Transcript Slide 1

Disorders characterized by early
onset and impaired movement and
posture. It is a non-progressive
disease.
 1.9-2.3
in every 1000 live births
in prevalence since the 1960’s due largely
to the improved survival of VLBW infants.

 Greatest
prevalence is seen in prematurely
delivered infants.
 Formerly
thought to be R/T perinatal birth
asphyxia, but now it is known that CP more
commonly results from existing prenatal
brain abnormalities.
 24%
of cases have no identifiable cause.
 Difficult
to determine exactly
 ANOXIA
is most significant factor to cause
pathologic brain damage. This is often 2ndary
to other etiology.
 The
area of the lesion in the brain mostly
determines the subsequent pathology.
•May involve one or both
sides.
•Hypertonicity with poor
control of posture, balance, &
•usually caused by hypoxic coordinated motion
infarction in the area
•Impairment of fine and gross
adjacent to the lateral
motor skill
ventricles.
•Active attempts at motion
•Upper motor neuron type increase abnormal postures
of muscular weakness.
and overflow of movement to
•See Box 40-3 (p.1693) for other parts of the body
Types of Spastic Cerebral
Palsy
Spastic:
MOST COMMON
Dyskinetic/
athetoid—
•Athetosis— slow, wormlike writhing
movements that usually involve the
extremities, trunk neck, facial muscles,
and tongue
•Abnormal involuntary
•Involvement of the pharyngeal,
movement
laryngeal, and oral muscles causes
drooling and dysarthria (imperfect speech
•Caused by kernicterus & articulation)
hemolytic disease of the
•Involuntary movements may take on
newborn which leads to
choreoid (involuntary, irregular, jerking
pigment deposits in the
movements) and dystonic (disordered
basal ganglia & some
muscle tone) manifestations that increase
cranial nerve nuclei.
in intensity with emotional stress and
around adolescence.
Ataxic
•Wide-based gait
•Rapid, repetitive movements
performed poorly
•Disintegration of movements of the
upper of the upper extremities when
the child reaches for objects
Mixed Type/
dystonic
•Combination of spasticity and
athetosis
 Delayed
gross motor development—
universal manifestation of CP

Especially significant if other developmental
behaviors e.g. speech & personal social are normal
 Abnormal
motor performance—
Early sign is preferential unilateral hand use
that may be apparent at ~6months of age.
May stand or walk on toes
 Alterations


 or  resistance to passive movements is a sign of
abnormal muscle tone.
Child may exhibit opisthotonic postures and stiffness
on handling, dressing, or diapering.
 Abnormal


of Muscle Tone—
Postures—
From an early age, a child lying in a prone position
will maintain the hips higher than the trunk with the
legs and arms flexed or drawn under the body.
Spasticity may be mild or severe.


Reflex abnormalities—
 Persistence of primitive infantile reflexes is one of the
earliest clues to CP.
Associated Disabilities and problems—
 Intellectual impairment—possible, but 70% are WNL
 ADHD—poor attention span, marked distractibility,
hyperactive behavior,and defects of integration
 Seizures—most common in postnatal acquired hemiplegia
 Drooling, feeding and speech needs, risk of aspiration &
possible inadequate gas exchange.
 Orthopedic complications
 Constipation
 Dental caries, malocclusion, gingivitis
 Nystagmus, amblyopia & hearing loss
 Neurologic
Examination & History are the
primary modalities for diagnosis
 Recognizing
etiologic factors that put the
infant at risk is critical in the assessment and
diagnostic process.

Broad aims:
1. Establish locomotion, communication, and selfhelp
2. Gain optimum appearance & integration of
motor functions
3. Correct associated defects as effectively as
possible
4. Provide educational opportunities adapted to
the needs and capabilities of the individual child
5. Promote socialization experiences with other
affected and unaffected children.
Mobilizing devices—braces, crutches, wheelchairs,
walkers
 Surgery—when spasticity causes further deformities
 Medication—drugs to  spasticity are often NOT
helpful in CP. Antianxiety meds may help child with
athetosis.Skeletal muscle relaxants e.g. baclofen,
methocarbamol (Robaxim), or dantrolene
(Dantrium) & Valium may help short-term for older
children & adolescents. Antiepileptic meds, e.g.
phenobarbital & phenytoin are used routinely for
children with seizures & CP.

 Technical
aids—e.g. electromechanical toys
that use biofeedback; microcomputers
combined with voice synthesizers, or activated
with a head-stick, tongue,or other voluntary
muscle movement over which the child has
control.
 Other Considerations—care of vision & hearing
deficits as well as dental care is essential early
on.
 Physical
Therapy—one of the most commonly
used conservative tx modalites. Involves PT,
family, and nsg
 Functional & Adaptive Training (Occupational
Therapy)—training in manual skills and ADL’s must
be started early
 Speech Therapy—start early to prevent speech
problems.
 Education—individualize to the needs of the child
 Recreation—sports, physical fitness, & recreation
programs are encouraged for children with CP




Reinforce therapeutic plan/assist in Normalization
Address Health Maintenance needs
 Watch for fatigue, nutritional needs, safety needs
Support family
 Help them cope with the emotional aspects of the disorder
 Make appropriate referrals to support groups e.g United
Cerebral Palsy Association.
 http://cerebralpalsy.org/
Support hospitalized child—
 the nurse’s actions should convey acceptance, affection,
and friendliness and promote a feeling of trust and
dependability.
Gradual degeneration occurs in muscle
fibers progressive weakness and
symmetric wasting away of skeletal muscle
 Pseudohypertophic




(Duchenne)
X-linked Recessive
1-3 years of age
Lordosis, Waddling gait
Rapid progression—



Death 15-25 after onset
Website Part 1 with newest Guidelines from MDA—12/09
Part 2 with newest multidisciplinary guidelines from MDA—1209: Bushby, K., Finkel, R., Birnkrant, D. J., Case, L. E.,
Clemens, P. R., Cripe, L., & ... Constantin, C. (2010).
Diagnosis and management of Duchenne muscular dystrophy,
part 2: implementation of multidisciplinary care. Lancet
Neurology, 9(2), 177-189. doi:10.1016/S1474-4422(09)70271-6
 Onset
after 8 y/o
 Weakness of proximal muscles of pelvic and
shoulder girdle
 Slow progression 


Incapacitated 20 years after onset
OR slight disability
 Early
adolescence
 Symptoms



Lack of facial mobility
Can’t raise arms over head
Shoulders slope forward
 VERY
SLOW PROGRESSION
 Serum
Creatinine Phosphokinase (CPK)
 Electromyography (EMG)
 Muscle Biopsy
 Supportive
 Physical
Therapy
 Orthopedic Trx (casting, bracing,
surgery) to minimize deformities and
maintain ability to perform ADL’s
 Most
severe + most common type
 X-linked recessive

Inherited MOTHER carrier/Son Symptoms
 Genetic
protein
mutation—ABSENT skeletal muscle
 Muscle
weakness by 3 y/o
 Hx delayed motor development
 Abnormal Gait, Waddling
 Falls Frequently
 Marked Lordosis when standing
 Gower’s Sign
 Enlarged
calves, upper arms, thighs 
fatty infiltration of muscle 
pseudohypertrophic
 Contractures
 12 y/o = unable to walk
 Weakened respiratory muscles
 Death
 Contracture
Deformities
 Atrophy
 Trx
PROM & AROM
 Casting/Bracing
 Rigid Corset
 Frequent Rest

 PT
3 hrs of
ambulaton/day
 Infections
d/t
decreased vital
capacity and atrophy
of resp muscles
 Obesity
d/t
overfeeding and
decreased activity
 Antibiotics
 Resp.
Trx
 Chest Physiotherapy
 Diet
 Recreation
as tol.
 Maintain mobility as
long as possible
 D/T
Weakening of
Cardiac Muscle
 Treatment


Digoxin
Diuretics e.g
furosemide
 Serum



Enzymes
Creatinine Phosphokinase
Aldoase
Glutamic-oxaloacetic transaminase (SGOT)
 Very
high levels in 1st 2 years of life
 Levels decrease as muscle deteriorates
 WNL when severe wasting and disability
 Muscle
Biopsy
Degeneration of muscle
fibers
 Fatty deposits
 Fibrosis

 EMG

Diminished duration
and amplitude of
existing motor unit
potentials
 Help
maintain independence
 Continual evaluation of capabilities
 Home Assessment


Set-up w/c assessible?, wide doors?, etc
Buying clothes
 Respite
Care
 Family Involvement
 Genetic Counseling