neuromusc disorders not on test

Question 1

Botulism

Answer 1

caused by botulinum toxin= neurotoxin
8 diff types: G and H= Human dz

Tx: Antitoxin
MOA: binds to toxin and prevents activity
CANNOT REVERSE EXISTING PARALYSIS
tx based on index of suspicion-> may still require ventilator support if resp compromise present

Botulinum antitoxin (BAT)= for “non-infant” botulism (foodborne)
from health department
AE: anaphylax bc derived form horse

Botulinum immune globulin= for infant botulism
infants <1 yr
IV
HIGHER THAN 1 YR B4 HAVING HONEY

Wound Botulism
Debride wound (low bacterial burden and removes contam tissue)
give tetanus booster if over 5 yrs since last
culture wound
Abx MAY not be beneficial-> SPECIFICALLY NOT RECOMMENDED FOR OTHER FORMS OF BOTULISM
IV PENICILLIN CAN DECREASE ORGANISM BURDEN
If wound infxn present then use ABX

Question 2

Myasthenia Gravis

Answer 2

symptomatic tx= Acetylcholinesterase inhibitors
Chronic immunosuppressive therapies= Glucocort and nonsteroidal immunosuppressive agents
Rapid, short acting immunomodulating tx= therapeutic plasma exchange and IV immune globulin
surg= thymectomy

Question 3

MG symptomatic tx

Answer 3

Acetylcholinesterase inhibitors= Neostigmine, pyridostigmine
DOES NOT INFLUENCE COURSE OF DZ
Goal: increase Ach in NMJ
ae: cramping, diarrhea, nausea, sweating salivation

Question 4

MG chronic immunosuppressive therapies

Answer 4

Prednisone daily
Ae w/ prolonged use: osteoporosis, weight gain, diabetes, increased risk of infection, increased body fat, redistribution of body fat

concurrent use of immunosuppressants may allow lower dose of steroids to be used

Question 5

MG plasmapheresis or IV immune globulin

Answer 5

plasmapheresis= therapeutic plasma exchange
may help in major disability or stabilize pt b4 thymectomy

Question 6

Myasthenia crisis

Answer 6

triggers include: infection, surgery, pregnancy, steroid taper, ab, beta blockers

admit to ICU:
1. Plasma exchange
2. Intravenous Immune Globulin (IVIG)
3. Highdose steroid

Question 7

MG tx for refractory dz

Answer 7

1. mainenance IVIG
2. Rituximab (Rituxan)- monoclonal ab
3. Eculizumab (Soliris)
4. Cyclophosphamide
   -decrease lymphocyte prolif

Question 8

Multiple sclerosis tx

Answer 8

acute episodes:
1. Dexamethasone
2. Methylprednisolone
3. Prednisone

Primary PRogressive:
Ocrelizumab (Ocrevus)- anti-CD20 monoclonal ab

1st line tx for relapse prevention:
Dimethyl fumarate (Techfidera), Fingolimod (Gilenya), Glatiramer acetate (Copaxone), interferons

Question 9

Amyotrophic lateral Sclerosis ALS

Answer 9

1. Riluzole (Exservan, Rilutek, Tiglutik) - glutamate inhibitor
   reduces glutamate levels and block NMDA receptors
   AE: dizzy, GI
2. Edaravone (Radicava)- free radical scavenger
   reduces decline in ADLS

Question 10

Spasticity

Answer 10

from stroke, brain/spinal injury, cerebral palsy, progressive MS
INCREASED RESISTANCE TO PASSIVE MOVEMENT PLUS HYPERREFLEXIA
can cause chronic pain

tx:
1. skeletal musc relaxants- baclofen, cyclobenzaprine, methocarbamol
2. benzodiazepines- clonazepam, diazepam
3. Centrally acting alpha 2 adrenergic agonist-
HIGH RISK OF SEDATION