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Neuro. CM3 > Neuromuscular Disorders > Flashcards

Neuromuscular Disorders Flashcards

1
Q

Onset of myasthenia gravis (decade):

A

F: 2nd - 3rd
M: 5th - 6th

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2
Q

What is affected in Myasthenia gravis?

A

Antibodies to the acetylcholine receptors (B and T cells are both involved)

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3
Q

When are the MG symptoms better?

A

With rest and with cold

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4
Q

When are MG symptoms worse?

A

In the summer and as the day progresses

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5
Q

Most common areas of weakness with MG:

A
  • Eyelid/eyes
  • Bulbar (swallowing)
  • Respiratory
  • Extremities (proximal > distal)
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6
Q

What is a distinctive feature of MG?

A

They maintain good pupil responses

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7
Q

What is generalized MG? What percent become generalized in the first year?

A

When it spreads anywhere other than the eyes - 75% has generalized in the first year

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8
Q

How can you bring ptosis out?

A

Sustained upwards gaze - one eye will have more than the other

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9
Q

What is the curtain sign in MG?

A

If you open the eye with more ptosis really wide, the other one will go down

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10
Q

Do you have intact sensation with MG?

A

Yes - only motor is lost

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11
Q

What is a myasthenia crisis? (15-20%)

A

Respiratory failure - very serious (need mechanical ventilation)

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12
Q

What is the Tensilon test?

A

Diagnoses MG - give a short-acting cholinesterase inhibitor *Need atropine available

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13
Q

What is CMAP?

A

Compound muscle action potential - will decrease over time in MG patients

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14
Q

Testing for MG (2):

A

AChR serum testing - if negative, run a Anti-MUSK

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15
Q

What two things are scanned for with a CT in a patient with MG?

A
  • Thymoma (10%)

- Thymic lymphofollicular hyperplasia

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16
Q

Two tests for formal diagnosis of MG:

A
  • EMG and Nerve conduction study

* Increased jitter in MG

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17
Q

Treatment for MG (3):

A
  • Cholinesterase inhibitor: Pyridostigmine
  • Corticosteroids: Prednisone
  • Chemotherapeutic (3)
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18
Q

What treatment is the first immune therapy choice for MG?

A

Corticosteroids - Remission in 50%

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19
Q

What are the three chemotherapeutic medications for MG?

A
  • Azathioprine - Takes 1 year
  • Cyclosporine - MUST be on BC
  • Eculizumab - 3rd line, last resort
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20
Q

What drug that is chemotherapeutic has a risk for meningitis infection with MG?

A

Ecluizumab

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21
Q

Treatment of Myasthenia crisis - rapid treatment (2):

A

IV Ig - HA is most common adverse reaction

Plasmapheresis - Remove antibodies and immune complexes

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22
Q

Option for MG patients if medications do not work at all:

A

Thymectomy - rec. If they have a thymoma (most helpful 1-2 years after symptoms)

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23
Q

What do you want to discontinue if someone is having a myasthenia crisis?

A

Anticholinesterase meds (will increase secretions)

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24
Q

What do you not want to treat someone with if they are having a myasthenia crisis?

A

Steroids - Can prolong the crisis

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25
Q

What is transitory neonatal MG?

A

baby receives antibodies from mom (1st four days - resolve sin 1 week)

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26
Q

Congenital arthrogryposis

A

Getting stuck in certain positions

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27
Q

Lambert-Eaton Myasthenia Syndrome physiology:

A

Antibodies at the P/Q calcium gated channels - decrease NT released

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28
Q

LEMS is _____ or ____

A

Autoimmune or paraneoplastic

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29
Q

If someone has LEMS what type of cancer do 50% of patients have?

A

Small cell carcinoma of the lung

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30
Q

What are the two oral symptoms of LEMS?

A

Metallic taste and dry mouth

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31
Q

What are the four autonomic symptoms with LEMS?

A
  • Orthostasis
  • Constipation
  • Impotence
  • Decreased sweating
32
Q

You can have pain with what neuromuscular disorder?

A

LEMS

33
Q

Repetitive Nerve Stimulation for LEMS:

A

Initially low and then doubles with marked facilitation

34
Q

If you have a patient with LEMS, you must always evaluate for:

A

MALIGNANCY - if not found, check again in 2-4 years

35
Q

Three treatments for LEMS:

A
  1. Remove the tumor if there is one
  2. 2,3-DAP: Blocks volatage gated K+ channels = prolonged action potential
  3. Guanidine hydrochloride: Inhibits Ca2+ reuptake
36
Q

What two drugs do not work for LEMS?

A
  • Cholinesterase drugs

- Immunosuppressives

37
Q

DTRs are ____ in LEMS

A

Absent or decreased

38
Q

DTRs are ___ in MG

A

Normal

39
Q

Botulism binds:

A

Irreversibly - ACh not released

40
Q

What two things will you find on PE with someone that has botulism poisoning?

A
  • DTRs are absent

- Pupils unreactive

41
Q

GI symptom onset with botulism:

A

3-4 hours

42
Q

Those with botulism will have these three symptoms:

A
  1. Descending paralysis
  2. Autonomic (constipation, postural hypotension, urinary retention)
  3. Ocularbulbar problems
43
Q

Treatment for botulism:

A

Intensive supportive care

***Trivalent botulism antitoxin may work but only on the nerves where the toxin has not bound yet

44
Q

When 5-10% of people die with botulism poisoning, what are the two causes of death?

A
  • Respiratory failure

- Pulmonary/systemic infections

45
Q

Genetics for Duchenne Muscular dystrophy:

A

Mutation in the DMD (dystrophin) - located on the X chromosome (recessive)

46
Q

If the female mom for DMD has symptoms, what two systems are affected?

A

cardiac or cognitive involvement

47
Q

Job of dystrophin protein:

A

Strengthen the muscle fibers and provide tensile strength, cell/cell communication.

48
Q

15% of DMD patients have which type of mutation?

A

Point mutation/substitution

49
Q

DMD milestone plans: Walking, wheel chair and death

A

Walking: 14 - 16 months
Wheel chair: 10 - 15 (current tx).
Death: 20’s - 30’s

50
Q

Three PE findings of DMD:

A
  1. Calf hypertrophy (fat)
  2. Proximal weakness
  3. DTRs diminished then gone
51
Q

What is the Gower’s Maneuver?

A

In DMD - Weak and need to push themselves up

52
Q

What neuromuscular disorder has the walling Trendelenburg gait?

A

DMD

53
Q

What three labs do you order for DMD?

A
  • CK (10-50x higher than normal)
  • Aldose
  • Gene test
54
Q

If you see high AST/ALT in a young male with weakness…think:

A

DMD - AST/ALT are also in muscles (breakdown)

55
Q

Medications for DMD (they also all get supportive care):

A

Corticosteroids (Emflaza/Prenisone)

56
Q

What four things are you monitoring if a patient with DMD is on corticosteroids?

A
  1. Hyperglycemia
  2. HTN (have them on low Na diet)
  3. Osteoporosis (annual bone scans)
  4. PUD (have on PPIs)
57
Q

Someone with DMD can have common complications in what three systems?

A
  1. Pulmonary (yearly PFTs)
  2. Cardiac (ACE/BB)
  3. Ortho (scoliosis, clinical evals/xray annually)
58
Q

Becker Muscular dystrophy is like DMD but:

A

More mild (later onset) - live longer/walk longer

59
Q

In terms of cardiac problems, people with Becker’s compared to DMD have:

A

Less cardiomyopathy but MORE CONDUCTION PROBLEMS

60
Q

Myotonic Dystrophy - Most common in:

A

Adults

61
Q

DMD is most common in:

A

Kids

62
Q

Myotonic dystrophy genetics:

A

Autosomal dominant, trinucleotide expansion disorder *Anticipation

63
Q

Five symptoms of Myotonic dystophy:

A
  1. Myopathic face (tent)
  2. Frontal balding
  3. Ptosis
  4. Distal over proximal weakness
  5. Cardiac problems - risk for SCD
64
Q

You must do a ___ to look for ___ in someone with Myotonic Dystrophy

A

EKG; conduction abnormalities

65
Q

CK will be __ in people with Myotonic Dystrophy

A

Mild/normal

66
Q

Two drug for the treatment of Myotonic Dystrophy:

A

Mexilitine, phenytoin

67
Q

Congenital Myotonic Dystrophy

A

Babies born with 3,000-4,000 repeats that have super severe disease - need respiratory support; high rate of death

68
Q

Spinal Muscular Atrophy types and genetics:

A

Type 1, Type 2 (rarer)
Chromosome 5

Autosomal-recessive *2nd most common

69
Q

What is SMA?

A

Progressive weakening of the lower motor neurons

70
Q

SMA type 1 is ___

A

Most severe (die in 7 months)

71
Q

SMA type 2 and 3 are ___

A

Better. Type 2 lives to 20’s and type 3 can live normally.

72
Q

SMA types and sitting/standing patterns:

A

1: Never sit - severe
2. Sit but don’t stand - Respiratory problems
3. Stand but not long term

73
Q

SMA type 1 mental status:

A

Intact

74
Q

Gene for SMA (2):

A

SMN1: Whole length protein
SMN2: Makes truncated protein

75
Q

Loss of the SMN1 gene results in:

A

Destruction of the anterior horn and peripheral nerves just like ALS

76
Q

Future genetic treatment - Exon skipping for SMA:

A

Nusinersen and Zolgensma

Neuro. CM3 (11 decks)

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