Common Neuromuscular

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Common Neuromuscular Dysfunctions in
Children PSUEDOTROPHIC DUCHENE MUSCULAR
DYSTROPHY CEREBRAL PALSY
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• PSUEDOTROPHIC DUCHENE MUSCULAR DYSTROPHY
• Muscle disease with progressive degeneration of
  muscle fibers
• Absence of dystrophin protein in skeletal
  muscle
• X linked recessive inheritance
• ? serum creatine phosphokinase SGOT seen
  before weakness appears

Diagnosis electromyography and muscle biopsy

• Assessment findings
• delayed motor development
• difficulty rising (Gower sign)
• waddling gait
• Lordosis
• muscular enlargement

• Management
• Complex - Supportive
• Maintain motor function
• -PT
• -Surgery to release contracture
• -Bracing
• -Self-help skills
• Counsel about genetic
• transmission
• Facilitate family coping
• Promote self-esteem

• Complications
• Contracture deformities
• Disuse atrophy
• Infections
• Obesity
• Cardiopulmonary problems

Continue or Quiz Me
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• Question 1
• A five year old boy has just been
• diagnosed with pseudohypertrophic
• Duchene Muscular Dystrophy. His care
• should include which of the following?
• Recommend genetic counseling.
• Explain that the disease is easily treated.
• Suggest ways to limit use of muscles.
• Assist family in finding a nursing facility to
  provide his care.

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CORRECT Duchene Muscular Dystrophy is an
X-linked recessive inherited disease. Parents
should be counseled about the possibility of
having other children with the disease. Next
Question Next Topic
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INCORRECT Duchene Muscular Dystrophy is a
progressive degenerative disease with no cure.
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INCORRECT The primary goal of treatment for
Duchene Muscular Dystrophy is to maximize
function of muscles as long as possible, so
intense physical and occupational therapy are
needed.
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INCORRECT The onset of Duchene Muscular Dystrophy
is typically early school-age and loss of muscle
function occurs slowly over many years. Children
do not require nursing home care until late
stages and only if parents are unable to cope and
care for the child.
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Question 2 Duchene Muscular Dystrophy
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CEREBRAL PALSY Nonprogressive neuromuscular
dysfunction characterized by impaired movement
and posture. Associated with prenatal, perinatal,
and postnatal hypoxia or trauma.
Major Types Spastic hypertonicity with poor
control of posture, balance and coordinated
motion impaired fine and gross motor
skills Dyskinetic athetosis--writhing movements
of extremities, trunk, neck, facial muscles,
tongue Drooling, dysarthria. Jerking
movement Ataxic wide-based gait poor
performance for rapid repetitive movements,
unable to control movements when reaching for
objects

• Assessments
• Delayed gross motor
• development performance
• Alteration of muscle tone
• Abnormal postures
• Reflex abnormalities
• Other disabilities problems

• Management
• Promote optimum growth/development
• Surgery to correct orthopedic defects
• Mobilizing devices
• Voice Synthesizers
• Exercises to stretch muscles
• Jaw control to facilitate eating
• Taught signing

Continue or Quiz Me
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Question 1 Spastic cerebral palsy is
characterized by which of the following? a.
Wide-based gait poor performance of rapid,
repetitive movements b. Athetosis dystonic
movements c. Hypertonicity poor control of
posture, balance, and coordinated motion d.
Tremors lack of active movement
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CORRECT Spastic Cerebral Palsy is characterized
by impaired fine and gross motor skills that
affect posture, balance and coordinated
motion. Next Question Next
Topic
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INCORRECT Wide base gait and difficulty with
rapid repetetive movement are characteristic of
Ataxic Cerebral Palsy
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INCORRECT Writhing athetotic movements are
characteristic of Dyskinetic Cerebral Palsy
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INCORRECT Tremors are not characteristic of
Spastic Cerbral Palsy. Also, the child is able to
move but movement is uncoordinated.
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Question 2
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FRACTURES IN CHILDREN