4.

Cerebral Palsy
DEFINISI
Cerebral Palsy (CP, Kelumpuhan Otak Besar) adalah suatu
keadaan yang ditandai dengan buruknya pengendalian otot,
kekakuan, kelumpuhan dan gangguan fungsi saraf lainnya.

Gejala lain yang juga bisa ditemukan pada CP:

- Kecerdasan di bawah normal
- Keterbelakangan mental
- Kejang/epilepsi (terutama pada tipe spastik)
- Gangguan menghisap atau makan
- Pernafasan yang tidak teratur
- Gangguan perkembangan kemampuan motorik (misalnya
menggapai sesuatu, duduk, berguling, merangkak, berjalan)
- Gangguan berbicara (disartria)
- Gangguan penglihatan
- Gangguan pendengaran
- Kontraktur persendian
- Gerakan menjadi terbatas.

CP bukan merupakan penyakit dan tidak bersifat progresif

(semakin memburuk).
Pada bayi dan bayi prematur, bagian otak yang mengendalikan
pergerakan otot sangat rentan terhadap cedera
CP terjadi pada 1-2 dari 1.000 bayi, tetapi 10 kali lebih sering
ditemukan pada bayi prematur dan lebih sering ditemukan
pada bayi yang sangat kecil.
PENYEBAB
CP bisa disebabkan oleh cedera otak yang terjadi pada saat:
- bayi masih berada dalam kandungan
- proses persalinan berlangsung
- bayi baru lahir
- anak berumur kurang dari 5 tahun.
Tetapi kebanyakkan penyebabnya tidak diketahui.

DIAGNOSA
Pada pemeriksaan akan ditemukan tertundanya perkembangan
kemampuan motorik.
Refleks infantil (misalnya menghisap dan terkejut) tetap ada
meskipun seharusnya sudah menghilang.
Tremor otot atau kekakuan tampak dengan jelas, dan anak
cenderung melipat lengannya ke arah samping, tungkainya
bergerak seperti gunting atau gerakan abnormal lainnya.

10-15% kasus terjadi akibat cedera lahir dan berkurangnya

aliran darah ke otak sebelum, selama dan segera setelah bayi
lahir.
Bayi prematur sangat rentan terhadap CP, kemungkinan
karena pembuluh darah ke otak belum berkembang secara
sempurna dan mudah mengalami perdarahan atau karena tidak
dapat mengalirkan oksigen dalam jumlah yang memadai ke
otak.

Berbagai pemeriksaan laboratorium bisa dilakukan untuk

menyingkirkan penyebab lainnya:
MRI kepala menunjukkan adanya kelainan struktur maupun
kelainan bawaan
CT scan kepala menunjukkan adanya kelainan struktur
maupun kelainan bawaan
Pemeriksaan pendengaran (untuk menentukan status fungsi
pendengaran)
Pemeriksaan penglihatan (untuk menentukan status fungsi
penglihatan)
EEG
Biopsi otot.

Cedera otak bisa disebabkan oleh:

Kadar bilirubin yang tinggi di dalam darah (sering
ditemukan pada bayi baru lahir), bisa menyebabkan
kernikterus dan kerusakan otak
Penyakit berat pada tahun pertama kehidupan bayi
(misalnya ensefalitis, meningitis, sepsis, trauma dan dehidrasi
berat)
Cedera kepala karena hematom subdural
Cedera pembuluh darah.

PENGOBATAN
CP tidak dapat disembuhkan dan merupakan kelainan yang
berlangsung seumur hidup. Tetapi banyak hal yang dapat
dilakukan agar anak bisa hidup semandiri mungkin.

GEJALA
Gejala biasanya timbul sebelum anak berumur 2 tahun dan
pada kasus yang berat, bisa muncul pada saat anak berumur 3
bulan.
Gejalanya bervariasi, mulai dari kejanggalan yang tidak
tampak nyata sampai kekakuan yang berat, yang menyebabkan
perubahan bentuk lengan dan tungkai sehingga anak harus
memakai kursi roda.

Pengobatan yang dilakukan biasanya tergantung kepada gejala

dan bisa berupa:
- terapi fisik
- braces (penyangga)
- kaca mata
- alat bantu dengar
- pendidikan dan sekolah khusus
- obat anti-kejang
- obat pengendur otot (untuk mengurangi tremor dan
kekakuan)
- terapi okupasional
- bedah ortopedik
- terapi wicara bisa memperjelas pembicaraan anak dan
membantu mengatasi masalah makan
- perawatan (untuk kasus yang berat).

CP dibagi menjadi 4 kelompok:

1.

2.

3.

Tipe Campuran (20% dari semua kasus CP),

merupakan gabungan dari 2 jenis diatas, yang sering
ditemukan adalah gabungan dari tipe spastik dan
koreoatetoid.

Tipe Spastik (50% dari semua kasus CP), otot-otot

menjadi kaku dan lemah.
Kekakuan yang terjadi bisa berupa:
- Kuadriplegia (kedua lengan dan kedua tungkai)
- Diplegia (kedua tungkai)
- Hemiplegia (lengan dan tungkai pada satu sisi
tubuh)
Tipe Diskinetik (Koreoatetoid, 20% dari semua kasus
CP), otot lengan, tungkai dan badan secara spontan
bergerak perlahan, menggeliat dan tak terkendali;
tetapi bisa juga timbul gerakan yang kasar dan
mengejang. Luapan emosi menyebabkan keadaan
semakin memburuk, gerakan akan menghilang jika
anak tidur
Tipe Ataksik, (10% dari semua kasus CP), terdiri dari
tremor, langkah yang goyah dengan kedua tungkai
terpisah jauh, gangguan koordinasi dan gerakan
abnormal.

Jika tidak terdapat gangguan fisik dan kecerdasan yang berat,

banyak anak dengan CP yang tumbuh secara normal dan
masuk ke sekolah biasa.
Anak lainnya memerlukan terapi fisik yang luas, pendidikan
khusus dan selalu memerlukan bantuan dalam menjalani
aktivitasnya sehari-hari.
Pada beberapa kasus, untuk membebaskan kontraktur
persendian yang semakin memburuk akibat kekakuan otot,
mungkin perlu dilakukan pembedahan.

Pembedahan juga perlu dilakukan untuk memasang selang

makanan dan untuk mengendalikan refluks gastroesofageal.

PROGNOSIS

Cerebral palsy is due to a brain abnormality that does

not progress in severity.

Prognosis biasanya tergantung kepada jenis dan beratnya CP.

Lebih dari 90% anak dengan CP bisa bertahan hidup sampai
dewasa.

The causes of cerebral palsy include prematurity,

genetic disorders, strokes, and infection of the brain.

Taking certain precautions during the pregnancy

might decrease the risk of cerebral palsy.

Asphyxia, the lack of oxygen to the brain, at birth is

not as common a cause of cerebral palsy as had been
thought.

There are different types of cerebral palsy based on

symptoms -- spastic, hypotonic, choreoathetoid and
mixed types.

The best approach for diagnosis, treatment, and

management is through and interdisciplinary team.

Cerebral palsy may be associated with many other

medical conditions, including mental retardation or
seizures. Many of these conditions can be treated
with improved quality of life.

Many children with cerebral palsy have a normal

intellect and have no seizures.

Treatment of cerebral palsy is for the symptoms only;

there are few treatments for the underlying causes.

There are many alternative medicines promoted for

the treatment of cerebral palsy that have never been
proven to be helpful. Families and advocates of
persons with cerebral palsy should be aware of the
lack of scientific basis for these treatments.

PENCEGAHAN
Sebagian besar kasus cerebral palsy tidak dapat dicegah,
meskipun dengan upaya terbaik orangtua dan dokter. Tapi,
jika Anda hamil, Anda dapat mengambil langkah-langkah ini
untuk tetap sehat dan meminimalkan kemungkinan komplikasi
kehamilan:
1.

2.

3.

Pastikan Anda diimunisasi. Imunisasi terhadap

penyakit-penyakit seperti rubella dapat mencegah
infeksi yang dapat menyebabkan kerusakan otak
janin.
Jaga dirimu. Semakin sehat Anda menuju kehamilan,
semakin kecil kemungkinan Anda akan mengalami
infeksi yang dapat mengakibatkan cerebral palsy.
Carilah perawatan awal dan berkesinambungan
pralahir. Rutin melakukan kunjungan ke dokter Anda
selama kehamilan adalah cara yang baik untuk
mengurangi risiko kesehatan untuk Anda dan bayi
yang belum lahir. Periksa ke dokter Anda secara
teratur dapat membantu mencegah kelahiran
prematur, berat lahir rendah dan infeksi.

Cerebral Palsy At A Glance

Cerebral palsy (CP) is an abnormality of motor

function, the ability to move and control movements.
Cerebral palsy is acquired at an early age, usually less
than a year of age.

which are permanent limitations in the ability of joint

movement. Contractures can be greatly limiting in the care of
children with cerebral palsy. Spasticity can also be quite
painful, requiring medication to relax the muscle tone.

What is spastic cerebral palsy?

Spastic cerebral palsy refers to a condition in which the
muscle tone is increased, causing a rigid posture in one or
more extremities [arm(s) or leg(s)]. This rigidity can be
overcome with some force, ultimately giving way completely
and suddenly -- very much like the familiar jackknife (or clasp
knife). The spasticity leads to a limitation of use of the
involved extremity, largely due to the inability to coordinate
movements. Often the spasticity occurs on one side of the
body (hemiparesis), but it can also affect the four limbs
(quadriparesis) or be limited to both legs (spastic diplegia).
When the condition occurs in both legs, the individual often
has a scissoring posture, in which the legs are extended
(straightened) and crossed.

The same fundamental processes that influence spasticity of

the limbs can also result in abnormalities of movement and
muscle tone in other body systems. In the muscles of the head
and face, for example, cerebral palsy can greatly limit the
coordination and production of speech, even when the child is
perfectly capable of understanding speech. There can also be
limitations of chewing, swallowing, and facial and eye
movements. These symptoms can be particularly troubling for
afflicted children and their families.
Many patients with spastic cerebral palsy cannot control their
output of urine. This inability is not necessarily due to
problems in thinking but is caused by heightened reflexes of
the bladder. When the bladder fills in these children, it is just
like tapping on it with a reflex hammer, thus making it
contract more vigorously than normal and causing a spilling of
urine. This incontinence can be quite embarrassing, especially
in a cognitively intact child.

Besides the increased muscle tone there is also increased deep

tendon reflexes, impaired fine and gross motor coordination,
muscle weakness, and fatigability among other problems.
Spasticity is often the result of damage to the white matter of
the brain, but it can also be due to damage of gray matter.
The degree of spasticity can vary, ranging from mild to severe.
Children who are mildly affected may experience few
limitations of their function while severely affected children
may have little to no meaningful use of the affected limb(s).
Spasticity, if not properly treated, can result in contractures,

What is choreoathetoid cerebral palsy?

Choreoathetoid cerebral palsy is associated with abnormal,
uncontrollable, writhing movements of the arms and/or legs.

Different from spastic cerebral palsy, persons with

choreoathetoid cerebral palsy have variable muscle tone often
with decreased muscle tone (hypotonia). Contractures of
extremities are less common. The abnormal movements are
activated by stress, as well as by normal emotional reactions
such as laughing. Any attempt to do voluntary movements, for
example extending the arm in an attempt to reach an object
might result in many involuntary movements in arms, legs,
trunk, and even the face. There are different types of abnormal
movements. Two of the most common are choreoathetotic
movement disorder with rapid, irregular, unpredictable
contractions of individual or small muscles groups and
dystonia with a persistent but not permanent, abnormal posture
of some body parts (arms, legs, trunk) due to abnormal muscle
contractions. The dystonic disorder also affects the muscle of
the facial expression, swallowing, deglutition and speech,
resulting in severe functional deficiencies.

Hypotonia is diminished muscle tone. The infant or child with

hypotonic cerebral palsy appears floppy -- like a rag doll. In
early infancy, hypotonia can be easily seen by the inability of
the infant to gain any head control when pulled by the arms to
a sitting position (this symptom is often referred to as head
lag). Children with severe hypotonias may have the most
difficulty of all children with cerebral palsy in attaining motor
skill milestones and normal cognitive development.
Hypotonic cerebral palsy is often the result of severe brain
damage or malformations. It is believed that hypotonic
cerebral palsy is the result of an injury or malformation at an
earlier brain developmental stage than that which causes
spastic or choreoathetoid cerebral palsy.
Hypotonia in infancy is a common finding in many
neurological conditions, ranging from very mild abnormalities
to severe or even fatal neurodegenerative or muscle disorders.
It is important to note that many children with spastic cerebral
palsy go through a short stage of being somewhat hypotonic in
early life, before presenting the full syndrome

These movements can be quite debilitating and greatly limit

the child's ability to perform many motor tasks. Furthermore,
the movements are akin to constant exercise, thereby causing
the affected child to metabolize a huge number of calories.
Choreoathetoid cerebral palsy is often associated with damage
to specialized brain structures that are involved in movement
control -- the basal ganglia. Like spastic cerebral palsy, the
degree of symptom severity often varies, from mild to severely
affected.

What is mixed cerebral palsy?

Many (possibly most) children with cerebral palsy have
multiple symptoms with combinations of the various forms of
cerebral palsy. For example, children with spastic cerebral
palsy often continue to have a head lag, which is
representative of hypotonia. Children with choreoathetoid or
hypotonic cerebral palsy often have increased deep tendon
reflexes, which are suggestive of some spasticity.

What is hypotonic cerebral palsy?

Seizures are a common finding in patients with cerebral palsy.

Perhaps a third of all cerebral palsy patients have seizures.
Seizures are caused by abnormal electrical activity of the
neurons in the brain. The damaged or malformed brain is more
prone to seizures. Moreover, cognitive disability is frequently
associated with epileptic seizures.

What other conditions are associated with cerebral palsy?

Since cerebral palsy is indicative of damage to or
malformation of the brain, it stands to reason that other
symptoms that are associated with brain dysfunction can be
present in children afflicted with cerebral palsy. In fact other
disorders, besides the motor dysfunctions already described,
are almost always seen in these patients. Some of them such as
poor speech, swallowing disorders, drooling, and poor fine or
gross motor coordination are the result of the motor disorder
affecting specific muscles involved in those functions. Other
conditions are the results of simultaneous injuries in areas of
the brain besides the motor areas.

The symptoms of seizures can vary depending on where in the

brain they originate. Generalized seizures engage the entire
cerebral cortex at once, while partial seizures only involve part
of the cerebral cortex. Often, generalized seizures begin as
partial seizures but spread throughout the brain rapidly.
Generalized seizures may take the form of true convulsions
("grand mal"), in which the entire body jerks in a rhythmic
fashion, or the form of absences ("petit mal"), which interrupt
the patient's activities for a brief period, but does not cause a
fall.

Cognitive disabilities, sometimes referred to as developmental

delay, is often associated with cerebral palsy. Up to 50% of
patients with cerebral palsy have cognitive disabilities.
However, many of these children can be educated and lead
productive lives. It is also just as important to note that many
children with severe motor impairment due to cerebral palsy,
as is the case with many children with the choreoathetotic or
the diplegic form of cerebral palsy, are only mildly or not at
all intellectually impaired.

Other forms of generalized seizures can occur in the cerebral

palsy patient. Atonic seizures cause the patient to slump
suddenly to the ground or forward in their chair, resembling a
marionette in which the puppeteer suddenly cut the strings.
Tonic seizures are just the opposite and cause the entire body
to suddenly stiffen. Both tonic and atonic seizures can result in
drop attacks in which the patient falls to the ground, often
resulting in injury.

Virtually all testing of a young child's cognitive development

involves some sort of motor activity on the part of the child. If
a child is capable of complex thoughts, but incapable of motor
activity, the observer will not be able to detect his or her
mental aptitude. Therefore, one must be very careful in
assigning labels to patients with cerebral palsy. Certain
features, however, are more likely to be associated with
significant cognitive disabilities in the patient with cerebral
palsy. These include extensive damage occurring on both sides
of the brain, children with spastic quadriplegia, microcephaly
(small head size), a documented genetic disorder, and a
documented prenatal infection.

Partial seizures may involve the jerking of the arm and leg on
the same side of the body. Alternatively, they may be
associated with strange sensory phenomena, such as flashing
lights, or emotions, such as fear, depending on where in the
brain the seizure occurs.
Vision deficiencies are frequently seen. Some of them, for
example, strabismus ("lazy eyes") can be corrected by surgical
procedures in the muscles of the eyes. Some can be corrected

with eye glasses (that may be difficult to implement in noncooperative children). In other children the visual deficiencies
are the result of brain injuries to the areas of the brain that are
associated with vision, rendering the child blind ("cortical
blindness") even if the eyes themselves are perfectly normal.
At the present time there is no treatment to improve this
condition.

It is difficult for children with cerebral palsy to gain weight

and frequently have delayed growth. This is the result of
several factors including feeding disorders, gastroesophageal
reflux, and in some instances, for example, children with
choreoathetotic disorders, excessive caloric consumption. On
the other, hand obesity could be a problem in those children
with cerebral palsy that have and limited mobility.

Children with cerebral palsy can have speech disorders of

many types. Some, like poor word pronunciation (dysarthria),
are the result of impairment of the peripheral mechanism of
speech (poor lips, tongue, or palate coordination). In another
circumstance there is brain injury in the gray matter of the
brain that controls the central mechanism of speech (aphasia).

Individuals with the choreoathetotic form of cerebral palsy

might have compressed nerves or damage to the neck bones
that can lead to damage to the spinal cord.

What are specific treatment plans for cerebral palsy?

spasticity and thereby helps the child walk with a more normal
gait. Most neurosurgeons performing dorsal rhizotomies very
carefully select only those patients whom they feel may be
helped by the surgery. From time to time, other surgical
interventions are required in children with cerebral palsy. In
very rare cases of choreoathetoid cerebral palsy, in which the
writhing movements severely limit the ability of the child to
function, highly selective neurosurgical techniques can curtail
these movements without significantly harming other
functions.

Other issues to be aware of are dental diseases, respiratory

problems, urinary tract infections, osteoporosis and
subsequent fractures, enuresis, encopresis, constipation

After the initial evaluation, specific treatment plans are

outlined for each child:
Seizure medication
If the child has seizures, the treatment is based on the type and
frequency of the seizures. Complete seizure control can often
be achieved using a single medication, but some children with
cerebral palsy have particularly difficult-to-control seizures.
Medication can have side effects affecting the brain, ranging
from sedation to hyperactivity. They can also affect liver
function, white and red blood cells, and bone metabolism.
Side effects are usually not harmful and resolve when the
offending medication is discontinued. The goal of the treating
physician should be for the child to become seizure free with
few or no side effects. It must be noted that it is of no benefit
to the child to be seizure-free but significantly impaired by
medication side effects.

Other surgical procedures

Ophthalmologists (eye specialists) can help strabismus by
operating on the muscles that control the movement of the eye
or to correct some other complications such as cataracts.
Neurosurgeons can treat intractable seizure control.
Operations such as callosotomy, hemispherectomy, focal
resections of areas of abnormal brain tissue responsible for the
seizures, might be indicated in some cases. An alternative
procedure for the treatment of epilepsy is the vagal nerve
stimulation, an implantable device, that can be useful in
selected patients with difficult to control seizures.

Medications for spasticity: The treatment of spasticity can

involve multiple health professionals. Treatments involve the
use of medications and surgical procedures to decrease the
spasticity, facilitate movement, and prevent contractions.
Among the most commonly medications are dantrolene
sodium (Dantrium) and diazepam (Valium). Diazepam is both
a muscle relaxant and a sedative. Baclofen (Lioresal) can be
taken by mouth or infused continuously with an implanted
pump (intrathecal infusion) directly in the cerebrospinal fluid
(the liquid that bathe the spinal cord and the brain). This
treatment might be specifically useful for patients with
spasticity in the lower legs. The most common complications
with these medications are drowsiness, sleepiness, some
degree of weakness. The sedative side effects of such
medications often limit their usefulness. In the case of the
baclofen pump the most common complication seen in small
number of patients is the infection of the catheter.
Additionally, a muscle relaxing agent called botulinum toxin
can be injected into tight muscles to relax them. When used
prudently, this procedure may prevent surgical intervention.

Scoliosis, or curvature of the spine, is often the result of severe

hypotonia. This condition can create discomfort for the patient
and difficulty for caregivers in performing the activities of
daily living. Furthermore, severe scoliosis may actually
restrict a patient's ability to breathe. Several surgical
procedures are available in specialized centers for the
correction of scoliosis.
Children who are unable to take adequate calories by mouth
may require the placement of a feeding gastrostomy tube
(PEG tubes) directly into the stomach.
Therapy
The treatment of spasticity can involve multiple allied
healthcare professionals. Physical and occupational therapists
play an important role.

Surgery

Physical therapy: The extent of physical therapy depends on

the degree of spasticity, hypotonia, and motor impairment.
The main therapeutic effect of physical therapy is maintaining
range of motion at the joints, thereby preventing contractures.
Some scientists and therapists feel that physical therapy
actually helps maintain the connections in the brain, although
this is controversial. Other skills, such as improved gait,
stance, and balance can be helped by physical therapy. A

Surgery for spasticity: In the case of severe muscle

spasticity, surgery may be a valuable option. Tendon release
procedures, usually performed by an orthopedic surgeon,
allow improved range of motion in some cases. Such
procedures are usually performed on the muscles of the calf or
inner thigh. A less commonly used procedure, is the dorsal
rhizotomy. During this operation, the surgeon cuts some of the
nerve roots that send sensory information from the muscles to
the spinal cord and brain. This procedure relieves some of the

strong, proactive physical therapy program greatly aids in the

life of a child with cerebral palsy.

Medical care
Medical care of children with cerebral palsy is often seriously
hampered by the inability of the child to communicate his or
her needs and sensations. Relatively common childhood
illnesses in children with cerebral palsy, such as ear infections,
urinary tract infections, and appendicitis, which are easily
treatable in most children, may prove to be life-threatening
due to delayed recognition on the part of caregivers and
physicians. Each child with cerebral palsy should have a
primary care practitioner that is experienced with the special
medical needs of affected children.

Occupational therapy: Occupational therapy assists children

with the skills needed for day-to-day life in school and at
home, including eating, writing, and work skills. In early
infancy, occupational therapists can provide assistance in
feeding a child with a poor or uncoordinated sucking response.
Speech therapy: Speech and language pathologists are
involved with the development and improvement of speech
production. Using different techniques the speech pathologist
helps to improve the quality and the quantity of the speech
production. The role of these specialists is not limited to
speech production alone, but they also teach the patients other
communication techniques (sign language, use of
communication boards) to facilitate the communication
abilities.

Because physicians have offered limited hope in curing

cerebral palsy, many families have turned to alternative
methods in the treatment of their children. Such therapies may
include diets, herbal remedies, aromas, play with animals, and
hyperbaric oxygen. The scientific evidence supporting the use
of diets is inadequate. Some of them such as hyperbaric
oxygen therapy, which is delivered in specialized centers, can
be quite expensive and has not been scientifically proven to
help children with cerebral palsy. Other remedies must be
investigated on a case-by-case basis. Hopefully, the family of
the affected child will be able to approach their physician
regarding these alternative treatments.