Autoimmune Disorders Flashcards
Dz list
*Myasthenia Gravis - NM junction
Peripheral=
- Guillain-Barre (AIDP) =
- Chronic inflammatory demyelinating Polyneuropathy
- Severe weakness increasing over time
- drooping eyelids/head
- double vision
- cough w/ green sputum
- normal intermittently
Myesthenia gravis
- WATCH AIRWAY
- myasthenic crisis
Myesthenia Gravis s/s
Differential
Neuromuscular transmission disorders
- lambert-eaton myasthenic syndrome
- toxins-organophosphates
CNS - unlikely
ALS = no ocular s/s
Myopathy = no ocular
Myesthenia Gravis
Dangerous Rx
Levaquin
Myesthenia.org= look at list
Myesthenia Gravis
Path
Autoimmune antibodies stick to Ach receptors (skeletal muscle)
Myesthenia Gravis
DX evaluation
1) suspicion
2) Tensilon, sleep icepack test
3) AChR Antibody (cheap)
4) muscle specific kinase antibody
Myesthenia Gravis
MC cause
Tumor - immune against tumor turns on muscle
CHEST CT or MRI = thymoma
Myesthenia Gravis
Other causes
- Thyroid function
- Ana profile, rheumatoid factor
- Tb testing
Myesthenia Gravis
Tx
Thymectomy MC
Thymoma tumor only in 10%
Myesthenia Gravis
Pyrodostigmine/Mestinon
Rx
(AchE inhibitor)
Rare that it completely works
- SE = stomach cramp, diarrhea, excess salivation/asthma, bradycardia
- nicotinic adverse effects
Myesthenia Gravis
Rx. Prednisone
*most effective
Many SE - LIST
Myesthenia Gravis
Acute Tx
Plasma exchange
Intravenous Immunoglobulin (IVIG) =
Myesthenia Gravis
Immunosuppressive Tx
Azathioprine, mycophenolate, cyclosporine, tacrolimus, rituxan
*long-term risk of cancer
- Ascending paralysis
- Demylenation
- Motor problems/weakness
- Quick acute progression- 24 hours
- 1-3 weeks slow progression
- Huge variation in progress
- absent reflexes
s/s?
Guillan barre syndrome
Guillan barre syndrome
Tx
Plasma exchange/IVIG
Just decrease course of ICU/hospital stay by 2-4 weeks (decrease progression)
Critical care during extreme weakness
Guillan barre syndrome
Who?
Male
Guillan barre syndrome
Path
Antibodies stick to myelin, demyelinate
Slow nerve conduction
Highly variable, can look like GBS
- some are resistant to treatment - death
- losing myeline = EMP? Distal to proximal stimulation = less
Present?
CIDP
Guillan barre syndrome
Tx
Prednisone = slow response
If No help = IV Ig therapy
Supportive if others fail
Immunosuppressives = poor response
Myesthenia Gravis
Dx - Nerve Stimulation
*EMG w/ Repetitive Stimulation
THEN
*Single-Fiber Examination
Myesthenia Gravis
Thymoma Evaluation
S+ Others
- Chest CT/MRI
- 10% patients
- Thyroid functions tests
- ANA profile/Rheumatoid factor
- TB Testing
Generalized MG
Rx
- Azathioprine - PANCYTOPENIA
* Mycophenolate mofetil - BIRTH DEFECTS
- Ascending paralysis
- Motor problems, weakness
- 1-3 weeks slow buildup
- LOSE position sense/toe sens/reflexes
- QUICK ACUTE PROGRESSION = 24 hours –> intubation
Guillen-Barre
Guillen-Barre
Dx
1ary
- Electromyography/Nerve Conduction studies (DEMYELENATION)
- CSF: UP protein, NO WBC
Guillen-Barre
Causes
- Response to vaccine
- Underlying Infxn
- Campylobacter
- EBV, CMV HIV
Acute weakness with normal sensorium
Differential
- Myesthenia Gravis
- Toxins (run thru field)
- Lyme, West N
- Spinal Cord Injury
- Critical Illness
