MD and DCD Flashcards
What is MD? (3)
Hereditary progressive disorder
Most common serious mm disease
MM weakness from birth to late childhood (skeletal mm)
Dystrophin role
Dystrophin
- part of sarcolemmal prtns (dystrophin glycoprotein)
- Mechanical stability for surface membrane of skeletal mm fibres and some synaptic plasticity in brain
Pathophysiology of Dystrophin
Lack leads to segmental necrosis (trunk and legs more prone)
Progressive mm fibre loss with fibrous and adipose tissue replacement
Describe Duchenne type (general - 3; impariments)
Most common
X-linked
Late teens to 30s
Primary impariment = progressive weakness after myofibril loss (evident by 3-5 yrs); often intellectual impairment
Secondary impairments = contractures, respiratory infections, fatigue, obesity
DMD - infancy to preschool (general - 4; posture - 2)
General
- no significant functional impairment
- 50% walking delay (18 months)
- positive Gowers from 3yrs
- no ROM limits before 5yrs
Posture
- may have weak neck flexors (seen with reduced head control)
- lordotic (weak gluteals) + scapulae winging
What family education for DMD in infacy? (3)
Activity
- avoid fatigue and maintain strength
- social activities + improve fn (swimming, bike riding)
- promote respiratory fn
DMD symptoms - Early school age (general, gait, other)
General:
- clumsiness, falling, atypical gait, waddling
- Gowers after 1 trial of floor to stand
- pseudohypertrophy of calves (fat, connective tissue)
- mm weakness in neck flxors, abs, interscapular, hip extn
Gait
- increased BOS
- lateral trunk sway
- toe walking
- lack of reciprocal arm swing
Other
- pumonary impairment begins
- fatigue
Physio for DMD (early school age) - General (2), Assessment (3), Rx (3)
General
- Community mobility limited (Gower’s to get up stairs)
- Possibly use graded resistance exercise?
Assessment
- Manual mm testing by 6-8yrs
- Respiratory function (chest wall excursion, cough, spirometry)
- Document impairment and progression
Rx
- submax abs, hip extn/abd, knee extns
- cycling and swimming encouraged
- breathing exercises
DMD symptoms at school age (6)
Symptoms
- increased falls
- general mm weakness
- more fatigue (walking)
- calf contracture
- intoeing
- pulmonary impairment
Physio for DMD at school age
Positioning and ROM
- For calves and TFL then hip flexors
- – 1-2 daily; 1-10 reps
- prone positioning with feet off end of mattress
- Monitor scoliosis
- w/c community mobility
DMD in adolescence (general, mobility)
General
- progression of disability
- consider Sx and orthoses
Mobility
- lose walking
- T/Fs and ADLs difficult
How to predict termination of walking (adolescence) (4)
Falls
Not getting up from floor
MM strength reduced by 50%
Knee extn lag in sitting
Physio for DMD in adolescence (Rx, equipment)
Rx
- Continued mm and joint ROM
- Prone lying
- Knee capsule
- T/Fs
- Scoliosis
Equipment
- Orthoses
- Power w/c
Define DCD (4) and other relevant factors (2)
- Daily activity coordination is substantially below expected for chronological age
- This disturbance affects academic and ADLs
- Not due to a general medical condition and not a developmental disorder
- If mental retardation, motor is in excess of normal
Other
- difficulties from early childhood
- may have Hx of perinatal complications (prematurity or VLBW)
DCD symptoms (movements - 3; general - 2)
Movements
- Not fluid/poorly coordinated
- Poor rhythm/timing of mm
- Poor sequencing of tasks
General
- slower responses
- difficulty identifying important details of/analysing/execution a task