Cerebral palsy describes a range of permanent motor function disorders arising from a one-time change to the fetal or infant brain.

Cerebral palsy symptoms are secondary to a brain injury or malformation before, during or shortly after birth, according to Christopher R. Barton, M.D., a pediatric neurologist with Norton Children’s Neuroscience Institute, affiliated with the UofL School of Medicine. Dr. Barton is also director of the Norton Children’s Neuroscience Institute Cerebral Palsy Clinic.

Understanding risk factors, their role in cerebral palsy diagnosis

Diagnosing cerebral palsy requires a three-step process, according to Dr. Barton.

“No. 1, do they have risk factors for cerebral palsy (including prematurity, birth complications, low birth weight, infections)? No. 2, has there been a motor dysfunction? And then, three, does the imaging make sense given the history and story?” Dr. Barton said.

Dr. Barton presented at the 22nd annual Pediatric Neurology Update continuing medical education opportunity.

Prenatal changes in the brain are responsible for 70% of cerebral palsy cases. The causes include hypoxia, stroke, toxins or drugs, TORCH infection, Rh disease, genetic mutation, and congenital malformation of the brain.

Birth and the first two years of life account for the other 30%, resulting from anoxia or hypoxia, traumatic delivery, prematurity, stroke, hemorrhage, and both accidental and nonaccidental trauma.

Congenital heart disease patients are at much higher risk for cerebral palsy, because they are more likely to have cardiac arrest or stroke, particularly if they’ve been on extracorporeal membrane oxygenation.

Risk factors for congenital cerebral palsy, according to the Centers for Disease Control and Prevention:

• Birth weight of less than 5 pounds, 8 ounces (2,500 grams)
• Born before the 37th week of pregnancy and especially before the 32nd week: Babies born very early are more likely to live than in the past, but many are at risk for cerebral palsy.
• Twins, triplets and other multiples have a higher risk, especially if one of the multiples dies before or shortly after birth. Multiples often are born preterm or with low birth weight.
• Chickenpox, rubella, cytomegalovirus and bacterial infections such as those involving the placenta, fetal membranes or maternal pelvis can lead to increases in cytokines.
• Kernicterus from untreated jaundice can cause cerebral palsy and other conditions. Sometimes, kernicterus results from ABO or Rh blood type difference between the mother and baby.
• Mothers with thyroid conditions, intellectual disability or seizures have a slightly higher risk of having a child with cerebral palsy.
• A birth injury such as detachment of the placenta, uterine rupture or issues with the umbilical cord can cause brain injury and result in cerebral palsy.

Factors that contribute to severity of cerebral palsy are the extent and location of brain injury or involvement. Depending on the location, however, even small injuries can cause significant issues. For example, an injury to the basal ganglia can cause severe symptoms, because it coordinates all movement.

Though imaging is important, MRI and clinical findings do not always correlate well. In fact, normal anatomy is present in 10% to 20% of cerebral palsy patients. Also, up to 25% of children who have significant lesions, such as periventricular leukomalacia, on MRI do not develop any neurologic disorders.

Looking at types of movement disorders and patterns, spastic cerebral palsy is the most common, followed by dyskinetic cerebral palsy, and then ataxic or mixed.

• Spastic cerebral palsy means velocity-dependent muscle stiffness and usually involves gray or white matter.
• Dyskinetic cerebral palsy (formerly known as choreoathetoid cerebral palsy) is marked by trouble controlling movement and results in sudden and twisting movements. Dyskinetic cerebral palsy is commonly the result of basal ganglia injuries and can present with multiple abnormal movements, including chorea, athetosis and dystonia.
• Ataxic cerebral palsy results in trouble with balance and coordination. It is caused by cerebellar injuries.
• A mixed phenotype can result from involvement in multiple parts of the brain.
• Hemiplegic cerebral palsy is much more common in patients who had a stroke in utero, or a hemorrhage or brain malformation. Usually, with hemiplegic cerebral palsy, the arm is affected more than the leg.
• Diplegic cerebral palsy is an upper motor neuron issue that mostly affects the legs and results in a scissoring gait and sometimes a crouching posture. Patients with diplegic cerebral palsy often also struggle with visual spatial reasoning.
• Quadriplegic spastic cerebral palsy is typified by low central tone and poor head control, making eating, sitting and swallowing difficult. Of these patients, 4 in 5 are not walkers long term.

Symptoms of cerebral palsy

Cerebral palsy symptoms in babies are lethargy, irritability, poor visual attention and poor head control early. Hand-fisting and a marked hand preference — where an infant uses one hand to the exclusion of the other — are additional signs.

Babies with cerebral palsy also can exhibit exaggerated development reflexes. For example, babies 4 to 6 months old with cerebral palsy will reflexively raise their knees up when held vertically. Often, they are very tight and may even cross their legs when held upright.

Watching for abnormal movement when a young child runs also can provide an excellent gauge of whether there has been a brain injury, according to Dr. Barton.

“A child may walk normally, but when they run, they’ll pick that arm up and not want to swing it. These are big clues to us. Maybe there was a brain injury,” he said.

Specialty referrals are key to long-term outcomes

In patients suspected of having cerebral palsy, referral by primary care physicians to physical therapy and pediatric neurology for an early diagnosis can make a big difference in long-term outcomes.

First Steps, a Kentucky program for children with a developmental delay or disability from birth to age 3 and their families, provides access to comprehensive services.

“So anytime you’re screening these patients, make sure you’re referring to First Steps, while also sending them to us, so we can confirm the diagnosis and get them started on the treatment they’ll need [in the] long term. Early therapy is most important.” Dr. Barton said.

Providing a multidisciplinary approach for cerebral palsy

The Norton Children’s Neuroscience Institute Cerebral Palsy Clinic is made up of board-certified, fellowship-trained physicians from across the Norton Children’s network of specialists who understand your child’s cerebral palsy. Our team aims to create a holistic, personalized treatment plan for each child’s unique needs once they are referred to the clinic.

Our multidisciplinary approach to care allows for a patient to be evaluated by several pediatric specialists all in the same visit. Key specialties include neurology, neurosurgery, orthopedics, physical therapy and pulmonology. Patients referred to the clinic are followed as they grow and develop symptoms that may change over time, such as spasticity.