presentation
On
CELEBRAL PALSY
BY
UDOH, CHRISTOPHER J.
OUTLINE
Introduction
Epidemiology
Etiology
Signs and symptoms
Pathophysiology
Classifications
diagnostic tests
Management
Pharmacist role
Prognosis
Conclusion
 References
INTRODUCTION
• Cerebral palsy(cp) is a static, non-progressive disorder of the CNS 20 to an
insult immature brain, resulting in varying degrees of motor milestone
delay and dysfunction.
• It is a term used t describe a group of non-progressive disorders of
movement and posture caused by abnormal development or damage to
motor control centers of the brain.
• The disorder was first described in 1862 by William John Little, an
orthopedic surgeon who observed that “children with tone and
developmental abnormalities often had prolonged labour, prematurity
and breech delivery”.
• Cerebral palsy was known as Little’s disease for decades.
• The term cerebral palsy originated with Willian Osler and Sigmund freud.
• Static encephalopathy has been used interchangeably with cp.
EPIDEMIOLOGY
The incidence of CP is about 2 per every 1000 live birth.
The incidence is higher in males than in females
Other associated problems include
• mental disadvantage ( IQ <50%) :31%.
• Active seizures : 21%.
• Mental disadvantage (IQ < 50%) and not walking: 20%.
• Blindness :11%.
The insult to the brain is believed to occur between the time of conception
to age 2 years, at which time a significant amount of motor development
has already occurred.
ETIOLOGY
• Causes of CP involve of prenatal, perinatal and postnatal causes.
• PRENATAL CAUSES.
Intrauterine infections
Obstetric complications : pre-eclampsia, eclampsia, placenta previa
Maternal disease : diabetes, hyperthyriodism
Medication use ( e.g thyroid,estrogen,progesterone)
Bleeding
Malnutrition
Previous premature birth.
These account for 70-80% of CP cases.
Etiology contd…
• PERINATAL CAUSES
Prematurity
Low birth weight
Complicated delivery
Asphyxia
Hyperbilirubinemia/kernicterus
Blood incompatibilities
Infections : herpes simplex, meningitis
Severe hypoglycemia Etc...
These account for 10% of CP cases.
Contd…
 POST NATAL CAUSES:
• Head trauma
• Non-accidental injury
• Infections : meningitis, encephalitis
• Near drowning
• Cerebral anoxia
• Cardio pulmonary arrest
SIGNS AND SYMPTOMS
PHYSICAL SIGNS.
• Poor head control at 3 months of age
• Stiff or rigid arms or legs
• Pushing away or arching back
• Floppy body posture
• Cannot sit up without support by 8 months
• Uses only one side of the body or only the arms to crawl
• Leg scissoring
• Seizures
• Sensory impairment ( hearing or vision)
• After 6 months of age, persistence tongue thrusting.
Contd…
BEHAVIORAL SIGNS:
• Extreme irritability or crying
• Feeding difficulties
• Excessive sleeping
• Little interest surrounding
PATOPHYSIOLOGY
• Periventricular leukomalacia(PVL) is the most common finding in CP.
• Bilirubin encephalopathy in basal ganglia is seen in athetoid CP
following a diagnosis of kernicterus.
• Brain malformations can be found on neuroimaging in approximately
10% of children with CP.
PATHOPHYSOLOGY contd…
CLASSIFICATIONS
• Because of the wide variability in presentation and types of CP, there
is no universally accepted classification scheme.
• CP can be classified based on:
1. The geographical region of the body affected.
2. Physiological classification.
3. Gross motor function classification.
• Classification based on region of the body
affected.
Contd…
hemiplegia
• In hemiplegia, one side of the
body is involved, with the upper
extremity usually more affected
than the lower extremity.
• Patients
with
hemiplegia,
(approximately 30% of patient
with CP) typically have sensory
changes in
the affected
extremities.
diplegia
• Diplegia is the most common anatomical
type of CP, constituting approximately
50% of all cases.
• Patients with diplegia have motor
abnormalities in all four extremities,
with the lower extremities more
affected than the upper extremities.
• The close proximity of the lower
extremity tract to the ventricles most
likely explains the more frequent
involvement of the lower extremities
with periventricular lesions.
• Here, intelligence is usually normal and
most children walk eventually but
walking is delayed till around age 4
years.
Quadriplegia
• In quadriplegia, all four
extremities are equally involved,
and many patients have
significant cognitive deficiencies
that makes care more difficult.
• Head and neck control are
present which helps with
communication, education, and
seating.
Physiological Classification
• Physiologically, CP can be divided into :
1. Spastic type, which affects the corticospinal(pyramidal) tracts, and
2. An extrapyramidal type, which affects the other regions of the
developing brain (Basal ganglia and the cerebellum).
• The extrapyramidal type of CP include:
oAthetiod
oChoreiform
oAtaxic
oRigid
oHypotonic
Contd…
Spastic cerebral palsy
• This is the most common form of CP.
• It affects 70-80% of all cases.
• The limbs are usually underdeveloped with stiff and jerky or awkward
movements.
• Here, when you try to stand the child the legs often stiffen or cross
like scissors.
The extrapyramidal types of cerebral palsy
• Athetoid CP
Involves abnormal involuntary movements that disappear during
sleep and increase with stress.
It interferes with speaking, feeding, reaching, grasping and any other
skills.
It affects 20% of CP cases.
• Choreiform
Choreiform CP is characterized by continual purposeless movements
of the patients wrists, fingers, toes, and angles. This continuous
movement can make bracing and sitting difficult.
Contd…
• Rigid CP
Patients with rigid CP are the most hypertonic of all CP patients.
These patients have a “cogwheel” or “lead pipe” muscle stiffness that
often requires surgical release.
• Ataxic CP
This is characterized by poor balance and lack of coordination and
wide-based gait.
There is tendency to fall or stumble and an inability to walk in a
straight line.
It occurs as a result of injury to the developing cerebellum and it’s
the least common type making 5-10% of CP cases.
Contd…
• It is important to distinguish true
ataxia from spasticity because
with treatment many children
with ataxia are able to improve
their gait function without
surgery.
Mixed CP
• A common combination of spastic and athetoid forms of CP.
• There is spastic muscle tone and involuntary movements.
• This type is fairly common and affects 25% 0f CP cases.
DIAGNOSTIC TESTS
Physical examination
History taking
Neurological assessment
Magnetic resonance imaging (MRI) which uses radio and magnetic waves
to study the brain in more details.
Ultrasound scan: uses sound waves to detect certain forms of anatomical
and structural abnormalities in the brain.
Computerized tomography(CT) scan, uses a series of X-rays that are then
assembled by computer to create a detailed 3-D model of the child’s
brain.
Diagnostic test contd…
Electroencephalogram (EEG), where small electrodes are placed on the
scalp to monitor brain activity.
Electromyogram (EMG) and nerve conduction studies (NCS) to test the
electrical activity of muscles and to measure the conducting function of
nerves.
Laboratory investigations, to detect any blood clotting and screen for
metabolic problems.
Management
• Pharmacological
 The goal of drug therapy is to reduce the effects of cerebral palsy
(spasticity) and prevent complications.
 Pharmacologic therapy of spasticity may be composed of oral, intrathecal,
and intramuscular therapies.
 Oral treatment options used in the management of spasticity in CP
patients include: Baclofen, clonidine, diazepam, dantrolene, and
tizanidine.
 For many patients, oral medications will not adequately control spasticity,
hence baclofen delivered intrathecally could be an option.
 Botulinum toxin-A is also administered intramuscularly directly into the
affected muscles to prevent muscle contractions.
 The doses of oral medications are summarized in the table below:
Contd…
Contd…
• If a patient is a candidate for ITB, a test dose is administered by
lumbar puncture. An initial dose of 50mcg is administered. And if no
response is noted, subsequent doses of 75mcg and 100mcg are
administered 24hours apart. If a response is evident, the patient may
have the pump implanted. The initial daily dose is calculated by
doubling the screening dose and delivering it continuously over 24
hours.
• For intramuscular Botulinum toxin, the spasticity study group has
developed dosing regimens based on units/kg of body weight. The
maximum dose for a large muscle is 3-6units/kg and for small
muscles, 1-2 units/kg. the maximum dose per injection site is 50
units.
Contd…
• Surgical treatment
Surgery is used to correct problems with bones and joints, by lengthening
any muscles and tendons that are too short and causing problems.
1. Orthopedic surgery: orthopedic surgery may be required to correct
contractures or spastic deformities, to provide stability for an
uncontrolled joint, to address bone malalignment, and to provide
balanced muscle power.
2. Selective dorsal rhizotomy(SDR): SDR is a surgical procedure that can
help children with particularly severe muscle stiffness in their legs to
improve their walking. The operation involves cutting some of the nerves
in the lower spinal column, which can help reduce leg stiffness.
Contd…
Contd…
3. Gastrostomy:
Gastrostomy may be performed to
improve feeding, correct
gastroesophageal reflux disease,
and correct associated dental
problems.
Contd…
• Other management procedures include:
1. Physical therapy: this is aimed at good skeletal alignment for
children with spasticity, it involves face, involuntary motion and gait
training. Physical therapy uses orthotic devices such as braces,
casting and splints to support and improve walking.
2. Occupational therapy: This involves the use of alternative
strategies and adaptive equipment to promote the child’s
independent participation in daily activities. Adaptive equipment
may include walkers, quadrupedal canes, seating system or electric
wheelchairs.
Contd…
3. Speech and language therapy: speech-language pathologist can help
improve the child’s ability to speak clearly or to communicate using
sign language.
4.Recreational therapy: this therapy can help improve the child’s
motor skills, speech and emotional well-being.
Example of some orthotic devices use in physical
therapy.
PHARMACIST ROLE
PROGNOSIS
1. CP is not a progressive disorder but the symptoms may become more or
less sever over time.
2. Intellectual level among ppl with CP varies from genius to intellectually
impaired, hence ppl with CP should not be underestimated.
3. The ability to live independently with CP varies widely depending on
severity of each case.
4. As the condition doe not directly affect reproductive function, some ppl
CP have children and parent successfully.
5. There is no evidence of increased chance of a person with CP having a
child with CP.
6. Ppl with CP tend to develop arthritis at a younger age than normal b/c of
pressure placed on joints by excessively toned and stiff muscles.
CONCLUSION
• Cerebral palsy is the leading cause of childhood disability.
• Spasticity is the most common movement disorder of cerebral palsy,
affecting 75% of patients.
• Pharmacologic management of spasticity may be composed of oral,
intrathecal, and intramuscular therapies.
• A comprehensive treatment plan for a patient with spasticity ivvolves
setting appropriate goals, utilizing physical therapy, orthopedic
therapy, surgical therapy, and pharmacologic therapy to achieve the
desired response.
REFERENCES
• Kelly Popec; (2008): Cerebral palsy, Pharmacologic treatment of
spasticity. US Pharm.33(1):22-26.
• Haase MR. Cerebral Palsy. In: schmock G, Brundage D, Chapman M, et
al eds(2006): Pharmacotherapy self-Assesment Program. 5th
ed.(2):213-240.
• Krach LE,(2001):Pharmacotherapy of spasticity: oral medications and
Intrathecal
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