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Muscular dystrophy
Muscular dystrophy is a collective
term referring to a group of
inherited neuromuscular diseases
characterized by progressive
muscle weakness due to various
mutations in several genes
involved in muscle structure and
function.
MUSCULAR DYSTROPHY is distinguished
from other diseases by 4 criteria:
Primary dystrophy
Genetic basis for disorder
Course is progressive
Degeneration and death of muscle fibres occur at some stage of the disease.
Muscles affected are symmetrical
Sensory- not affected
Tendon reflexes are preserved till last stage
Complications
Respiratory Scoliosis
Contracture
s
Some children
become wheelchair
dependent bound by
9-10 years.
The progression:
Upto 7 years-
ambulatory
stage
Upto 12
years- wheel
chair
Upto 19-20
years- bed
bound
Rehabilitation
Ambulatory
stage:
Early detection & management of contractures
Positions encouraging contractures should be avoided.
Long leg sitting & prone lying are encouraged
Passive stretching daily
Splinting
Supportive physical therapy exercises: endurance exercise, respiratory training, occupational therapy
Monitoring and prevention of cardiac complications
Psychological support
Wheelchair
dependent
stage:
Respiratory management
Upper limb weakness : UL Orthosis
Adl independence training
Prevention of scoliosis
Stage of
prolonged
survival
Ventilation assistance
Facilitation of ADL independence
Communication aids as possible
DOWN’S SYNDROME
Downs syndrome is one of the common
cause of mental sub normality in children
Incidence : 1.2/1000 live births.
Cause : chromosomal abnormality called
Trisomy 21.
Clinical features
Skull- small face- flat
Delayed closure of fontanelles
Mental sub normality
Speech – delayed
Congenital malformations- present
Ligaments – lax & hypermobile
Muscles- hypotonic & motor
milestones- delayed
• Deformities: scoliosis, pes planus& subluxation of joints
Management
Positioning :
• Child prefers supine lying & sitting to avoid energy expenditure.
• Balance reactions – should be trained.
Sensory & proprioceptive re education:
• Strengthening towards motor milestonnes ,muscular activities
• ADL training, IQ assessment ,special education, behaviour
modifications.
Genetic counselling
Take history
Construct
pedigree
Establish
diagnosis
Counselling
to parents
Risk
estimation
Follow up