Cerebral palsy (CP) is non progressive (constant) non-contagious (non infective) conditions that cause Physical disability in human development
Cerebral
refers to the cerebrum, which is the affected area of the brain (although the
disorder most likely involves connections between the cortex and other parts of
the brain such as the cerebellum), and palsy refers to disorder of movement. CP
is caused by damage to the motor control centers of the young developing brain
and can occur during pregnancy (about 75 percent), during childbirth (about 5
percent) or after birth (about 15 percent) up to about age three.
It is a
non-progressive disorder, meaning the brain damage does not worsen, but
secondary orthopedic difficulties are common. There is no known cure for CP.
Medical intervention is limited to the treatment and prevention of
complications arising from CP's effects.
Onset of arthritis and osteoporosis can occur much sooner in adults with cerebral palsy. Further research is needed on adults with CP, as the current literature body is highly focused on the pediatric patient. CP's resultant motor disorder(s) are sometimes, though not always, accompanied by "disturbances of sensation, cognition, communication, perception, and/or behavior, and/or by a seizure disorder".
Classification
CP is
divided into four major classifications to describe the different movement
impairments. These classifications reflect the area of brain damaged. The four
major classifications are:
- Spastic
- Athetoid/Dyskinetic
- Ataxic
- Mixed
In 30
percent of all cases of CP, the spastic form is found along with one of the
other types. There are a number of other, less prevalent types of CP, but these
are the most common.
A
general classification is as follows:
Spastic
Spastic cerebral palsy is by far the most common
type, occurring in 70% to 80% of all cases. People with this type are hypertonic
and have a neuromuscular condition
stemming from damage to the corticospinal tract or the motor cortex that
affects the nervous system's ability to receive gamma
amino butyric acid in the area(s) affected by the spasticity.
Spastic CP is further classified by topography dependent on the region of the
body affected; these include:
- spastic hemiplegia (one side being affected).
Generally, injury to the left side of the brain will cause a right body
deficit, and vice versa.
- spastic diplegia ( the lower extremities are affected
more than the upper extremities). Most people with spastic diplegia do
eventually walk. The gait of a person with spastic diplegia is typically
characterised by a crouched gait. Toe walking and flexed knees are common.
Hip problems, dislocations, and side effects like strabismus (crossed eyes)
are common. Strabismus affects three quarters of people with spastic
diplegia. This is due to weakness of the muscles that control eye
movement. In addition, these individuals are often nearsighted. In many
cases the IQ of a person with spastic diplegia is unaffected by the
condition.
- spastic quadriplegia (Whole body affected; all four limbs
affected equally). Some children with quadriplegia also have hemiparetic
tremors; an uncontrollable shaking that affects the limbs on one side of
the body and impairs normal movement. A common problem for children with
quadriplegia is fluid buildup. Diuretics and steroids are medications
administered to decrease any buildup of fluid in the spine that is caused
by leakage from dead cells. Hardened feces in a quadriplegia patient are
important to monitor because it can cause high blood pressure. Autonomic
dysreflexia can be caused by hardened feces, urinary infections, and other
problems, resulting in the overreaction of the nervous system and can
result in high blood pressure, heart attacks, and strokes. Blockage of
tubes inserted into the body to drain or enter fluids also needs to be
monitored to prevent autonomic dysreflexia in quadriplegia. The proper
functioning of the digestive system needs to be monitored as well.
Occasionally,
terms such as monoplegia, paraplegia, triplegia, and pentaplegia may also be
used to refer to specific manifestations of the spasticity.
Ataxic
Ataxia (ICD-10 G80.4) type symptoms can be
caused by damage to the cerebellum. Forms of ataxia are less common types of
Cerebral Palsy, occurring in at most 10% of all cases. Some of these
individuals have hypotonia and tremors. Motor skills like writing, typing, or
using scissors might be affected, as well as balance, especially while walking.
It is common for individuals to have difficulty with visual and/or auditory
processing of objects.
Athetoid/dyskinetic
Athetoid or dyskinetic is mixed muscle tone—sometimes hypertonia
and sometimes hypotonia
(Hypotonia will usually occur before 1 year old; the muscle tone will be
increased with age and progress to Hypertonia). People with athetoid CP have
trouble holding themselves in an upright, steady position for sitting or
walking, and often show involuntary motions.
For some people with athetoid CP, it takes a lot of work and concentration to
get their hand to a certain spot (like scratching their nose or reaching for a
cup). Because of their mixed tone and trouble keeping a position, they may not
be able to hold onto objects (such as a toothbrush or pencil). About one
quarter of all people with CP have athetoid CP. The damage occurs to the extrapyramidal motor system and/or pyramidal tract and to the basal ganglia. It
occurs in 40% of all cases.
Incidence and
prevalence
In the
industrialised world, the incidence of cerebral palsy is about 2 per 1000 live
births. The incidence is higher in males than in females; the Surveillance of
Cerebral Palsy in Europe (SCPE) reports a M:F ratio of 1.33:1 Variances in
reported rates of incidence across different geographical areas in
industrialised countries are thought to be caused primarily by discrepancies in
the criteria used for inclusion and exclusion. When such discrepancies are
taken into account in comparing two or more registers of patients with cerebral
palsy (for example, the extent to which children with mild cerebral palsy are
included), the incidence rates converge toward the average rate of 2:1000.
In the
Overall,
advances in care of pregnant mothers and their babies has not resulted in a
noticeable decrease in CP. This is generally attributed to medical advances in
areas related to the care of premature babies (which results in a greater
survival rate). Only the introduction of quality medical care to locations with
less-than-adequate medical care has shown any decreases. The incidence of CP
increases with premature or very low-weight babies regardless of the quality of
care.
Prevalence
of cerebral palsy is best calculated around the school entry age of about six
years, the prevalence in the
The
SCPE reported the following incidence of comorbidities in children with CP (the
data are from 1980-1990 and included over 4,500 children over age 4 whose CP
was acquired during the prenatal or neonatal period):
- Mental retardation (IQ < 50): 31%
- Active seizures: 21%
- Mental retardation (IQ < 50) and
not walking: 20%
- Blindness: 11%
The
SCPE noted that the incidence of comorbidities is difficult to measure
accurately, particularly across centers. For example, the actual rate of mental
retardation may be difficult to determine, as the physical and communicational
limitations of people with CP would likely lower their scores on an IQ test if
they were not given a correctly modified version.
Apgar scores have
sometimes been used as one factor to predict whether or not an individual will
develop CP.
Symptoms
All
types of CP are characterised by abnormal muscle tone, posture (i.e. slouching
over while sitting), reflexes, or motor development and coordination. There can
be joint and bone deformities and contractures (permanently fixed, tight
muscles and joints). The classical symptoms are spasticity, spasms, other
involuntary movements (e.g. facial gestures), unsteady gait, problems with
balance, and/or soft tissue findings consisting largely of decreased muscle
mass. Scissor walking (where the knees come in and cross) and toe walking are
common among people with CP who are able to walk, but taken on the whole, CP
symptomatology is very diverse. The effects of cerebral palsy fall on a
continuum of motor dysfunction which may range from virtually unnoticeable
to"clumsy" and awkward movements on one end of the spectrum to such
severe impairments that coordinated movements are almost impossible on the
other end of the spectrum.
Babies
born with severe CP often have an irregular posture; their bodies may be either
very floppy or very stiff. Birth defects, such as spinal curvature, a small
jawbone, or a small head sometimes occur along with CP. Symptoms may appear,
change, or become more severe as a child gets older. Some babies born with CP
do not show obvious signs right away.
Secondary
conditions can include seizures, epilepsy,
speech or communication disorders, eating problems, sensory impairments, mental
retardation, learning disabilities,
and/or behavioral disorders.
History
CP, then
known as "Cerebral Paralysis", was first identified by English
surgeon William Little in 1860. Little
raised the possibility of asphyxia during birth as a chief cause of the
disorder. It was not until 1897 that Sigmund Freud, then a neurologist, suggested
that a difficult birth was not the cause but rather only a symptom of other
effects on fetal development. Research conducted during the 1980s by the
National Institute of Neurological Disorders and Stroke (NINDS) suggested that
only a small number of cases of CP are caused by lack of oxygen during birth.
Causes
Despite
years of debate, the cause of the majority of cases of CP is uncertain.
Some
contributing causes of CP are asphyxia, hypoxia of the brain, birth trauma, premature
birth, central nervous system infections and certain infections in the mother
during and before birth. CP is also more common in multiple birth.
Studies
at the University of Liverpool has led to the hypothesis that many cases of
cerebral palsy, and other conditions that an infant has at birth, are caused by
the death in very early pregnancy of an identical twin. This may occur when
twins have a joint circulation through sharing the same placenta. Not all
identical twins share the same blood supply (monochorionic twins), but if they
do, the suggestion is that perturbations in blood flow between them can cause
the death of one and damage to the development of the surviving fetus . It is
common knowledge amongst obstetricians and midwives that a small dead fetus (fetus papyraceus) may sometimes be found attached
to a placenta following birth. In the past, this has not been considered
important and knowledge of the so called ‘vanishing twin’ has been suppressed
to avoid triggering feelings of loss, grief, or guilt in mothers. The pathological
consequences depend on the severity and the stage of development of the fetus
when the imbalances in blood flow between the fetuses occur. It has been
proposed that such pathology could account, not just for cerebral palsy, but
for developmental abnormalities of the eye, heart, and gut, and other specific
brain abnormalities such as neuronal migration disorders e.g. lissencephaly and
holoprosencephaly, which occur during very early fetal development.
Between
40% and 50% of all children who develop cerebral palsy were born prematurely.
Premature infants are vulnerable, in part because their organs are not fully
developed, increasing the risk of hypoxic injury to the brain that may manifest
as CP. A problem in interpreting this is the difficulty in differentiating
between CP caused by damage to the brain that results from inadequate
oxygenation and CP that arises from prenatal brain damage that then
precipitates premature delivery.
Recent
research has demonstrated that intrapartum asphyxia is not the most important
cause, probably accounting for no more than 10 percent of all cases; rather,
infections in the mother, even infections that are not easily detected, may
triple the risk of the child developing the disorder, mainly as the result of
the toxicity to the fetal brain of cytokines that are produced as part of the
inflammatory response. Low birthweight is a risk factor for CP--and premature
infants usually have low birth weights, less than 2.0 kg, but full-term infants
can also have low birth weights. Multiple-birth infants are also more likely
than single-birth infants to be born early or with a low birth weight.
After
birth, other causes include toxins, severe jaundice, lead poisoning, physical
brain injury, shaken baby syndrome, incidents involving hypoxia to the brain
(such as near drowning), and encephalitis
or meningitis. The three most common causes of asphyxia in the young child are:
choking on foreign objects such as toys and pieces of food; poisoning; and near
drowning.
Some
structural brain anomalies such as lissencephaly may present with the clinical
features of CP, although whether that could be considered CP is a matter of
opinion (some people say CP must be due to brain damage, whereas these people
never had a normal brain). Often this goes along with rare chromosome disorders and CP is not genetic or
hereditary.
Diagnosis
The diagnosis
of cerebral palsy has historically rested on the patient's history and physical
examination. Once diagnosed with cerebral palsy, further diagnostic tests are
optional. The
Presentation:
bones
In
order for bones to attain their normal shape and size, they require the
stresses from normal musculature. Osseous findings will therefore mirror the
specific muscular deficits in a given person with CP. The shafts of the bones
are often thin (gracile). When compared to these thin shafts (diaphyses) the
metaphyses often appear quite enlarged (ballooning). With lack of use,
articular cartilage may atrophy, leading to narrowed joint spaces. Depending on
the degree of spasticity, a person with CP may exhibit a variety of angular
joint deformities. Because vertebral bodies need vertical gravitational loading
forces to develop properly, spasticity and an abnormal gait can hinder proper
and/or full bone and skeletal development. People with CP tend to be shorter in
height than the average person because their bones are not allowed to grow to
their full potential. Sometimes bones grow at different lengths, so the person
may have one leg longer than the other
Prognosis
CP is
not a progressive disorder (meaning the actual brain damage does not worsen),
but the symptoms can become worse over time due to 'wear and tear.' A person
with the disorder may improve somewhat during childhood if he or she receives
extensive care from specialists, but once bones and musculature become more established,
orthopedic surgery may be required for fundamental improvement. People who have
CP tend to develop arthritis at a younger age than normal because of the
pressure placed on joints by excessively toned and stiff muscles.
The
full intellectual potential of a child born with CP will often not be known
until the child starts school. People with CP are more likely to have some type
of learning disability, but this is unrelated to a person's intellect or IQ
level. Intellectual level among people with CP varies from genius to mentally
retarded, as it does in the general population, and experts have stated that it
is important to not underestimate CP sufferer's capabilities and to give them
every opportunity to learn.
The
ability to live independently with CP also varies widely depending on the
severity of the disability. Some individuals with CP will require personal
assistant services for all activities of daily living. Others can live
semi-independently, needing support only for certain activities. Still others
can live in complete independence. The need for personal assistance often
changes with increasing age and the associated functional decline. However, in
most cases persons with CP can expect to have a normal life expectancy;
survival has been shown to be associated with the ability to ambulate, roll,
and self-feed. As the condition does not directly affect reproductive function,
some persons with CP have children and parent successfully.
According
to OMIM, only 2% of cases of CP are
inherited (with glutamate decarboxylase-1 as one known enzyme involved.) There
is no evidence of an increased chance of a person with CP having a child with
CP.
Treatment
There
is no cure for CP, but various forms of therapy can help a person with the
disorder to function and live more effectively. In general, the earlier
treatment begins the better chance children have of overcoming developmental
disabilities or learning new ways to accomplish the tasks that challenge them.
The earliest proven intervention occurs during the infant's recovery in the neonatal
intensive care unit (NICU). Treatment may include one or more of the following:
physical therapy; occupational therapy; speech therapy; drugs to control
seizures, alleviate pain, or relax muscle spasms (e.g. benzodiazepienes,
baclofen and intrathecal phenol/baclofen); hyperbaric oxygen; the use of Botox to relax contracting muscles; surgery to
correct anatomical abnormalities or release tight muscles; braces and other
orthotic devices; wheelchairs and rolling walkers; and communication aids such
as computers with attached voice synthesizers. For instance, the use of a standing
frame can help reduce spasticity and improve range of motion for people with CP
who use wheelchairs. Nevertheless, there is only some benefit from therapy.
Treatment is usually symptomatic and focuses on helping the person to develop
as many motor skills as possible or to learn how to compensate for the lack of
them. Non-speaking people with CP are often successful availing themselves of augmentative
and alternative communication systems such as Blissymbols.
Early
Nutritional Support In
one cohort study of 490 premature infants
discharged from the NICU, the rate of growth during hospital stay was related
to neurological function at 18 and 22 months of age. The study found a significant
decrease in the incidence of cerebral palsy in the group of premature infants
with the highest growth velocity. This study suggests that adequate nutrition
and growth play a protective role in the development of cerebral palsy.
Physical
therapy (PT) programs are
designed to encourage the patient to build a strength base for improved gait
and volitional movement, together with stretching programs to limit
contractures. Many experts believe that life-long physical therapy is crucial
to maintain muscle tone, bone structure, and prevent dislocation of the joints.
Occupational
therapy helps adults and
children maximise their function, adapt to their limitations and live as
independently as possible.
Orthotic
devices such as ankle-foot
orthoses (AFOs) are often prescribed to minimise gait irregularities. AFOs have
been found to improve several measures of ambulation, including reducing energy
expenditure and increasing speed and stride length.
Speech therapy
helps control the muscles of the mouth and jaw, and helps improve
communication. Just as CP can affect the way a person moves their arms and legs,
it can also affect the way they move their mouth, face and head. This can make
it hard for the person to breathe; talk clearly; and bite, chew and swallow
food. Speech therapy often starts before a child begins school and continues
throughout the school years.
Hyperbaric oxygen therapy Recent studies have demonstrated a dramatic improvement in CP symptomology
when hyperbaric oxygen therapy is used as a treatment. In 1989, researchers in
Nutritional counseling may help when dietary needs are not met
because of problems with eating certain foods.
Both massage therapy and hatha yoga
is designed to help relax tense muscles, strengthen muscles, and keep joints
flexible. Hatha yoga breathing exercises are sometimes used to try to prevent
lung infections. More research is needed to determine the health benefits of
these therapies for people with CP.
Surgery for people with CP usually involves one
or a combination of:
- Loosening tight muscles and releasing
fixed joints, most often performed on the hips, knees, hamstrings, and
ankles. In rare cases, this surgery may be used for people with stiffness
of their elbows, wrists, hands, and fingers.
- Straightening abnormal twists of the
leg bones, i.e. femur (termed femoral anteversion or antetorsion) and
tibia (tibial torsion). This is a secondary complication caused by the
spastic muscles generating abnormal forces on the bones, and often results
in intoeing (pigeon-toed gait). The surgery is called derotation
osteotomy, in which the bone is broken (cut) and then set in the correct
alignment.
- Cutting nerves on the limbs most
affected by movements and spasms. This procedure, called a rhizotomy,
"rhizo" meaning root and "tomy" meaning "a
cutting of" from the Greek suffix 'tomia' reduces spasms and allows
more flexibility and control of the affected limbs and joints.
- Botulinum Toxin A (Botox) injections into muscles that are
either spastic or have contractures, the aim being to relieve the
disability and pain produced by the inappropriately contracting muscle.
Another
way is that a new study has found that cooling the bodies and blood of
high-risk full-term babies shortly after birth may significantly reduce
disability or death.
Cord
Blood Therapy: There are
no published randomized controlled trials
or meta-analysis of this treatment modality in cerebral palsy. In March 2008 a
boy that was diagnosed with cerebral palsy appeared on the Today Show with his
family.[ The parents noted that he could not walk on his own and
appeared to be "swallowing his tongue" at times. He was eventually
diagnosed with cerebral palsy and could only walk with the aid of a walker for
a short time. Earlier this year he participated in a clinical trial involving
his own cord blood that his parents had saved when he was born. Within 5 days
after the procedure he was walking on his own and talking. Something his mother
said he was not capable of on his own and it was doubtful he would ever be able
to do on his own. The doctors also told his parents that if his rate of progress
continues uninterrupted until he is 7 he will be pronounced cured. The parents
message to the audience was "Bank your babies cord blood or donate it if
you do not want to keep it. But you never know when you may need it."
Conductive
education (CE) was
developed in
Biofeedback is an alternative therapy in which people
with CP learn how to control their affected muscles. Some people learn ways to
reduce muscle tension with this technique. Biofeedback does not help everyone
with CP.
Neuro
- cognitive therapy. A
new approach to treating cerebral palsy from [Snowdrop]. It is based upon two
proven principles. (1). Neural Plasticity. The brain is capable of altering its
own structure and functioning to meet the demands of any particular
environment. Consequently if the child is provided with an appropriate
neurological environment, he will have the best chance of making progress.
(2)Learning can lead development. As early as the early 1900s, this was being
proven by a psychologist named Lev Vygotsky. He proposed that children’s
learning is a social activity, which is achieved by interaction with more
skilled members of society. There are many studies, which provide evidence for
this claim. There are however, as yet no controlled studies on neuro -
cognitive therapy.
Patterning is a controversial form of alternative
therapy for people with CP. The method is promoted by The Institutes for the
Achievement of Human Potential (IAHP), a