Autoimmune Disorders

Question 1

Dz list

Answer 1

*Myasthenia Gravis - NM junction

Peripheral=

• Guillain-Barre (AIDP) =
• Chronic inflammatory demyelinating Polyneuropathy

Question 2

• Severe weakness increasing over time
• drooping eyelids/head
• double vision
• cough w/ green sputum
• normal intermittently

Answer 2

Myesthenia gravis

• WATCH AIRWAY
• myasthenic crisis

Question 3

Myesthenia Gravis s/s

Differential

Answer 3

Neuromuscular transmission disorders

• lambert-eaton myasthenic syndrome
• toxins-organophosphates

CNS - unlikely

ALS = no ocular s/s

Myopathy = no ocular

Question 4

Myesthenia Gravis

Dangerous Rx

Answer 4

Levaquin

Myesthenia.org= look at list

Question 5

Myesthenia Gravis

Path

Answer 5

Autoimmune antibodies stick to Ach receptors (skeletal muscle)

Question 6

Myesthenia Gravis

DX evaluation

Answer 6

1) suspicion
2) Tensilon, sleep icepack test
3) AChR Antibody (cheap)
4) muscle specific kinase antibody

Question 7

Myesthenia Gravis

MC cause

Answer 7

Tumor - immune against tumor turns on muscle

CHEST CT or MRI = thymoma

Question 8

Myesthenia Gravis

Other causes

Answer 8

• Thyroid function
• Ana profile, rheumatoid factor
• Tb testing

Question 9

Myesthenia Gravis

Tx

Answer 9

Thymectomy MC

Thymoma tumor only in 10%

Question 10

Myesthenia Gravis

Pyrodostigmine/Mestinon

Rx

Answer 10

(AchE inhibitor)

Rare that it completely works

• SE = stomach cramp, diarrhea, excess salivation/asthma, bradycardia
• nicotinic adverse effects

Question 11

Myesthenia Gravis

Rx. Prednisone

Answer 11

*most effective

Many SE - LIST

Question 12

Myesthenia Gravis

Acute Tx

Answer 12

Plasma exchange

Intravenous Immunoglobulin (IVIG) =

Question 13

Myesthenia Gravis

Immunosuppressive Tx

Answer 13

Azathioprine, mycophenolate, cyclosporine, tacrolimus, rituxan

*long-term risk of cancer

Question 14

• Ascending paralysis
• Demylenation
• Motor problems/weakness
• Quick acute progression- 24 hours
• 1-3 weeks slow progression
• Huge variation in progress
• absent reflexes

s/s?

Answer 14

Guillan barre syndrome

Question 15

Guillan barre syndrome

Tx

Answer 15

Plasma exchange/IVIG

Just decrease course of ICU/hospital stay by 2-4 weeks (decrease progression)

Critical care during extreme weakness

Question 16

Guillan barre syndrome

Who?

Answer 16

Male

Question 17

Guillan barre syndrome

Path

Answer 17

Antibodies stick to myelin, demyelinate

Slow nerve conduction

Question 18

Highly variable, can look like GBS

• some are resistant to treatment - death
• losing myeline = EMP? Distal to proximal stimulation = less

Present?

Answer 18

CIDP

Question 19

Guillan barre syndrome

Tx

Answer 19

Prednisone = slow response

If No help = IV Ig therapy

Supportive if others fail

Immunosuppressives = poor response

Question 20

Myesthenia Gravis

Dx - Nerve Stimulation

Answer 20

*EMG w/ Repetitive Stimulation
THEN
*Single-Fiber Examination

Question 21

Myesthenia Gravis

Thymoma Evaluation

S+ Others

Answer 21

• Chest CT/MRI
• 10% patients
• Thyroid functions tests
• ANA profile/Rheumatoid factor
• TB Testing

Question 22

Generalized MG

Rx

Answer 22

• Azathioprine - PANCYTOPENIA

* Mycophenolate mofetil - BIRTH DEFECTS

Question 23

• Ascending paralysis
• Motor problems, weakness
• 1-3 weeks slow buildup
• LOSE position sense/toe sens/reflexes
• QUICK ACUTE PROGRESSION = 24 hours –> intubation

Answer 23

Guillen-Barre

Question 24

Guillen-Barre

Dx

Answer 24

1ary

• Electromyography/Nerve Conduction studies (DEMYELENATION)
• CSF: UP protein, NO WBC

Question 25

Guillen-Barre Causes

Answer 25

* Response to vaccine * Underlying Infxn - Campylobacter - EBV, CMV HIV

Question 26

Acute weakness with normal sensorium Differential

Answer 26

* Myesthenia Gravis * Toxins (run thru field) * Lyme, West N * Spinal Cord Injury * Critical Illness