Mixed cerebral palsy

How does this happen?

Movement depends on at least two systems working together. One carries the command from the outer brain down to the muscles. Another, centered on the basal ganglia, smooths the command out and filters what is not wanted.

Injury that is confined to one system produces one type. Injury that crosses both produces a mix. That is why mixed cerebral palsy is more common after widespread injury, such as severe hypoxic-ischemic encephalopathy, extensive bleeding in a baby born early, or a serious infection of the brain. ²

Practically, a child may have stiff legs that scissor and, at the same time, a hand and face that make movements they did not intend. Each needs a different treatment, and the treatments pull against each other.

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

The causes are the same as for cerebral palsy generally, weighted toward the more widespread injuries.

• Severe hypoxic-ischemic encephalopathy affecting both deep structures and the outer brain. ⁴
• Extensive bleeding or white matter injury in a baby born very early. ⁵
• Brain infection in the newborn period.
• Malformations of the brain that formed during pregnancy.
• Genetic conditions. ¹

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.

• An unrecognized or unacted-on loss of oxygen during labor. ⁶
• Cooling not offered or started late in a baby who met the criteria for therapeutic hypothermia. ⁷
• Newborn infection not recognized and treated promptly. ⁸
• Severe jaundice not treated, which contributes the dyskinetic component. ⁹
• Preterm care standards not met, including magnesium sulphate for neuroprotection before 32 weeks and antenatal corticosteroids. ^{10 11}

Whether any of these caused a particular child's injury takes a physician expert reading the whole record.

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

Depends on the cause. After severe hypoxic-ischemic encephalopathy there may be low Apgar score scores, seizures and an abnormal examination. After very preterm birth, an abnormal head ultrasound.

The first week

• Seizures.
• Tone that is hard to describe, sometimes stiff and sometimes floppy in the same examination.
• Feeding difficulty.

Around 3 months

• Stiff limbs together with a trunk that has no tone.
• Arching.
• Movements of the hands or face that are not intended.

Around 6 months

• Not rolling. ¹²
• Stiffness in the legs alongside wandering, unintended movement in the arms.
• Head control that is inconsistent.

Around 12 months

• Not sitting independently. ¹²
• A mix of stiffness and unintended movement that varies through the day.

Toddler years

• Movement that is hard for the family to describe, because it changes with position, effort and mood.
• Speech and feeding difficulty, since mouth control is often affected.
• Therapists may disagree about the label, which is common and does not change the plan much.

School age

• Contractures from the spastic component, and painful posturing from the dystonic component.
• High likelihood of needing AAC.
• Fatigue and pain, both under-treated.

How is it diagnosed?

Diagnosis is clinical, using the same three-part approach as other cerebral palsy: MRI, a standardized motor assessment, and a neurological examination. ¹³

The extra step here is naming the components. Ask your team to write down which pattern is dominant, what else is present, and which one is causing the most functional difficulty right now. That answer drives the treatment plan and it changes over time.

MRI usually shows widespread injury, or injury in more than one region. ²

Genetic and metabolic testing is reasonable where the MRI does not explain the picture. ¹

What is the treatment?

The core difficulty in mixed cerebral palsy is that treating one component can worsen another.

• Reducing spasticity with medicine or surgery can leave a child with less to brace against, so unintended movement becomes more visible and function can drop.
• Medicine aimed at dystonia can increase floppiness and fatigue.

This is why plans here are built around a specific goal rather than around a diagnosis. Deciding "we want him to sit unsupported for 10 minutes at the table" gives everyone something to measure against. ¹⁴

Approaches used:

• Targeted botulinum toxin injection into a specific muscle, rather than broad tone reduction.
• Intrathecal baclofen, which can help both stiffness and dystonia in some children.
• Dystonia medicines, tried one at a time with a clear measure.
• Seating and positioning, which often does more than medicine.
• AAC, early.
• selective dorsal rhizotomy is generally not recommended where there is significant dystonia, because reducing tone can make the dystonia more disabling. Ask this question directly if surgery is proposed.

What is the long-term outlook?

The GMFCS level predicts everyday function better than the type. ³ Mixed cerebral palsy tends to follow more widespread injury, so it is more often associated with higher GMFCS levels, epilepsy, and communication needs.

As with dyskinetic cerebral palsy, intelligence can be typical while movement and speech are severely affected. Assume competence, get communication in place, and let the child show you. ¹

What does daily life look like?

Seating, communication technology, feeding support, medicines with trade-offs, and a therapy team that has to keep re-balancing.

The practical advice from families is to hold one clear goal at a time and judge every proposed treatment against it. Otherwise the number of moving parts becomes unmanageable.

School needs the communication system present and used, seating set up correctly in every room, and a plan for fatigue. Write all three into the IEP.

What does care cost over a lifetime?

No agency publishes a cost estimate for mixed cerebral palsy specifically. The CDC figure of about $921,000 in 2003 dollars is for cerebral palsy as a whole. ¹⁵ Mixed presentations tend to sit at the higher end because they more often involve communication technology, complex seating and personal care.

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

What can you do this week?

1. Ask which pattern is dominant and what else is present, in writing.
2. Ask which component is causing the most functional trouble right now.
3. Ask what the treatment trade-off is for anything being proposed.
4. Set one goal for the next three months with the therapy team.
5. Ask for an AAC assessment.
6. Refer to early intervention or the school district.

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 or physiatrist

• Which movement patterns does my child have, and which is dominant?
• If we reduce the stiffness, what happens to the other movements?
• Which one is limiting function most right now?
• What is the plan if the first medicine does not work?

For the surgeon, if surgery is proposed

• Does my child have a dystonic component, and how does that affect this operation?
• What specifically will be better afterward, and how will we measure it?
• What could get worse?

For the therapy team

• What is our one goal this quarter?
• How does seating change what my child can do with their hands?
• What do we do at home, and can you watch me try it?

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