Cerebral Palsy (CP)

Cerebral Palsy

Cerebral Palsy, (CP), is a general term encompassing a plethora of neurological disorders all of which surface from infancy to early childhood. The symptoms of CP are neither contagious nor progressive and usually surface within the first few years after birth [3]. In most cases of CP, the child is born with the disease even if the signs and symptoms may not show for months to years later [3]. Most patients diagnosed with CP showed signs of ataxia (decreased muscle coordination during voluntary movement); spasticity, when one foot or the entire leg drags while walking; tip-toeing, crouched walking or a "scissored" step; and a stiff or flaccid muscle tone [3]. For children who acquired the disease post-birth, the cause is usually from early brain damage due to abuse, repercussions of a motor vehicle accident, or a bad fall [3]. Some infectious diseases may also lead to CP-- bacterial meningitis and viral encephalitis [3].

The Discovery of 'CP'

Though there is little written proof on the earliest cases of Cerebral Palsy (CP), it is most certainly a condition that has existed for as long as women have been giving birth. The first formal description of this condition came in 1861, and was put forth by the English surgeon William Little. Dr. Little described a condition which he originally coined "Cerebral Paralysis" as a "crippling disorder that made children's muscles still, weak, and prone to twitch" [1]. Dr. Little noted that children with this particular disorder had trouble grasping, crawling, and/or walking, and also observed that many of the children born with such muscle control issues were born following difficult deliveries. This considered, Little suggested asphyxia during birth as a primary cause of the disorder [2].

Naturally, as time passed, arguments arose in response to Little's suggested cause. In 1897, psychiatrist Sigmund Freud observed that children with Cerebal Palsy also exhibited medical issues such as mental retardation, visual impairment, and seizures. Because medical concerns extended beyond those concerning the muscles and arising during birth, Freud suggested that CP was a condition that manifested itself prior to birth [1].

More recently, in the 1980's, a government study was conducted examining over 35,000 births. While researchers did find evidence indicating that birth trauma WAS indeed the cause of 1000's of cases of CP and that a small number of CP cases are caused by asphyxiation during birth [2], they were unable to determine any one single cause in the majority of cases [1].

Symptoms and Diagnosis

All forms of CP involve atypical muscle tone including the slouch in the back and shoulders while sitting [2]. Sluggish reflexes, coordination and motor development are also indicators of CP [2]. Although the disease is not usually detectable until childhood when the nervous system has matured, some delays in development during infancy may indicate CP. Of these are difficulty with head control, rolling from front to back and vice versa, reaching out using only one hand, sitting alone with no support, and walking [4].

The body movements of a person with CP may be noticeably spastic or sluggish and appear to be uncontrolled or involuntary [4]. Skeletal deformities are also common resulting in limbs on the side affected by the CP to be shorter than the limbs on the other side of the body [4]. Joint contractures, where there is different tone and strength placed on different joints may cause tetany in the surrounding muscles [4].

Some persons may also be mentally handicapped and about one third of those affected by CP will experience seizures [4]. These seizures may be a primary problem or they may have resulted from previous brain damage--these seizures may not show up until later in life [4]. Although some people with CP do not experience speech and language problems due to muscle troubles in the oral cavity, they are not uncommon in CP patients [4]. Loss of hearing is another symptom. A child may have a partial loss of hearing and not hear all sounds directed towards them or they may have a total loss of hearing and not respond to any noises.

Due to muscle weakness, 75% of Cerebral Palsy patients suffer from strabismus [4]. Strabismus occurs when the muscles around the eye controlling movement are hindered. This will cause one eye to migrate to the side while the other remains in focus. Dental problems are also problematic because the amount of cavities in a CP patient is abnormally high due to tooth enamel abnormalities and/or problems brushing teeth [4]. Eating becomes extremely challenging for a person suffering from cerebral palsy due to the fact that swallowing involves numerous muscles all of which may be affected by CP [4]. Difficulty sucking, drinking and controlling saliva result from muscle damage as well. At times,the patient may accidentally inhale solid food or liquids while eating which will then cause infection or sometimes suffocation leading to death[4]. The control of urination and defecation may also be hindered because of poor muscle control [4].

Those diagnosed with CP usually require further testings such as a CT scan and an MRI to show the neural damage. These scans also have the ability to reveal conditions such as hydrocephalus, porencephaly, arteriovenous malformation, subdural hematomas and hygromas, and a vermian tumor [2]. Vermian tumors are present in 5-22% of patients diagnosed [2].

Below is a cartoon demonstrating different limbs and sections of the body that may become unmanageable due to poor muscle control resulting from CP.

http://www.dinf.ne.jp/doc/english/global/david/dwe002/dwe002g/dwe00211g20.gif

Biological Mechanisms at Work

Each classification of CP has a different affected section of the brain and a different site of action. While the exact cause of effects are still not known it is thought that damage happened during development in the womb.[2]

In cases of Spastic CP (the most common) it is believed that damage to motor cortex or damage to the corticospinal tract. In these areas of the nervous system the damage causes a problem with gamma amino butyric acid reception leading to symptoms.[2] Butyic acid is a fatty acid that is also used to help fermentation in industry. Its chemical structure is CH3-CH2-CH2-COOH.[8]

In cases of Athetoid CP the area that is thought to be affected is the pyramidal tract and the extrapyramidal motor system. Sometimes it is damage to both of them or just one. However it is the basal ganglia that are affected.[2]

In cases of Ataxic CP it is damage to the cerebellum that will lead to symptoms.[2] The damage could be cause by a multitude of different ways. It could be caused by chemicals that enter into the developing brain or if you suffered major trauma to the cerebellum.

It is common that someone that suffers from CP will have more than one classification CP at a time. There are other classification of the disorder and listed above are the three most common.

Treatment of CP

Unfortunately, there is no one specific cure for Cerebral Palsy. In general, however, the earlier treatment begins, the better chance children have to overcome developmental disabilities. Strategies for the early detection and diagnosis of CP are very important, and include multiple measures of the "underlying brain abnomalities and their neurodevelopmental consequences" [9]. Beginning young, it becomes more possible for children to learn to accomplish tasks that the disease makes more challenging [3]. Actual treatment techniques may include:

Physical Therapy
Occupational Therapy
Speech Therapy
Drugs (Controlling seizures, relaxing muscle spasms, and lessening pain)
Surgery (To correct anatomical abnormalities or release tight muscles)
Braces/Orthotic devices
Wheelchairs/Rolling walkers
Communication Aids (Such as computers with voice synthesizers)[3]



Because CP is defined as a "group of non-progressive group of disorders of movement or posture" [8] rather than one strictly defined condition, it is very difficult to treat. Though Cerebral Palsy will always have physical consequences that affect motor development, each individual will face different specific challenges. Depending on the type of movement disorder that IS present, resulting disabilities may be reduced by therapeutic interventions. Intervention will be tailored to individual needs and goals, as well as specified to the type of CP present.

Researching CP

The majority of current research being done to cure Cerebral Palsy deals with prevention. Fetal development of the brain is being studied to understand how neural cells specialize, and how they form the correct connections within the brain[3]. Also, much work is being done to understand phenomena such as bleeding within the brain, epileptic seizures, breathing and circulation problems [3]. These events cause an elevated amounts of chemicals to be released in the brain, which lead to the damage that is eventually diagnosed as CP [3]. An example of this is illustrated when studying the chemical glutamate which is released in high amounts in specific areas where brain bleeding occurs. This event results in neurons becoming overly excited leading to their destruction [3]. Understanding how chemicals like this become toxic in excess amounts, will hopefully lead to the production of new medicine that will block the adverse effects these chemicals have on the brain [3]. Furthermore, genetic defects are being traced through family lineage to discover which genes contribute to abnormalities in brain formation[3]. Also, studies are being performed to examine whether or not substances in the brain that protect neurons can be used in aiding them during such episodes as oxygen deprivation and stroke [3].Another area of Cerebral palsy being studied is brain damage in premature babies. Research is being done to study techniques that may be used to save infants from the disorder periventricular leukomalacia, which is the leading cause of brain damage in infants [3].

Considering that CP is not always preventable, studies are being done to find which methods of physical therapy are most successful in rehabilitating those affected by CP. Studies are currently being conducted to test whether conventional physical therapy, or constraint induced therapy (CIT) is best for patients struggling with Cerebral Palsy [3]. In addition, optimal length and intensity must be determined for CIT before it can be completely affective [3]. Lastly, electrical stimulation is being used to strengthen weak muscles affected by CP. Advancements in this technology have made it much more useful, this was done by decreasing the size of the equipment, making it easier to implant, and activate these muscles' stimulators [3]. Increasing research and treatments for this disease will hopefully minimize the crippling affects Cerebral Palsy has on those affected.

Driving donations for research of CP are people such as D.J. Gregory, who suffers from the disease. Recently, he made a vow to walk every round of the PGA tour, showing that he will not be restricted by the limitations that Cerebral Palsy cause. Attached is a link to a video which shows his journey, consider that dealing with a disease such as CP usually requires all of ones energy just to get through the day [7].

http://www.youtube.com/watch?v=Cfk7NXgVHns

References

[1]link title 4 My Child: Help and Hope for Life

[2]link title Wikipedia: Cerebral Palsy

[3][1] National Institute of Neurological Disorders and Stroke

[4][2] eMedicineHealth: Practical Guide to Health

[5][3] Mayo Clinic

[6][4] Wikipedia: Butyric Acid

[7][5]: Cerebral Palsy Research News

[8] Blair, E., & Stanley, F. J. (1997). Issues in the classification and epidemiology of cerebral palsy. Mental Retardation and Developmental Disabilities Research Reviews, 3, 184-193.

[9] Palmer, Frederick. (2004). Strategies for the early diagnosis of cerebral palsy. Boling Center for Developmental Disabilities. Elsevier.

[10] [6]: Photo Depicting Muscle Restriction