Transcript Cerebral Palsy

Cerebral Palsy
ParalyticDisorders
POLIOMYELITIS
»
MYELOMENINGOCELE
ARTHROGRYPOSIS MULTIPLEX CONGENITA
BRACHIAL PLEXUS PALSY
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Cerebral Palsy
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ETIOLOGY
(1) Some degree of motor impairment is
present
(2) an insult to the developing brain has
occurred
(3) a neurological deficit is present that
is nonprogressive
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The insult to the brain is believed to
occur between the time of conception
and age 2 years, at which time a
significant amount of motor
development has already occurred.
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neurological deficit is
Permanent
Nonprogressive
.
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PREVALENCE:
amount and quality of prenatal care,
the socioeconomic condition of the parents,
the environment,
obstetrical and pediatric care the mother and
child receive.
prevalence = 0.6 to 7cases per 1000 live births
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prenatal,
perinatal,
postnatal.
Contrary to popular belief,
fewer than 10% of
injuries that result in cerebral palsy
occur during the birth process.
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prenatal
fetus
(most commonly genetic disorders),
mother (seizure disorders, mental retardation, and previous pregnancy loss),
pregnancy itself (Rh incompatibility, polyhydramnios, placental rupture, and
drug or alcohol exposure).
External factors, such as TORCH (toxoplasmosis, other agents,
rubella, cytomegalovirus, herpes simplex).
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perinatal
asphyxia or trauma that occurs during labor.
Oxytocin augmentation,
umbilical cord prolapse
breech presentation.
Low-birth-weight infants (<1500 g)
incidence of 60 per 1000
births compared with two per 1000 births in normal-weight infants.
periventricular blood vessels.
Pregnancies involving multiple births
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white matter
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postnatal
Hypoxic-ischemic encephalopathy,
persistent fetal circulation with true ischemia
meconium
aspiration
which is characterized by hypotonia, decreased
movement, and seizures
Infections such as encephalitis and meningitis
Traumatic brain injury
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Geographical Classification
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Monoplegia
very rare
usually occurs after
meningitis.
Most patients diagnosed with
monoplegia actually have
hemiplegia
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Diplegia
most common 50%
premature infants
intelligence normal
Most children with diplegia
walk eventually
although walking is delayed
usually until around age
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4 years
Hemiplegia
30%,
typically have sensory
changes
Hemiplegic patients also
may have a
leg-length discrepancy
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Quadriplegia
significant cognitive
deficiencies
that make care more difficult.
Head and neck control
which helps with communication,
education, and seating.
Treatment goals
straight spine level pelvis,
17 located mobile hips, plantigrade
feet that..
Total Body
profound cognitive deficits
head and neck control.
full-time assistance
for activities of daily living and
specialized seating systems to
assist with head positioning.
Drooling, dysarthria, and
dysphagia
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Paraplegia
is very rare
triplegia
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Physiological Classification
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different pathways of the brain are
myelinated at different times,
spastic diplegia
8 to 10 months of age;
hemiplegia,
20 months of age
athetoid cerebral palsy,
after 24 months of age.
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DIAGNOSIS
History and physical examination are the primary…
spastic paraparesis and congenital ataxia.
Ancillary studies, such as radiographs, hematological studies, .
Diagnosis of cerebral palsy before age 2 years can be very difficult.
Transient dystonia of prematurity is a condition characterized by
increased tone in the lower extremities between 4 and 14 months
old and often is confused with cerebral palsy.
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DIAGNOSIS
Knowledge of normal motor developmental milestones and primitive reflexes
allows identification of children who are delayed in their motor
development.
Motor development usually occurs in a cephalad-to-caudal pattern, starting with
swallowing and sucking, which are present at birth, and proceeding to
sphincter control, which occurs at 24 to 36 months of age.
Primitive reflex patterns of motor activity that are outgrown as
part of the normal maturation process persist longer than normal and in
some cases permanently in children with cerebral palsy.
Neurological quotient can be determined, which is useful in
determining prognosis and treatment.
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Prognostic Factors
If a child has not learned to walk by age 8 years, and he or
she is not limited by severe contractures, it is unlikely he
or she will ever walk at all.
sitting independently before age 2 years was not a good
predictor of ultimate ambulatory ability, but the inability to
sit independently by 4 years predicted nonambulation
Bleck used the presence or absence of primitive reflexes to
determine prognosis for ambulation for children with
cerebral palsy
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ASSOCIATED CONDITIONS
In one study, adults with cerebral palsy ranked what was most important to them:
education and communication
activities of daily living
Mobility
Ambulation
Because of the often complex nature of these
conditions, a multidisciplinary team approach to
patients with cerebral palsy is essential.
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ASSOCIATED CONDITIONS
mental impairment or learning disability (40%);
seizures (30%);
complex movement disorders (20%);
visual impairment (16%);
malnutrition and related conditions (15%)
hydrocephalus (14%).
Bulbar involvement can lead to drooling, dysphagia, and speech difficulties, which can
limit cognitive and social development further.
Many children with cerebral palsy (50% in some series) have significant visual difficulties,
with 7% having a severe visual defect. Common visual disturbances include myopia,
amblyopia, strabismus, visual field defects, and cortical blindness.
Hearing loss has been reported to occur in 10% to 25% of children with cerebral palsy,
which can exacerbate communication and learning difficulties further.
Hearing screenings, similar to visual screenings, should be part of the routine evaluation
of patients with cerebral palsy.
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ASSOCIATED CONDITIONS
Seizures(30%),
most commonly patients with :
hemiplegia,
quadriplegia,
postnatally acquired syndromes.
Osteopenia with increased risk of fracture also is
common in children with cerebral palsy, especially
children who are more severely affected. The
nonoperative and operative treatment of these fractures
Bisphosphonates have been shown in small studies
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ASSOCIATED CONDITIONS
Severe medical problems, such as aspiration pneumonia and profound feeding
problems, can lead to malnutrition, immune suppression, and metabolic
abnormalities.
Gastroesophageal reflux often can be managed medically and with positioning, but
fundoplication may be necessary. Enteral feeding augmentation often is necessary
because of swallowing dysfunction and the risk of aspiration pneumonia. This can be
done with a gastrostomy or jejunostomy tube.
Emotional problems add to these associated conditions. The child's self-image plays
an important role, especially in adolescence, when the differences between the
affected child and peers become more apparent.
Communication difficulties also may affect self-image at this stage.
As young adulthood is reached, concerns about employment, self-care, sexual function,
marriage, childbearing, and caring for aging parents may become emotional
stressors.
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TREATMENT
a multidisciplinary team approach
Four basic treatment principles exist.
1- cp is nonprogressive, the deformities are progressive.
2-treatments currently available correct the secondary deformities only
and not the primary problem.
3-deformities typically become worse during times of rapid growth.
4-operative or nonoperative treatment should be done to minimize the
impact it has on the patient's socialization and education.
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TREATMENT
Nonoperative
splinting and bracing
physical therapy,
most common agents =diazepam and baclofen, which act centrally,
dantrolene, which acts at the level of skeletal muscle.
Baclofen
mimics the action of g-aminobutyric acid, a powerful inhibitory
neurotransmitter centrally and peripherally, whereas diazepam potentiates
the activity of g-aminobutyric acid.
Dantrolene acts at the level of skeletal muscle
develop profound weakness, hepatotoxicity
New trend intrathecal baclofen and intramuscular botulinum toxin injections
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TREATMENT
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TREATMENT
Botulinum toxin
a potent neurotoxin, of which there are seven serotypes, produced by
..Clostridium botulinum
Contraindications
BTX-A therapy include known resistance or antibodies, fixed deformity or
contracture, concurrent use of aminoglycoside antibiotics, failure of previous
response, and certain neurological conditions such as myasthenia gravis
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TREATMENT
Physical therapy
The therapist plays a crucial role in all aspects of care, including identifying children who
may have cerebral palsy, treating their spasticity and contractures, fabricating splints
and simple braces, providing family education and follow-up, acting as a liaison with
the school and other health care providers, and implementing home stretching and
exercise programs with the patients and their families.
Bracing,
The most commonly used braces for the treatment of cerebral palsy include ankle-foot
orthoses, hip abduction braces, hand and wrist splints, and spinal braces or jackets. A
patient-centered approach should be used.
The goals of bracing for an ambulatory child differ from the goals for a child with severe
involvement. Bracing of the lower extremities, most commonly with ankle-foot
orthoses, is common in patients with cerebral palsy.
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TREATMENT
Operative
(1) correct static or dynamic deformity,
(2) balance muscle power across a joint
(3) reduce spasticity (neurectomy), and
(4) stabilize uncontrollable joints.
Often, procedures can be combined—an adductor tendon
release can be done at the time of pelvic osteotomy for
hip subluxation
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TREATMENT
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TREATMENT
HIP
In patients with cerebral palsy, all hips should be considered abnormal until proved otherwise
Deformities of the hip in patients with cerebral palsy range from
mild painless subluxation to complete dislocation with joint
destruction, pain, and impaired mobility.
prevalence of hip pain in 234 patients (mean age 28 years old)
was 47%
It has been estimated that progressive hip instability occurs in
approximately 15%.
commonly severely affected patients and patients with spastic
quadriplegia
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TREATMENT
KNEE
Hip and Knee Relationships
Pelvic, hip, knee, ankle, and foot deformities are interrelate
because of the muscles that cross both joints, the “two-joint
muscles.”
Flexion is the most common knee deformity in patients
with cerebral palsy and frequently occurs in ambulatory
children.
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POLIOMYELITIS
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POLIOMYELITIS
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POLIOMYELITIS
a viral infection localized in the
anterior horn cells of the spinal cord
and certain brainstem motor nuclei.
One of three
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types of poliomyelitis viruses
poliomyelitis vaccine,
the incidence of acute anterior poliomyelitis in
the Western world has decreased
dramatically.
Live oral poliovirus vaccine (OPV)
Inactivated poliovirus vaccine (IPV)
OPV remains the vaccine of choice for global
eradication in many parts of the world,
specifically
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Pathological Findings
When the poliomyelitis virus invades the
body through the oropharyngeal route, it
multiplies….
The incubation period is 6 to 20 days.
.
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The anterior horn motor cells may be
damaged
directly by viral multiplication or toxic byproducts of the virus or
indirectly by ischemia, edema, and
hemorrhage in the glial tissues
surrounding them.
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Destruction of the spinal cord occurs
focally, and within 3 days,
wallerian degeneration is evident throughout the length of the individual nerve
fiber.
Macrophages and neutrophils surround and partially remove necrotic
ganglion cells, and the inflammatory response gradually subsides.
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After 4 months,
residual areas of gliosis and lymphocytic cells fill
the area of destroyed motor cells in the spine.
Reparative neuroglial cells proliferate.
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Continuous disease activity has been
reported in spinal cord segments 20
years after onset of the disease
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The number of individual muscles affected by
the resultant flaccid paralysis and the severity
of paralysis vary;
the clinical weakness is proportional to the
number of lost motor units.
weakness is clinically detectable only when
more than 60% of the nerve cells supplying
the muscle have been destroyed.
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Muscles innervated by the cervical and
lumbar spinal segments are most often
affected
paralysis occurs twice as often in the lower
extremity muscles as in upper extremity
muscles.
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lower extremity
quadriceps, glutei, anterior tibial, medial
hamstrings, and hip flexors
upper extremity
the deltoid, triceps, and pectoralis major
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recovery of muscle function
depends on the recovery of damaged, but not
destroyed, anterior horn cells.
first month
almost complete within 6 months,
although limited recovery may occur for
about 2 years
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Clinical Course and Treatment
acute
convalescent
chronic
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Acute Stage
lasts 7 to 10 days
Symptoms range from mild malaise to generalized
encephalomyelitis with widespread paralysis.
In younger children,
systemic symptoms include listlessness, sore throat, and a
slight temperature elevation; these may resolve, but
recurrent symptoms, including hyperesthesia or
paresthesia in the extremities, severe headache, sore
throat, vomiting, nuchal rigidity, back pain, and limitation of
straight leg raising, culminate in
characteristically asymmetrical paralysis.
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Acute Stage
In older children and adults,
symptoms
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slight temperature elevation
marked flushing of the skin
apprehension
muscular pain is common. Muscles are tender
even to gentle palpation.
 Superficial reflexes usually are absent first, and
deep tendon reflexes disappear when the
muscle group is paralyzed.
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Acute Stage
Differential diagnoses
include Guillain-Barré syndrome
other forms of encephalomyelitis.
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Acute Stage
Treatment
bed rest, analgesics, hot packs, and anatomical
positioning of the limbs to prevent flexion
posturing and contractures.
Padded foot boards, pillows, sandbags, and slings
can help maintain position.
Gentle, passive range-of-motion exercises of
all joints should be carried out several times
each day
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Convalescent Stage
begins 2 days after the temperature returns
to normal and continues for 2 years.
Muscle power improves spontaneously
during this stage, especially during the first
4 months and more gradually thereafter.
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Convalescent Stage
Treatment
is similar to that during the acute stage.
Muscle strength should be assessed monthly for 6
months and then every 3 months.
Physical therapy
Muscles with more than 80% return of strength
recover spontaneously without specific therapy.
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Convalescent Stage
Vigorous passive stretching exercises and
wedging casts can be used for mild or
moderate contractures.
Surgical release of tight fascia and muscle
aponeuroses and lengthening of tendons may
be necessary for contractures persisting longer
than 6 months. Orthoses should be used until
no further recovery is anticipated.
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Chronic Stage
The chronic stage of poliomyelitis usually
begins 24 months after the acute illness.
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Chronic Stage
treatment goals
correcting any significant muscle imbalances and
preventing or correcting soft-tissue or bony
deformities.
Static joint instability
usually can be controlled indefinitely by orthoses.
Dynamic joint instability
eventually results in a fixed deformity that cannot be controlled with
orthoses.
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Chronic Stage
Young children are more prone to develop bony
deformity than are adults because of their growth
potential.
Soft-tissue surgery, such as tendon transfers,
should be done in young children before the
development of any fixed bony changes;
bony procedures for correcting a deformity usually
can be delayed until skeletal growth is near
completion.
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