Myasthenia Gravis Flashcards
Myasthenia Gravis
is an autoimmune disease of the neuromuscular junction marked by fluctuating weakness of certain skeletal muscles that worsens with activity.
Etiology
is caused by an autoimmune process in which antibodies attack and destroy ACh receptors
Clinical Manifestations
The key feature of MG is fluctuating weakness of skeletal muscles
Muscles engaged in repetitive movements are affected the most
This includes muscles used to move the eyes and eyelids, chew, swallow, speak, and breathe.
Muscles are often strongest in the morning.
A period of rest usually restores strength
ocular muscles
the first muscles involved causing ptosis (drooping of the eyelids) in 1 or both eyes and double vision
Complications
difficulty chewing and swallowing food.
Speech is affected. The voice often is hoarse or fades after long conversations.
The muscles of the trunk and limbs easily fatigue.
Of these, the proximal muscles of the neck, shoulder, and hip are more often affected than the distal muscles.
Drugs that can worse MG
β-adrenergic blockers, calcium channel blockers, lithium, quinidine, and aminoglycoside antibiotics
Myasthenic crisis
is an acute worsening of muscle weakness triggered by respiratory infection, surgery, stress, pregnancy, exposure to certain drugs, or starting corticosteroid treatment.
major complications of a myasthenic crisis
results from muscle weakness in areas that affect swallowing and breathing.
This can result in aspiration or respiratory insufficiency.
The patient may need a ventilator or noninvasive respiratory support (BiPAP).
Diagnostic Studies
history and physical assessment
Single-fiber EMG is highly sensitive in confirming MG.
edrophonium test - rapid improvement after administration of edrophonium indicates MG
Atropine
cholinergic antagonist, should be readily available to counteract the effects of edrophonium when it is used diagnostically.
CT scan
may be done to evaluate the thymus
Drug Therapy
anticholinesterase drugs, alternate-day corticosteroids, and immunosuppressants
Pyridostigmine (Mestinon)
the best drug of this group for the long-term treatment of MG.
cholinergic crisis
involuntary muscle contraction, extreme weakness, flaccid muscle paralysis, sweating, excessive salivation, diarrhea, and constricted pupils.
In a cholinergic crisis,
giving edrophonium increases muscle weakness. The use of corticosteroids in addition to edrophonium have reduced the incidence of cholinergic crisis.