Follow-Up Corner: Moving Toward Earlier Diagnosis of Cerebral Palsy

Overview

Historically, the diagnosis of cerebral palsy (CP) occurs at ~ 18 months of age. The current endeavor seeks to identify CP as early as 3 months of age and certainly before 6 months corrected age. Why? The impetus is to intervene during the period of neuroplasticity, which is greatest in early infancy. The overarching aim of early detection is provision of optimal developmental trajectories for children with interventions targeted not only to motor skills, but also to vision, hearing, sensory, pain, and sleep issues.

Instruments

What tools are utilized to provide early detection?

History: birth history, course in the intensive care nursery

Imaging: results of cranial ultrasounds or MRI if available

Neurological Examination: findings on an examination conducted in neonatal follow-up clinic or primary care office

Biomarkers:

• General Movement Assessment (GMA) - General movements are part of a spontaneous movement repertoire, present from fetal life to approximately 6 months of life. They are described as “ fluent and elegant,” waxing and waning in intensity, force, and speed (Ment Retard Dev Disabil Res rev 2005, 11(1): 61-67). With neurologic injury, movements become “monotonous and poor.” Two specific patterns are associated with CP:
  • persistent pattern of cramped synchronized movements
  • absence of fidgety movements
• Each has a sensitivity and specificity of 95%. The GM requires a 2-minute videotaping session to be reviewed by an individual certified in GM assessments. We initiated the GMA assessment in our Neonatal Follow-up Program in January 2020.
• Hammersmith Infant Neurological Examination (HINE) - This 26-item evaluation was described in a prior Follow-up Corner article. Briefly, it assesses cranial nerves, posture, quantity and quality of movements, muscle tone, and reflexes and reactions. It is conducted by trained providers and physical therapists and includes optimality or “cut-off” scores for predicting cerebral palsy in both preterm and term infants.

Comment

As reported in JAMA Pediatrics in 2017, the 3 tools with the best predictive validity for detecting CP before 5 months corrected age are:

1. Neonatal MRI (86%-89% sensitivity)
2. GMA (98% sensitivity)
3. HINE (90% sensitivity)

Currently in CHOP Neonatal Follow-up Programs we utilize the HINE. Based on tools described above we identify some infants as at “high risk for CP” as early as 3-4 months, with future evaluations to confirm the diagnosis or not. The CP early detection initiative at CHOP follow-up clinics is being spearheaded by Andrea Duncan, MD, site investigator for this Cerebral Palsy Foundation sponsored endeavor. 

Communicating the Diagnosis of Cerebral Palsy

Communicating the diagnosis of cerebral palsy to parents and families is a conversation that must be carried out with honesty and compassion. The following points regarding conduct of this communication were made at the Implementation of Early Detection and Intervention for Cerebral Palsy Conference in Spring 2018, and form the basis of our communication strategy.

Communicating the diagnosis of CP

• Be honest, realistic and hopeful
• Use simple words; offer numbers if parents want more details
• Do not be too general or use platitudes
• High risk as a diagnosis is OK … our conversations will continue and the diagnosis may be revisited
• Admit that we may be wrong
• Explain the whole spectrum for a child
• Explain why an early diagnosis is important
• Know that parents want earlier diagnosis; they prefer any information vs. none
• It is better to be wrong than not to have had the conversation

If you are making an early diagnosis …

• Recap the clinical history
• Review what is noted during the current evaluation
• Demonstrate the abnormalities as you found them during evaluation
• Make certain the parent agrees
• Tell the truth
• Share hope
• Let the family share the event

Families want you to …

• Have an upfront, transparent conversation
• Have both parents present
• Demonstrate respect for the child
• Allow quiet time
• Sit down
• Remind family of child’s strengths; convey hope
• Provide a follow-up interview
• Take lead from the family; allow them control
• Acknowledge privacy of the diagnosis
• Help them in how to convey diagnosis to others
• Ask them to write down their questions

Pitfalls include …

• Talking too much
• Beating around the bush
• Too much medical jargon
• Not being prepared for sadness or anger
• Assuming we know fears rather than asking
• Not sitting down
• Forgetting to acknowledge that parents may be too overwhelmed to have questions at that moment
• No support person available
• Having a prepared interpreter

Avoiding Pitfalls

• Listen
• Allow room for silence … WAIT … Why am I talking?
• Be honest regarding challenges and limits of our knowledge
• Give hope in specific ways
• Think about what your child can do rather than what they cannot
• Avoid prognostications
• Provide educational materials and support systems
• Establish follow-up

Next in Follow-up Corner: Parental feelings regarding early diagnosis of CP