Cerebral Palsy (CP) is a whole spectrum of disorders of movement and posture caused by a non-progressive injury to a developing brain. It is important to realize that it is a spectrum of disorders, not a specific disease, and consists of a wide variety of physical manifestations and degrees of severity, as well as a wide variety of causes. To make this even more confusing, in many cases the etiology (cause) is never found. Additionally, there is a spectrum of associated findings that are commonly found. Also, like many things in medicine, part of what we think we know about cerebral palsy changes over time as medical technology and information improves.
We will review the current understanding of cerebral palsy as far as etiologies, risk factors, classification systems and diagnostic work up. We will also discuss associated conditions, including seizure disorder, mental retardation, visual disturbances, hearing deficits, oral motor incoordination (including feeding and communication issues), sleeping disturbances, bowel and bladder issues, long-term problems of contractures, scoliosis, osteopenia and osteoporosis. We will also review therapeutic approaches including physical therapy, occupational therapy, speech therapy, medications, bracing and medical equipment. We will discuss social, school, work and age-related issues. We will review current medical treatment interventions that are relatively hot topics such as botulinum toxin and phenol injections, intrathecal Baclofen pumps and selective dorsal rhizotomy.
What is Cerebral Palsy?
Cerebral palsy has many definitions that have changed over time as more is learned about the disorder. There has been controversy over almost every aspect of its definition due to questions about the key physical findings in cerebral palsy, the associated physical findings, the time frame of diagnosis and of onset and whether it is truly non-progressive lesion or if, because of the changes that occur over time in child development and again in aging, if that part of the diagnosis can still be considered to be true.
Cerebral palsy is a group of movement disorders. It is quite variable in the degree of muscle involvement and in associated conditions. It is the most common motor problem in children. Even with about 2.5 per 1000 live births and 5 per 1000 children, this is relatively uncommon in the overall population. Because of this, it is frequently frightening for the family to hear the diagnosis of cerebral palsy. It is important for families to understand the vast spectrum of cerebral palsy. It is likely someone who first receives this diagnosis for someone they love will have preconceptions of cerebral palsy. Unfortunately, it seems for many the picture they have in mind is a scary one and certainly not what they would choose for their loved one. It is important to realize that cerebral palsy, besides not being one diagnosis but a spectrum of disorders, is a motor deficit from a nonprogressive lesion of a developing brain. Therefore, significant worsening of function is not always what the future holds.
By optimizing care, improvements in function can actually be achieved. Additionally, even if their loved one does have a very severe form of cerebral palsy, it is quite likely that with optimal care, the outlook can be much brighter than what they may have seen as a casual and uninformed observer. There are cute and functional wheelchairs for mobility. There are excellent bracing options for mobility and daily activities. There are medications for treatment of drooling. There are many feeding alternatives. There are communication devices and treatments that are being frequently updated. There are new treatments for spasticity.
In summary, a diagnosis of cerebral palsy is one that frightens parents. It is one that can show a wide variety of physical presentations, clinical course and outcomes during adulthood and there are a host of opportunities for treatment and maximizing each step. One study actually showed that half of the children who were diagnosed as having cerebral palsy by 12 months of age actually had so much improvement by the time they were 7, they no longer had this diagnosis. This is one indicator of how the body can change with development and maximizing care; in all likelihood, however, some of it may be attributable to misdiagnosis. With improvements in brain imaging, it is likely this change in diagnosis will occur less frequently.
Historic Views of Cerebral Palsy
In 1861, William John Little was the first to report a link between prematurity and adverse events with perinatal asphyxia (breathing problems at birth) leading to poor outcome. He described this condition as cerebral palsy in a lecture to the Obstetrics Society of London, but his audience did not agree with his conclusions. Little described newborn children who had “a rigidity and distortion of the limbs, who may be born on the seventh or eighth month of gestation, with possible difficulty with delivery with resuscitation required.” Even though he described asphyxia as a risk factor, he reported that the great majority recovered unharmed. He also described treatment using tenotomy (cutting tendons to improve position) “on every part of the frame.” (He himself had tenotomy of the Achilles tendon to cure his own club-foot deformity, a result of poliomyelitis that he had when he was four years old. He learned this procedure as a young orthopedic surgeon and became an authority on club-foot and tenotomy.)
Sir William Osler published a monograph in 1889 entitled “The Cerebral Palsies of Children” in which he described this non-progressive neuromuscular disease of children. He reported that cerebral palsy was usually from birth, theorizing that the trauma leading to meningeal hemorrhage (bleeding in the brain) and compression of the brain and spinal cord was the major cause. He stated, however, that is was nearly impossible to be sure of this.
Sigmund Freud was also an early major contributor to cerebral palsy investigation, publishing many articles on spastic diplegia in the late 1890s. He felt that diplegia might be congenital, with early development problems of the brain or might be due to asphyxia. He stated that the difficult birth may be merely a symptom or a result of the already present (prior to delivery) abnormality causing cerebral palsy. Besides being the first to state that abnormal brain development may be the cause of cerebral palsy, Freud was also the first to discuss the classification of cerebral palsy. It is interesting that 150 years after these first descriptions, there is still discussion about the possible causes of cerebral palsy and many questions remain unanswered. Improvements in imaging technology have improved this debate but not resolved it.
Cerebral palsy is characterized by disorders of movement and posture. It is seen in approximately 2-3 per 1,000 live births or 1-2 per 1,000 first graders in the U.S. It was hypothesized that these numbers would decrease with improvements in medical care, particularly neonatology and obstetrics. However, the numbers have not changed even though there has been a dramatic decrease in the mortality (death rate) of very low birth-weight and extremely low birth-weight infants.
