General definition

Cerebral palsy, CP for short, is an umbrella term referring to a group of movement disorders caused by an early brain injury or disturbance in neurological development that affects they areas of the brain and nervous system involved with motor control and coordination. People who have CP may also have other symptoms and conditions beyond motor control and function such as epilepsy, difficulty with visual processing (CVI-cortical visual impairment) or learning difficulties. The symptoms and conditions the individual may face relates to the how the brain injury/disturbance has affected the brain’s functions. Although CP is caused by “non-progressive/unchanging disturbances” in the brain, that do not worsen over time, the expression of the initial injury may change as the brain develops further and there is ongoing wear and tear on the muscles and bones. At this time there is no cure for cerebral palsy but medical and therapeutic interventions can improve symptoms. Although often thought of as a childhood disorder, cerebral palsy, the most common motor disability in childhood, also affects a large adult population.

**There is often much misunderstanding around the cerebral diagnosis among families, the public and even professionals. This year I wrote a blog post about tweaking context for presenting and treating people with cerebral palsy as an early developmental brain injury, which I refer to as an EDBI. I am very pleased to share that a version of this post was also published in the medical journal “Developmental Medicine and Child Neurology”. Although cerebral palsy hones in on the parts of the brain responsible for motor function affected by the initial injury or disturbance in development, that same injury may have affected other areas of function as well. Those potential problems are not necessarily clear to parents or clinicians unfamiliar with treating people with cerebral palsy. It is my hope and intention that better and more proactive care and treatment will result from clarifying the context of this diagnosis.  -Michele

How CP differs from a traumatic brain injury?

In CP the disturbance in brain development has occurred before, during or shortly following birth. The injury may also occur after the neonatal period, but must be during the first few years of life, during early neurological development to be considered cerebral palsy. The reason for this boundary (albeit a bit hazy) is that in CP the individual has not developed their early motor skills. This is in contrast to later brain injuries classified such as traumatic brain injuries or TBIs, where neurological development is further along or has ended and the individual loses already established motor skills. Also, unlike CP, which has a primary focus on motor disorders, TBI is a term that captures the range of all potential symptoms and conditions that result from the brain injury.

1 The 2004 International Summit on defining and classifying CP Rosenbaurm et al. (2007).

Here is the summary document from the workshop on defining cerebral palsy that is mentioned above.

This article published in Discovery Health on 2-14-2013 is one of the most comprehensive yet concise summaries of cerebral palsy information I have come across.

What Causes CP?

**Check out this excellent article which documents the historical understanding of CP and where we are now in our quest of prevention and treatment.

There are many factors that may make an individual vulnerable to a brain injury/disturbance in early brain development leading to CP. Knowledge has shifted from a place where all cases were thought to be caused by complications due to lack of oxygen during the birthing process, to now seeing a diversity of factors and causal pathways. These even include some genetic forms of CP where gene mutations cause disturbances in early brain development leading to motor impairment. Despite having several trends in risk factors such as prematurity, low birth weight, multiple pregnancies, intrauterine infection and more, the underlying causes that ultimately lead to CP are not well understood.

It is thought that most causes of cerebral palsy are caused by risk factors associated with the prenatal period. A few of the most common ones are intrauterine infection, intrauterine growth restrictions, and prematurity (with low birth weight posing a greater risk).(1) The premature brain is at higher risk for bleeding since blood flow is difficult for the premature infant to regulate. After the neonatal period, a child may also suffer a brain injury which leads to CP. Injuries to the brain during this time are less common causes of CP and account for approximately 10-18% of cases.(2) These cases may be due to stroke, head trauma, or severe hypoxic events such as near-drowning. Bacterial, viral, and fungal infections may also lead to CP.

It is important to understand that a premature infant presenting with motor delays alone does not dictate a diagnosis of cerebral palsy. In fact, a documented brain injury seen on an MRI or head ultrasound does not dictate a particular outcome either. Pre-term infants may present with motor problems or delays that resolve as late as after one year of age, but in cerebral palsy the motor issues persist. For example, an infant may have a bleed that resolves and heals without the presentation of CP (although there could possibly be other delays or issues from the damage that occurred). Alternatively, a child may present with cerebral palsy with no known or recognizable injury/lesion in the MRI. One of the reasons physicians often take their time in diagnosing cerebral palsy is because the brain has the ability to change and heal (referred to as plasticity) particularly during early development. What may present as a problem initially could resolve over time and this would not be considered cerebral palsy.

Research from the 1980’s provided new information that discounts a still commonly held belief that most cases of cerebral palsy are caused by a lack of oxygen or trauma to the brain during delivery (birth asphyxia). However, asphyxia is rarely a cause of cerebral palsy. Birth complications including asphyxia are now estimated to account for only about 6 percent of congenital cerebral palsy cases.(3) We still need much more research in order to understand the specifics behind the trends that have been established with CP causation. For instance prematurity is a commonly associated risk factor for CP but why do some premature babies develop CP and others do not?

How is CP diagnosed?

Receiving a diagnosis of CP is not always a straightforward path. It is up to the physician/s including pediatricians, developmental specialists, and neurologists to make this determination. This is done by the physician/s observing and feeling the child’s patterns of movement and tone, considering the mother and child’s medical history during the pregnancy, and reviewing imaging of the brain including head ultrasounds and MRIs. It may take several months or up to a year (sometimes more) to confirm a diagnosis of CP.

According to the Emory University School of Medicine, Dept. of Pediatrics the following conditions must be present for a diagnosis of CP:

1. Movement of muscles has to be adversely affected.

2. The motor impairment has to be due to a neurological injury.

3. The injury or lesion must be static (not getting worse, but no longer resolving).

4. The injury has to occur while the motor cortex of the brain is in early development. *

5. The impairment in movement does not resolve with time.

Does an MRI or neurological events dictate a diagnosis of CP?
When our daughter had an intraventricular hemorrhage at 35 weeks of age (2 weeks after birth), we were told we would have to wait and see if she “develops” cerebral palsy. That was a misleading statement. The truth is that she would not “develop” cerebral palsy, but if her bleed led to persisting developmental symptoms, these symptoms may lead (and in our cases did) to a diagnosis of CP. Later, when we were confronted with the actual diagnosis, I asked the neurologist if Maya could just be delayed rather than having CP. The answer I received was that it is not the damage that necessarily dictates the diagnosis, but rather a collection of information, symptoms, and histories merging together over time.

One of the most difficult yet helpful pieces of information I can offer you is that nothing about CP is black and white. The diagnosis, symptoms, and trajectory of development of each child will vary according to his/her brain injury. We have even found that knowing which areas and sides of the brain are affected does not necessarily correspond to the presentation of symptoms in the body. One child with CP may have vastly different symptoms from another affected child even when their brain MRIs look similar.

Your journey with your child does not come with a road map. If you can accept this now (or start the process of acceptance) you will be better prepared for the road ahead. No doctor, therapist, parent, nurse, or grandparent can predict how your child with CP will develop.  The earlier you understand this, the better.  Hopefully with this understanding, you will not sacrifice your time and energy searching for answers that no one may have, and you will save yourself the disappointment of false expectations regarding your child’s development. On the other hand, you also may find you are pleasantly surprised with how well your child develops over time.  For more on this topic visit the Coping with the Diagnosis page.

Source Citations: 1,23, from “Epidemiology and etiology of cerebral palsy” from Up to Date Journal. Written by Dr. Geoffrey Miller of Yale University. Last update at the time this was written was April 11, 2012.

Reference resources:

Cerebral Palsy: A Complete Guide for Caregiving By Dr. Freeman Miller and Professor Steven J. Bachrach.

Dana and Christopher Reeve Foundation website

Emory University School of Medicine Developmental Progress Clinic website

“Epidemiology and etiology of cerebral palsy” from Up to Date Journal, written by Dr. Geoffrey Miller of Yale University.

“Clinical features of cerebral palsy” from Up to Date Journal, written by Dr. Geoffrey Miller.

Kennedy Krieger Institute’s online resource finder

MedicineNet.com

Maya’s Mom’s insight into describing CP

I meet very few people who understand cerebral palsy. VERY simply, people with cerebral palsy all have one thing in common; an early neurological disturbance that leads to permanent interference in motor control/coordination. The degree to which an individual’s daily life is impacted and which parts of the body are involved, varies from one person to another. In addition, the challenges an individual with cerebral palsy faces may not end with motoric challenges and the secondary outcomes on the musculoskeletal system. The initial interference/injury may have affected parts of the brain beyond those that are responsible for motor control/coordination.

An individual with CP may have epilepsy, learning difficulties, speech and hearing challenges, eating/swallowing disorders, sensory processing and integration difficulties, sleep disorders, hydrocephalus, and more. On the other hand, some people with CP may have (for example) a slight limp in their gait and nothing further.

The brain is the control system for the entire body. Anything that it controls may have been affected by that early injury that lead to a diagnosis of cerebral palsy. The way that initial injury presents in the individual leads to a diversity of challenges people with CP may face.

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When special needs blogger Ellen Seidman from Love That Max got into a conversation with a 12 year old about cerebral palsy his interpretation was ingenious! He called it “rebel muscles”. Check out the post and how Ellen applies the concept to other disabilities that offers an opportunity for a huge perceptual shift in thinking.

Types/classifications of CP

CP is commonly classified according to the pattern of movement or muscle abnormality and by which specific limbs are involved. Classifications for cerebral palsy are evolving as researchers learn more about CP and strive for a universal language that is consistent in meaning. Consensus meetings among leading physicians and researchers are leading to changes in descriptions that include more precise information about how the condition affects an individual’s function and ability to participate in activities. This is in contrast to solely describing which limbs are affected (i.e. diplegia, hemiplegia, quadriplegia) and subjective terms of degree (mild, moderate, severe). This more meaningful and precise approach is accomplished by using the Gross Motor Function Classification System or the GMFCS, GMFM, ICF, and other universal scales for assessing individual function.

Here are some short-hand illustrations and descriptions based on the GMFCS scale which parents, individuals with CP, and therapists, clinicians can use to discuss CP presentations more clearly and quickly.

Types of muscle/movement abnormalities associated with CP:

Spastic Cerebral Palsy — About 70 to 80 percent of affected individuals have spastic cerebral palsy. In spastic CP, muscles are stiff, making movement difficult.

Athetoid/Dyskinetic Cerebral Palsy — About 10 to 20 percent have the this form, which affects the entire body and is caused by damage to the cerebellum or basal ganglia. It is characterized by the involuntary movements of athetosis, chorea and dystonia. There are two categories of Dyskinetic CP based upon the predominant type of irregular movement. One type is mainly athetoid and the second type is characterized by mainly dystonic movements. Dyskinetic CP is characterized by fluctuations in muscle tone (varying from too tight to too loose) and sometimes is associated with uncontrolled movements (which can be slow and writhing or rapid and jerky). This type of CP is typically not associated with pre-term birth related incidents.

Ataxic Cerebral Palsy — About 5 to 10 percent have the ataxic form which is the rarest and is caused by damage to the balance centers of the brain. The diagnosis of ataxic CP is made by exclusion of the other forms since all patients with CP have difficulty with coordination and posture. Ataxic CP affects balance, depth perception, and coordination. Most patients have congenital hypotonia. Motor milestones and language skills are typically delayed. When engaging in fine motor movements there is often a tremor or shakiness that is present. Most patients with a diagnosis of Ataxic cerebral palsy have been born at term.

Mixed Cerebral Palsy— Patients present with symptoms of more than one of the previous three forms. The most common mixed form includes spasticity and dyskinetic movements but other combinations are also possible.

Important CP facts

1. An average of 1 in 303 8-year old children in the US are affected by CP and over 800,000 Americans. Source-CDC-This number comes most recently from a study conducted in 2008. It used population information from the CDC’s Autism and Developmental Disabilities Monitoring (ADDM) Network in the US including Alabama, Georgia, Wisconsin, and Missouri. Exact numbers of CP in the US are truly unknown at this time. Here is a link to the CDC’s most recent report.

2. CP affects more than 17 million people worldwide. Source-Cerebral Palsy Alliance, Australia

3. Cerebral palsy is the most common motor disability affecting children. Source-CDC, NINDS, NIH

4. Despite the above statistic, there is no dedicated line item federal funding for cerebral palsy research for the CDC or NIH :(

5. A lack of in-depth causation and epidemiology research for CP, means that in most cases the underlying cause of why cerebral palsy develops is still unknown.  For instance prematurity is a risk factor associated with the development of cerebral palsy. However, not all premature babies develop CP. Source- Cerebral Palsy: From Diagnosis to Adult Life, 2012.

The following link is to the “Cerebral Palsy Fact Sheet” created by the Centers for Disease Control and Prevention (CDC).

NINDS (National Institute of Neurological Disorders and Stroke)  also offers a comprehensive cerebral palsy page on their website.

“Learn More about CP”: a video created by Reaching for the Stars and featuring Dr. Lisa Thornton, a Pediatric Physical Medicine and Rehabilitation doctor.

History of CP

Researchers have identified references to the presentation of cerebral palsy dating back to ancient Greece and Egypt. In fact, one of the oldest and well-known possible examples of cerebral palsy is a mummy of the Pharaoh Siptah who ruled toward the end of the 19th dynasty (1196-1190 B.C). The skeletal photographs and x-rays of the Pharaoh led some Egyptologists and researchers to draw this conclusion. In ancient Greece, Hippocrates, known as the Father of Medicine, discussed the underpinnings of cerebral palsy in his work “Of the Eight-Month Foetus”. In this work he mentions the association of prematurity, congenital infection, and prenatal stress in relation to the development of brain damage in children. It is Hippocrates who is credited with offering the first medical description of cerebral palsy in the Ancient World. (1)

In the 1800′s three influential figures emerged in the study and description of cerebral palsy. William John Little, the founder of orthopedic surgery in England, is credited as the first person to intensely study and professionally discuss CP. Little published nine lectures on pediatric orthopedics and within them he described CP’s inception as occurring at the moment of birth or shortly thereafter (2). By the end of the 19th century, Little had described the condition of spastic diplegia as “Little’s Disease” which he attributed to prematurity and birth asphyxia. After Little, Sir William Osler, a professor of clinical medicine in Pennsylvania, reviewed 152 cases of cerebral palsy and wrote a book entitled “The Cerebral Palsies of Children,” first published in 1889. Osler concluded that the development of these palsies, was strongly related to intracranial hemorrhaging.

The third of the most influential CP researchers of the 19th century, famed psychoanalyst Sigmund Freud, has been credited with developing some of the most influential and accurate theories on cerebral palsy. Between 1891 and 1897 Sigmund Freud wrote several works titled “Cerebral Palsy”.  He also developed a classification system still in use today including congenital, during birth, and acquired post-natal forms of cerebral palsy. Freud made his observations by examining brains from patients with the diagnosis of hemiplegia and diplegia. Unlike Little, Freud believed that all “diplegias” attributed to birthing abnormalities, actually had their pathological origin during intrauterine life.(3) Freud’s research into cerebral palsy paved the way for many of the field’s future advances.

The belief that birth complications cause most cases of cerebral palsy was widespread among physicians, families, and even medical researchers until very recently. In the 1980′s scientists analyzed extensive data from a government study of more than 35,000 births and found that complications during labor account for only a fraction of cases of cerebral palsy (probably less than 10 percent) rather than the majority of cases. These findings from the National Institute of Neurological Disorders and Stroke (NINDS) perinatal study have profoundly altered medical theories about cerebral palsy and have spurred researchers to explore alternative causes.(4)

After Freud, in the early 1900′s there was much less interest or published research about cerebral palsy. The next big advancement for the CP community came in the 1930′s. Winthrop Phelps, an orthopedic surgeon in Baltimore, gave rise to modern orthopedics for cerebral palsy. He was the first clinician to treat patients with CP and to describe cerebral birth injuries in an orthopedic context instead of a neurological one. (5)

Since the 1940s, different groups, such as the founders of the American Academy for Cerebral Palsy and Developmental Medicine and The Little Club in the UK, have have helped advance the way professionals articulate concepts and descriptions of CP. Their efforts have led to people with CP having better access to treatment, services, advocacy, and research efforts. The definition of CP was revisited in 1964 and again in 2004 during an international workshop in Bethesda, MD (USA). “The task of the attendees [at this workshop] was to revisit and, if possible, update the definition and classification of CP in the light of emerging understanding of developmental neurobiology and changing concepts about impairments, functional status, and ‘participation’.” Discussions at this workshop revolved around establishing a definition and classification of CP that would meet the needs of clinicians, investigators, health officials, families and the public and would provide a common language for improved communication.(6) This workshop led to this short definition of cerebral palsy (which is elaborated upon in several pages of the published article):

 The 2004 consensus meeting led to the following definition of cerebral palsy:

“Cerebral palsy (CP) describes a group of disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, cognition, communication, perception, and/or behaviour, and/or by a seizure disorder.”

The way cerebral palsy is defined is a dynamic discussion among clinicians, researchers, and health professionals. As we learn more about cerebral palsy and its pathways of causation, we will likely see that the definition continues to evolve and change to reflect new knowledge.

Source Citations:

1. Panteliadis C., P Panteliadis, F. Vassilyadi  “Hallmarks in the history of cerebral palsy: From antiquity to mid-20th century” Brain & Development, 2013: 35: 285.

2. Little W. Course of lectures on the deformities of the human frame. Lancet 1843; 41: 350-354.

3. Freud S. Zur Kenntnis der cerebralen Diplegien des Kindesalters. Vienna: Deuticke; 1893.

4. From the Reaching for the Stars a Foundation of Hope for Children with Cerebral Palsy-www.reachingforthestars.org section “About CP”.

5. Panteliadis C., P Panteliadis, F. Vassilyadi  “Hallmarks in the history of cerebral palsy: From antiquity to mid-20th century” Brain & Development, 2013: 35: 289.

6. Bax MGoldstein MRosenbaum PLeviton APaneth NDan BJacobsson BDamiano DExecutive Committee for the Definition of Cerebral Palsy. “Proposed definition and classification of cerebral palsy”, April, 2005. Developmental Medicine and Child Neurology. 2005 Aug;47 (8): 571.