ALS

Question 1

Manifestations of ALS

Answer 1

Progressive weakness and atrophy of muscle
Cramps, twitching and lack of coordination
Spasticity, deep tendon reflex – brisk and overactive
Difficulty speaking, swallowing, breathing

Question 2

ALS Management

Answer 2

No cure
Maintain or improve function, well-being, and QOL
Riluzole – glutamate antagonist
Prolong life for 3-6m
Most patients managed at home or in community
Common reasons for hospitalization
Malnutrition
Pneumonia
Respiratory failure
Mechanical ventilation may be needed for alveolar hypoventilation

Question 3

Myasthenia Gravis

Answer 3

Autoimmune disorder affecting the neuromuscular junction

Antibodies attack acetylcholine (ACh) receptors and impair transmission of impulses

Prevents ACh molecules from attaching to receptors and stimulating muscle contractions

Question 4

Myasthenia Gravis Manifestations

Answer 4

Fluctuating weakness of skeletal muscles
Initial symptoms: ocular muscle – diplopia and ptsosis
Weakness of facial muscles, swallowing and voice impairment (dysphonia)
Generalized weakness

Question 5

Medical Management of MG

Answer 5

Directed at improving function and removing circulating antibodies
Pharm:
Anticholinesterase meds
Pyridostigmine
Immunosuppresive therapy
IV immune globulin (IVIG)
Plasmapheresis
Thymectomy: some studies show thymus is related to MG

Question 6

Myasthenic Crisis

Answer 6

Disease exacerbation or precipitating event most commonly a respiratory infection
Severe generalized muscle weakness in areas that affect swallowing and breathing
May develop respiratory compromise and failure 🡪 mechanical ventilation

Question 7

Guillain-Barré Def

Answer 7

Autoimmune disorder
Occurs a few days or weeks after a viral or bacterial infection
Acute inflammatory polyneuropathy
Rapid nerve demyelination results in 
Respiratory failure
Autonomic nervous system dysfunction
CV instability 
Acute, ascending, rapidly progressive, symmetric weakness of the limbs

Question 8

Clinical Manifestations of GB

Answer 8

Starts with lower extremities and progresses upwards
Weakness or paralysis
Paresthesia and pain
Diminished or absent reflexes
Bulbar weakness
Cranial nerve symptoms
Bradycardia or tachycardia
Hypotension or hypertension

Question 9

Interprofessional Care of GB

Answer 9

Requires intensive care management
Continuous monitoring with respiratory support
Plasmapheresis and IVIG
Reduce circulating antibodies
Recovery rates vary, but most patients recover completely; takes months to years