Dyskinetic cerebral palsy

How does this happen?

The basal ganglia act as the brain's smoothing system. They do not start movements. They filter out the ones that are not wanted and keep the wanted ones steady.

These structures have a very high metabolic rate, which means they burn oxygen fast and have almost no reserve. When oxygen drops suddenly and completely, they are among the first areas injured. ²

bilirubin injures the same area by a different route. Bilirubin that rises high enough crosses into the brain and is toxic to the basal ganglia and to the hearing pathways. That combination, movement disorder plus hearing loss plus problems with upward gaze, is the classic picture of kernicterus. ³

With the filter damaged, unwanted movements get through. The result is movement the child does not intend, often worse when they are trying hardest, and usually gone during sleep.

What causes it?

Two different questions get mixed together here. The first is what leads to this injury in the body. The second is whether anyone could have prevented it. They are not the same question, and the answer to the first does not settle the second.

Happens even with perfect care

• A sudden, complete loss of oxygen from a placental abruption, a umbilical cord prolapse or a uterine rupture, which can happen without warning. ⁴
• Severe blood group incompatibility that causes rapid red cell breakdown even when Rh prevention was given correctly. ⁵
• Genetic and metabolic conditions that injure the basal ganglia. ¹
• Rare inherited bilirubin disorders in which levels rise despite normal care.

Associated with gaps in care

These are situations where published standards say what the care team should watch for and do. A gap here does not prove anyone caused your child's injury. It points to where the medical records will have an answer.

• Jaundice not measured or not acted on. Guidelines set treatment thresholds by hour of age with risk factors built in, and describe the follow-up timing after discharge. ³ See missed jaundice.
• Phototherapy started late, or exchange transfusion delayed once the level crossed the threshold. ³
• A newborn discharged early without a bilirubin check or a follow-up plan. ³
• Rh prevention not given to an Rh-negative mother when it was indicated. ⁵
• A prolonged or unrecognized loss of oxygen during labor. ⁶

Kernicterus is one of the few outcomes in this whole site with a clear, published, hour-by-hour prevention standard. That does not decide any individual case. It does mean the records will show the bilirubin values, the times they were drawn, and what was done next.

What are the signs, by age?

Signs change as a child grows. A newborn cannot show you a walking problem. Some children look fine at first and show differences months later.

At birth and the first hours

Nothing specific. Where the cause was oxygen loss, there may be low Apgar score scores, a low cord blood gas pH and an abnormal newborn examination. Where the cause is jaundice, the baby is usually well at birth and becomes yellow over the following days.

The first week

• Deepening yellow color, sleepiness, poor feeding, a high-pitched cry, or arching of the neck and back. These are late signs of very high bilirubin and they are an emergency. ³
• Seizures, if the cause was oxygen loss.
• Fluctuating tone.

Around 3 months

• Tone that changes from floppy to stiff and back.
• Arching backward, especially when upset or excited.
• Trouble feeding, with a tongue that pushes food out.
• Failing or missing the newborn hearing screen, in kernicterus. ⁷

Around 6 months

• Movements that are not intended, most visible in the hands and face when the child reaches for something.
• Difficulty holding a steady position for sitting.
• Head control that comes and goes.

Around 12 months

• Reaching that overshoots or wanders.
• Sitting that needs support because posture keeps shifting.
• Movements that get worse with effort or excitement and stop during sleep.

Toddler years

• Twisting postures of the trunk, neck or limbs, sometimes painful.
• Speech that is hard to understand because the same control problem affects the mouth and breath.
• Drooling.
• Frustration, which is often about being understood rather than about behavior.

School age

• Dystonia that can become painful and may need medicine or a pump.
• Communication needs that are best met by AAC.
• Learning that is often typical, and access that depends entirely on getting communication and seating right.

How is it diagnosed?

The diagnosis is clinical. A doctor watches the child move, at rest and while trying to do something, and looks for movements that are not intended, tone that fluctuates, and posturing that increases with effort. ¹

MRI typically shows injury in the basal ganglia and thalamus. In kernicterus the globus pallidus is characteristically affected on both sides. ²

Two tests belong in the workup and are sometimes forgotten.

• Hearing. Bilirubin injury affects the hearing pathway, and a specific pattern called auditory neuropathy can be present even when the newborn screen passed. Ask for a full audiology assessment, not only the newborn screen. ⁸
• Genetic and metabolic testing, especially if there is no clear injury on MRI and no history of oxygen loss or high bilirubin. ¹

If jaundice is part of the story, ask for every bilirubin value with the time it was drawn and the baby's age in hours at that moment. The thresholds are set by hour of age, so a value without a time cannot be interpreted. ³

What is the treatment?

Movement

• Medicines for dystonia, including trihexyphenidyl, baclofen, gabapentin and clonidine. Responses vary a great deal between children, so this is often a process of trials.
• An intrathecal baclofen pump for severe generalized dystonia.
• Deep brain stimulation is used in some centers for severe dystonia. The evidence in dyskinetic cerebral palsy is weaker than it is in genetic dystonias, so ask specifically what results are expected. ⁹
• Botulinum toxin for a specific troublesome muscle.

Seating and positioning

Seating matters more here than in almost any other type. A well-fitted supportive seat gives the trunk a stable base, and a stable trunk makes the hands more usable. This is a therapy intervention, not a comfort item.

Communication

Start AAC early. Children with dyskinetic cerebral palsy are the group most often underestimated, because the muscles used for speech are the ones most affected. Eye-gaze and switch access systems exist for children with very limited hand control.

Hearing

If hearing is affected, hearing technology and communication support come first, because everything else depends on access to language. ⁸

Therapy

Goal-directed practice aimed at something the family chose beats general handling. ⁹

What is the long-term outlook?

Movement is usually significantly affected, and intelligence often is not. That gap is the defining feature of this type, and it drives everything about how a child should be taught and treated. ¹

Many children with dyskinetic cerebral palsy use wheeled mobility and a communication device, and go on to read, learn and work. The single biggest predictor of how a child's life goes is often whether the people around them assumed there was a person in there from the beginning.

Dystonia can become painful in adolescence and adulthood, and pain here is under-recognized and treatable. Raise it directly. ¹

What does daily life look like?

Seating and positioning equipment, a communication device, and a lot of patience from everyone about time. Answers take longer to give when producing them is physical work.

Feeding is often hard. Some children need thickened liquids or a G-tube.

School needs to be set up so the child's communication system is present, charged and used in every class, not left in a bag. That belongs in the IEP in writing.

What does care cost over a lifetime?

The CDC lifetime estimate of about $921,000 in 2003 dollars is an average across all types of cerebral palsy. ¹⁰ Dyskinetic cerebral palsy tends to sit above the average because of communication technology, seating, and personal care needs.

If hearing loss is present as well, CDC's separate estimate for hearing loss was about $417,000 per person in 2003 dollars. These are separate population estimates and they cannot simply be added together for one child. ¹⁰

See the cost of care estimator for ranges with sources attached.

What can you do this week?

1. If jaundice was part of this, request every bilirubin result with the exact time and the baby's age in hours.
2. Ask for a full audiology assessment, including testing for auditory neuropathy, not just the newborn screen.
3. Ask for an AAC evaluation. Do not wait for speech to develop.
4. Ask for a seating assessment. Trunk support changes what the hands can do.
5. Ask what the MRI showed in the basal ganglia and thalamus.
6. Refer to early intervention today.

What should you ask each specialist?

Take these with you. Write the answers down in the moment, because you will not remember them later.

For the neurologist

• What did the MRI show in the basal ganglia and thalamus?
• Is this dystonia, athetosis, or both, and which is causing the most trouble?
• Which medicine would you try first, and how will we know if it is working?
• Should we do genetic or metabolic testing?
• Was hearing fully assessed, including auditory neuropathy?

For the therapy and AAC team

• What communication system should we start with, and how do we get it funded?
• How do we set up seating so the hands are freer?
• What do we do when movements get worse with excitement?

For the neonatologist or pediatrician, if jaundice was involved

• What were all the bilirubin values, and at what hour of age was each drawn?
• What was the treatment threshold for my baby at each of those times?
• When was phototherapy started, and was exchange transfusion considered?
• What was the discharge plan for rechecking bilirubin?

You can also build a printable list with the question generator.