Neuromuscular Disorders

Question 1

Information gathering MG/GB

Answer 1

Muscle tone
Deep tendon reflex
Gag reflex
Ability to swallow
Glasgow
EEG
EMG
ICP

Question 2

Treatment/decision making GB/MG

Answer 2

VC and NIF monitoring
O2 therapy (>90%)

Question 3

Recommend intubation for GB/MG when

Answer 3

VC<1L or <15ml/kg (or rapidly declining)
NIF< -25 MEP<40 (or rapidly declining)
Inability to cough, swallow and protect airway
ABG: respiratory failure
Aspiration pneumonia/severe hypoxemia

Question 4

GB information gathering

Answer 4

General assessment (recent febrile illness, ascending, pain)

Question 5

GB diagnostic tests

Answer 5

Lumbar puncture (increased protein, low WBC)
EMG/NCS
Antibody tests
Liver enzymes
Serology
Breath sounds
Spirometry
ABG
CXR

Question 6

MG info gathering/dx tests

Answer 6

Painless, descending paralysis
Antibody test: increased anti-AchR
Positive tensilon (edrophonium) test
CT/MRI: thymoma
EMG/NCS
Breath sounds
Spirometry
ABG
CXR

Question 7

GB treatment/decision making

Answer 7

VS/SpO2/ECG monitoring
Plasmapheresis
IV immunoglobulin
NSAIDS/opioids
Fluids/trendelenburg (severe hypotension)
DVT
Rehab during recovery

Question 8

MG tx/decision making

Answer 8

Acetylcholinesterase inhibitor (mestinon)
Immunosuppressant therapy
Thymectomy
Plasmapheresis
IV immunoglobulin
DVT
rehab during recovery

Question 9

Muscular Dystrophy stage 1 assessment/IG

Answer 9

Normal respiratory function
Immunizations
Annual physician visits (reassess baseline)
Baseline respiratory function (SpO2, spirometry, peak flow, NIF/MEP)

Question 10

Muscular dystrophy stage 1 treatment/DM

Answer 10

Pt/caregiver disease education:
preventative care
airway clearance strategies
monitor respiratory function

Question 11

Muscular dystrophy stage 2 assessment/IG

Answer 11

Adequate ventilation, ineffective cough (VC<80% predicted,over age of 12)
Biannual pulmonary visits

Question 12

Muscular dystrophy stage 2 treatment/DM

Answer 12

Airway clearance regimen (cough assist) when: Peak flow <270L/min or MEP <60
Volume recruitment/deep lung inflation when: FVC<40% or <1.25L

Question 13

Muscular dystrophy stage 3

Answer 13

Adequate daytime ventilation, inadequate nighttime ventilation
Quarterly pulmonologist visits
Awake PetCO2
NPPV when:
s&s hypoventilation (fatigue, dyspnea, headache lack of concentration, hyper somnolence)
SpO2 <95% or PetCO2 < 45 while awake
Apnea/hypopnea index >10 or 4 ODI
No CPAP!

Question 14

Muscular dystrophy stage 4

Answer 14

Inadequate daytime/nighttime ventilation
Quarterly pulmonologist visits
Continuos SpO2 monitor
PetCO2
Daytime NPPV when:
PetCO2 >50
SpO2 <92% when awake
Mouth piece
Trach eventually

Question 15

Tetanus assessment/IG

Answer 15

Trismus/spasms
Dysphasia/abnormal gag
Neck stiffness
Abdominal wall rigidity
EMG
Toxicology

Question 16

Tetanus treatment/DM

Answer 16

IM tetanus immunoglobulin
Metronidazole (flagyl )
Intubate w sucks
IV benzo