
  • Myasthenic crisis due to resp muscles
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8
Q

Triggers than can worsen myasthenia gravis

A 
Hot temps
Surgery
Emotional stress
Illness
Menstruation, pregnancy, childbirth
Medication
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9
Q

Drugs to avoid with myasthenia gravis

A

Fluoroquinolones
BBs
Hydroxychloroquine (Plaquinil)

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10
Q

How to diagnose myasthenia gravis

A

History and physical
Maintain upward gaze x 1 min (ptosis due to fatigability)
Ice pack test in pt with ptosis should improve sxs
NCS and EMG show decrease response on repetitive nerve stimulation and fatigability

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11
Q

Serologic tests for myasthenia gravis

A 
Autoantibodies:
AChR Ab (Ach receptor antibodies)
MuSK Ab (muscle specific kinase antibodies)
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12
Q

Edrophonium (Tensilon) Test

A

Used to be used for myasthenia gravis (prolongs presence of Ach to stimulate limited number of AChR and it shows immediate increase in muscle strength)

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13
Q

How to tell generalized MG from ALS

A

ALS does not have ptosis or diplopia

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14
Q

Lambert-Eaton myasthenic syndrome

A

Reduced Ach release in NMJ
Autoimmune disease often associated with malignancy (SCLC)
Proximal muscle weakness and sxs worse in AM and improve with exercise!!

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15
Q

Symptomatic tx for myasthenia gravis

A

Oral acetylcholinesterase inhibitor (Pyridostigmine/Mestinon) similar to Tensilon so short acting

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16
Q

Chronic immunotherapies for myasthenia gravis

A

Immunomodulating agents (glucocorticoids or azathioprine)

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17
Q

Rapid (short acting) immunotherapies for myasthenia gravis

A

Plasmapheresis/plasma exchange-directly removes AChR antibodies from circulation
IVIG-pooled immunoglobulin from thousands of donors
(can be used as bridge for chronic therapy)

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18
Q

Last choice for myasthenia gravis tx

A

Thymectomy in select pts (remission is possible)

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19
Q

Use of pyridostigmine/Mestinon

A

Limb and bulbar sxs improve more than the ocular sxs
Allows prolonged effect of Ach strength
Titrate and watch for cholinergic SEs (not great for generalized MG tho)

20
Q

Epidemiology of multiple sclerosis

A

Usually diagnosed 15-45 YO
More females
Leading cause of permanent disability in young adults

21
Q

What is multiple sclerosis?

A

Immune mediated attack on axons and myelin sheaths of nerve fibers causing plaques or demyelination and affects nerve transmission

22
Q

Imaging test of choice for MS

A

MRI with and without contrast of brain (revealing plaques within myelin)

23
Q

Hallmark of MS

A

Symptomatic episodes occur months-yrs apart and different anatomic locations (based on clinical findings alone or clinical findings and MRI)
*separation of time and space

24
Q

Possible risk factors for MS

A 
Viral infections
Geographic factors related to latitude
Sunlight and Vit D (may be protective)
Genetic susceptibility
Tobaccos
25
Q

Types of MS

A

Relapsing-remitting disease
Secondary progressive
Primary progressive
Clinically isolated syndrome (1st attack-no prior sxs but now have acute symptomatology)

26
Q

Relapsing-remitting MS

A

Most common type of MS

Clearly defined relapses with full recovery or some deficit

27
Q

Secondary progressive MS

A

Initially RRMS with gradual worsening

Usually 10-20 yrs after dx

28
Q

Primary progressive MS

A

Progressive sx increase from disease onset with no remission

29
Q

What is Uhthoff phenomenon?

A

Specific to MS-transient worsening due to elevated body temp

30
Q

Typical patient with RRMS

A

Young adults with episodes (relapses/attacks/exacerbations) of CNS dysfunction
Paresthesia
Optic neuritis
Weakness
Sxs develop over hrs-days and resolve wks-mos

31
Q

Most common initial feature of MS

A

Paresthesia (sensory sxs)

32
Q

What is Lhermitte sign?

A

MS

Neck flexion causes electrical shock sensation down back/limbs

33
Q

What is the Marcus Gunn Pupil?

A

Due to optic neuritis with MS: defective pupillary rxn to light (affected pupil does not constrict in response to bright light)

34
Q

Other signs of MS

A

Cerebellar involvement: nystagmus, incoordination, tremor

UMN signs: hyperreflexia, Babinski, clonus

35
Q

How to diagnose MS

A

Combo of clinical findings from history and physical and MRI finding (imaging of brain and spinal cord may reveal plaques)

36
Q

What is shown on CSF analysis for MS?

A

Oligoclonal bands (CNS inflammation)
Appearance and pressure are normal
Total WBC count are normal (if elevated than consider other causes)

37
Q

Evoked potential studies for MS

A

Electrical events generated in CNS by peripheral stimulation of a sensory organ
Measure time a stimulus takes to reach cerebral cortex

38
Q

What are leukodystrophies?

A

Rare disease affecting myelin (brain, spinal cord and peripheral nerves)-can be seen on MRI

39
Q

1st line tx for RRMS

A

Disease-modifying therapy (infusion, injection, oral therapy)

40
Q

Tx for primary and progressive MS

A

More difficult to treat and fewer option
Some benefit with DMT
Others can try immune modulating tx (methotrexate, steroids, stem cells transplant, IVIG)

41
Q

Disease modifying therapies for MS

A

Immune modulating
Decrease relapse rate and slower accumulaiotn of MRI brain lesions
Lower long term risk of disease progression

42
Q

What must be ruled out before using steroids for MS

A

Infection

43
Q

First line tx for acute exacerbation of MS

A

Glucocorticoids (IV methylprednisolone 1000 mg daily x 5 days and short prednisone taper)

44
Q

Other options for acute exacerbation of MS tx

A 
ACTH injections (expensive)
Plasma exchange or plasmapheresis (if poor response to steroids)
45
Q

When to admit with MS

A

Pt unable to manage at home

Severe relapses

46
Q

Who to refer with MS

A

All pts with MS should have neurologist (multidisciplinary team)

X Clin Med III Final Exam (6 decks)

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