Muscular Dystrophy + Spinal Muscular Dystrophy Flashcards
General info on dystrophy and atrophy?
inherited disorders of motor unit or LMN
progressive nm diseases
death usually because of respiratory complication (no cure)
progressive loss of muscle contractility due to destruction of myofibrils
muscular dystrophy
dx by EMG/labs/biopsy
include low IQ
progressive*
What are the criteria for classification?
mode of inheritance age of onset rate of progression localization muscle changes any other organs involved?
What is gower movement?
what do you wanna do with pt who has that?
climbing up legs with arms
refer and treat.
2 types of dystrophy u gotta know and age of onset?
Duchenne –floppy(1-4 years)
Becker– 5-10 years
Rapidly progressive dystrophy
when is loss of walking and death?
Duchenne
8-14 yrs
late teens-3rd decade
slowly progressive dystrophy
what happens with walking?
lifespan?
Beckers Dystrophy
maintained thru early teens
die in 4-5th decade
Weight of DMD
and complications?
get fat then at 17 they lose all the weight..
complications of MS/pulm/scoliosis
usually die from respiratory so work on that.
specificityc impairments of DMD and where do contractures develop?
weak neck flexor/abs/scap/hip ext
achilles/ITband/hip flexor/hammy/elbow flexors and wrist
What happens in early phase duchenne?
2-6 yr til 6-10 yrs
a lot of Fx loss
Tx focuses on strength/coordination/flexibility/orthotics…
DO NOT USE FOOD AS REWARD
What happens in transitional phase of DMD?
6-12 yrs
muscle weakness/fx loss/postural adjustments.
Tx for activity w/o fatigue/ same as early/ begin respiratory ex/ modify home/ refer for scoliosis
Strength patterns of DMD?
Biceps>tris
Sup>pro
wrist ext> flex
DMD loss of ambulation phase?
8-14 yrs.
90% of time in chair.
want to maintain everything get some equipment and work with MD.
Adult phase of DMD?
15+ yrs
heart and muscle deteriorate (increased MI)
again just wanna maintain,
What are medical Tx for DMD?
steroids to increase amb/ decrease progression.
Molecular therapeutics: what every that means