neuromuscular disease

Question 1

asthenia

Answer 1

Motor impairment, not d/t loss of power (strength)Affected task performance“Heavy” or “Stiff” limbs Pain uncommon

Question 2

muscle weakness

Answer 2

fxnal impairment → loss of power S/t other dis processes → CVS, infection, inflammatory disease, depression, pain commonly assoc

Question 3

asthenia vs muscle weakness

Answer 3

asthenia: d/t motor impairmentmuscle weakness: d/t loss of power from fxnal impairment

Question 4

multiple sclerosis

Answer 4

CNS issue: inflammation, demyelination, axon degen - demyelinating – acquired (inflammation)- dysmyelinating – Genetic (abnormal myelin formation) Chronic activation of T-cells, B-cells, & macrophagesParesthesia (45%), Motor weakness (27%), Gait/balance disturbance (35%), Vision loss/optic neuritis (17%), diplopia/vertigo (13%)

Question 5

multiple sclerosis s/s

Answer 5

Paresthesia (45%), Motor weakness (27%), Gait/balance disturbance (35%), Vision loss/optic neuritis (17%), diplopia/vertigo (13%)

Question 6

Internuclear Opthalmoplegia

Answer 6

MULTIPLE SCLEROSISInternuclear Opthalmoplegia – delay resp in looking, one eye goes external field one stays middle (upper neuron issue)

Question 7

Marcus Gunn pupil

Answer 7

MULTIPLE SCLEROSISshoot a little bit of air into the pupil, either no rxn, sluggish rxn, or inappropriate rxn d/t CN lesions

Question 8

Guillain-Barre Syndrome

Answer 8

progressive, symmetrical muscle weakness- onset days away from event* paresthesia in hands, feet dx: nerve conduction study (gold standard*), IgG ab to GQ1b- progressive weakness 1+ limb- areflexiarecover 2-4 wks after progression stops

Question 9

gold standard for guillain-barre syndrome dx

Answer 9

nerve conduction study

Question 10

guillain-barre syndrome tx

Answer 10

Supportive care – resp (may need intubation), autonomic support d/t brainstem, pain (neuropathic or disuse syndrome), bowel & bladder & rehab – big deal: lose much muscle strengthPlasma exchange/PlasmapheresisIVIG

Question 11

amyotrophic lateral sclerosis (ALS)

Answer 11

progressive, presently incurable, neurodegenerative disorderMotor neuron degeneration → deathCortical motor cell loss w retrograde axonal lossGliosis replaces lost neuronsInitial Presentation: body segment, asymmetric weakness

Question 12

myasthenia gravis

Answer 12

fluctuating variable weakness of ocular, bulbar, thoracic, & lumbosacral muscles, T-cell mediated attack atACTH receptors/proteins- auto-ab against AChR- T-cell against proteins @ post-syn membrane of NMJ

Question 13

myasthenia crisis

Answer 13

Acute Respiratory Failure – cholinergic crisis (Anticholinergic OD)Evaluation – respiratory status, secretion/airway mgmt, work of breathing, impaired vital capacity Rapid Treatment – Plasmapheresis, IVIGImmunosuppressants – glucocorticoids, Mycophenolate, azathioprine, cyclosporine

Question 14

myasthenia dx

Answer 14

tensilon, ice pack, electrophysiologic

Question 15

MG tx

Answer 15

Symptomatic treatments: anticholinesterase agents → Pyridostigmine bromide, NeostigmineChronic immunomodulating treatments: glucocorticoids & other immunosuppressive drugs Plasmapheresis, IVIG, Surgical treatment - thymectomy

Question 16

Lambert-Eaton Myasthenic Syndrome

Answer 16

Uncommon NMS jxn disorder- primary sx muscle weakness: symmetrical involvement of proximal muscles, legs/depressed or absent DTRs/Xerostomia- disease assoc: malignancy↓ ACh release from presynaptic nerve terminalsAntibodies against VGCC result in abnormal Ca flux needed to release ACh

Question 17

Duchene Muscular Dystrophy

Answer 17

Defective X chromosome producing dystrophinEarly onset