Case 11: Duchenne Muscular Dystrophy Flashcards
Creatine Kinase
Converts ADP to ATP
Higher concentrations in brain / striated muscle / other tissues that rapidly generate ATP
Gowers’ Sign
Starting w/ hands on floor
Placing hands on knees and hyperextending knees while pushing on thighs
Compensating for hip extension weakness
Hypercapnia
Excess CO2 in blood
Usually results from lung disease / hypoventilation / impaired consciousness
Pseudohypertrophy
Increase in size of muscle that is NOT due to increase in size of individual skeletal muscle fibers
Muscles replaced by fibrous tissue and fat in DMD
Transaminase
Elevated levels in the blood may indicate liver dysfunction
Precautions During PT (Patients with DMD)
No resisted or forceful ROM to extremities or trunk due to high fracture risk and damage to muscles
Assistance with WB activities required during transfers to decrease risk of fall or injury
Close monitoring of skin when wearing orthoses
Muscular Dystrophy (Definition)
Predictable pattern of muscle group deterioration that leads to atrophy / progressive weakness / deformity / contracture / progressive disability
Individuals with DMD lack Dystrophin (muscle protein) - causes muscle cells to be easily damaged
The presence of what two things in males may suggest DMD?
Gowers’ Sign
Pseudohypertrophy (common in calf muscle)
What does research state regarding glucocorticoid usage in relation to DMD?
The earlier the glucocorticoid was initiated, the more sustained the neuromuscular function
Death in persons with DMD usually results from ___.
respiratory insufficiency
Egen Klassification Scale (EK Scale)
Measures functional ability for non-ambulatory individuals with DMD
Using / transferring from / balance in WC, stand, move arms, use hands / arms for eating, turning in bed, cough, speak, physical well being
Predicts need for assisted ventilation in conjunction with Forced Vital Capacity
5 Stages of DMD
Presymptomatic
Early Ambulatory
Late Ambulatory
Early Non-Ambulatory
Late Non-Ambulatory
Glossopharyngeal Breathing
“Frog Breathing”
Gulping air repeatedly to increase air volume into lower airways
What is the most common muscular dystrophy and muscle disease of childhood?
Duchenne Muscular Dystrophy
What is the typical life expectancy of patients with DMD?
20-30 years
Gait Characteristics (Patients with DMD)
Wide BOS
Lumbar lordosis
Knee hyperextension
Toe walking
When is ambulation typically/often lost in patients with DMD?
By 12 years of age
What spinal deformity is associated with DMD? What delays the development of this / when is surgical treatment considered?
Scoliosis
Surgery recommended when curve reaches 30 degrees (esp. if child is under 14)
Prevented by prolonged walking and standing
What pattern of involvement is associated with DMD?
Proximal to distal pattern
Neck flexors / abs (early)
Progresses to pelvic girdle (hip extensors and abductors) as well as knee extensors
Progresses eventually to distal muscles of upper and lower limbs
DMD vs. ITW
DMD associated with sudden trouble with walking
ITW involves walking on toes that stopped and returned
