exam 1 practice questions pt.2

What are the 4 types of muscular dystrophies?

Duchenne (DMD), Becker (BMD), Facioscapulohumeral (FSHD), Limb-girdle (LGMD)

What are the 2 inflammatory myopathies?

Polymyosisitis (PM) and dermatomyositis (DM)

How are muscular dystrophies defined?

Genetic disorders that cause progressive muscle degeneration

What is the protein that is typically absent or structurally abnormal in individuals with muscular dystrophy?

Dystrophin

In individuals with abnormal dystrophin, damaged muscle fibers become replaced by what?

Fat and connective tissue

What are the common signs of Duchenne Muscular Dystrophy (DMD)?

Calf pseudohypertrophy, Gower’s maneuver, scoliosis

What is the diagnostic standard for Duchenne Muscular Dystrophy?

Genetic testing (X-linked condition)

While corticosteroids can slow functional decline and delay loss of ambulation in individuals with DMD, it does not…

Prevent wheelchair dependence

Which muscular dystrophy is associated with a “Beevor” sign?

Facioscapulohumeral Muscular Dystropy

In DMD treatment, early _______ prevention is important

contracture

Individuals with DMD should not partake in _____ resistance exercises

maximal

What is the difference in time of onset between DMD and BMD (Becker Muscular Dystrophy)

DMD typically onsets between 2-6 years. BMD onsets later; typically during adolescence or adulthood

How does dystrophin differ between DMD and BMD?

In DMD, dystrophin is absent or severely dysfunctional. In BMD, dystrophin is present but abnormal or reduced.

What is a common sign of muscle breakdown in individuals with muscular dystrophies?

Dark urine after intense activity

What is the typical presentation of facioscapulohumeral muscular dystrophy? (FSHD)

Asymmetrical weakness of the face, scapular stabilizers, and upper arms. Sensation and bowel/bladder are typically preserved

What are some “clinical clues” for FSHD?

Unable to whistle, unable to close the eyes completely, positive Beevor Sign

Which functional problems are the same between FSHD and Limb-Girdle?

Difficulty rising from a chair, negotiating stairs, and reaching overhead

How does weakness typically present in Limb-Girdle Muscular Dystrophy (LGMD)?

Symmetrical, progressive weakness of the hips and shoulder girdles.

Which outcome measure can be a marker of impending loss of independent ambulation in DMD?

Four-stair climb

In inflammatory myopathies, typical weakness is where?

Proximal areas: neck, shoulders, hips

What findings are specific to dermatomyositis?

Characteristic skin findings in addition to the typical weakness

How can we distinguish between critical illness myopathy and critical illness polyneuropathy?

Critical illness myopathy has preserved sensory nerve action potentials

What are the 3 variables for consideration when determining whether an individual is in the basic, intermediate, or complex level of wheelchair needs?

Balance and postural control in sitting, the ability to achieve a neutral sitting posture, the degree of postural support needed to achieve a neutral posture

Alignment of the ______ takes priority over alignment of other body segments

pelvis

Which wheelchair type would be most useful for an indiviual post-stroke who may need to wheel themselves with 1 arm and 1 leg? Or an individual who requires wheeling with their feet only?

K002 - lower seat to floor height

Which wheelchair type allows for camber and what purpose does it serve?

K005 - tilts the wheels so that the BOS is wider and more stable. Good for more athletic chairs

What type of patient population is a K005 chair intended for?

Full-time wheelchair users

What is the primary difference between a group 2 power chair and a group 3 power chair?

Group 2 power chair does not include power seating

Why is it often difficult to justify a power scooter to insurance?

The large turning radius makes it difficult to use in a home, and insurance will only pay for a device if the patient requires it in the home for MRADLs

Which cushion type is the best for pressure relief?

Air cushion

Which patient type is likely to require custom-molded seating?

Patients with severe postural deformities, scoliosis, pelvic obliquities, windswept, contractures

What is the requirement for a patient to qualify for custom molded seating?

They must have failed use of a prefabricated option