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Flashcards in neuromuscular disease Deck (17)


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


muscle weakness

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


asthenia vs muscle weakness

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


multiple sclerosis

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%)


multiple sclerosis s/s

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


Internuclear Opthalmoplegia

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


Marcus Gunn pupil

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


Guillain-Barre Syndrome

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


gold standard for guillain-barre syndrome dx

nerve conduction study


guillain-barre syndrome tx

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


amyotrophic lateral sclerosis (ALS)

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


myasthenia gravis

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


myasthenia crisis

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


myasthenia dx

tensilon, ice pack, electrophysiologic


MG tx

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


Lambert-Eaton Myasthenic Syndrome

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


Duchene Muscular Dystrophy

Defective X chromosome producing dystrophinEarly onset