Demyelinating, myopathic and neuromuscular Dz

Question 1

-Onset 1-5yrs, muscle weakness distributed 1st) pelvis, shoulder girdles, 2nd) limbs and respiratory muscles. Intellectual retardation. -Prednisone for muscle strength.

Answer 1

Muscular Dystrophy-Duchenne Type

Question 2

-Demyelination and axonal damage -Relapsing-remiting disease w/ symptoms coming on over hours to days and then remitting. Fatigue, weakness/numbness/parathesias, spastic paraparesis, optic atrophy, -Must have separation in time (1mo) and space -Corticosteroids for acute relapses Women>men

Answer 2

MS

Question 3

-Progressive degeneration of motor neurons -Upper and lower motor neuron degeneration. 30-60yrs. Difficulty swalloing, gait abnormalites, muscle weakness, cramps -Riluzole

Answer 3

Amyotrophic Lateral Sclerosis (ALS)

Question 4

-Fluctuating weakness of voluntary muscles. Increased weakness with activity. Diplopia, ptosis, difficulty swallowing. -Autoimmune -Serum ACh receptor antibodies -Anticholinesterases for symptoms; IVIG, plasma exchange,

Answer 4

Myasthenia Gravis

Question 5

-Primarily affects motor nuclei of cranial nerves—>drooling, decreased gag reflex, pooling of saliva, weak cough, wasted fasciculation tonuge

Answer 5

Progressive bulbar palsy