How low can we go? Recognizing infants at high risk of cerebral palsy earlier SARAH MCINTYRE 1,2 1 Cerebral Palsy Alliance Research Institute, Sydney, NSW; 2 Telethon Kids Institute Perth, WA, Australia. doi: 10.1111/dmcn.12787 This commentary is on the original article by Granild-Jensen et al. on pages 931–935 of this issue.
Cerebral palsy (CP) is an umbrella term for motor impairment due to a non-progressive cerebral lesion acquired early in life. The majority of CP registers exist to report trends in birth prevalence for their region.1 Now that neuroprotective strategies are moving from research into clinical settings, CP registers are a powerful tool to evaluate the real world efficacy of such treatments, so an accurate count is extremely important. As such, CP registers do not report birth year prevalence until children’s CP is confirmed, typically at 5 years of age. The article by Granild-Jensen et al.2 reports that the median age at which CP was diagnosed in Denmark was 11 months. This suggests that the Danish national system of seven free health examinations and referral of all infants with suspected neurological conditions is working well. The predictors of an earlier diagnosis were increased motor severity, abnormal cognition, and epilepsy. It is not surprising that the more severe the outcome the earlier the CP label was given. But as the authors suggest, where does this leave us and can we do better? CP is not strictly a diagnosis but a clinical description, and families want the answers that a true diagnosis provides: a definitive test to rule it in or out, the cause, the prognosis, and the most appropriate treatment. The CP label is not able to do this. Clinical signs of CP are not present at the time of birth for almost all, and indeed may not appear for 6 to 18 months for approximately 50%. However, the good
news is that clinicians no longer need to wait for a child to be old enough to observe the clinical signs of CP to confidently label them ‘high risk of CP’.3 There are now motor and neurological assessments and imaging modalities, with high sensitivity and specificity to predict CP.3 Infants with moderate to severe encephalopathy (hypoxic-ischaemic or not) and those born extremely preterm receive specialist care and follow-up, and can be assessed with these instruments in the first 3 months of life.3 A high risk of CP label should entitle these infants to enter early intervention trials and programs, and concurrently allow time for metabolic and genetic testing to eliminate differential and degenerative disorders essential for a CP register confirmation. However, for half of all infants with CP their first clinical signs are missing motor milestones and/or hypertonicity. Further research is required to test the predictive properties of instruments that can be used up to age 2 in low-risk infants.4 There is limited evidence to support the established concept that the earlier we intervene with neurorehabilitation the better the motor outcome (much more exists for cognitive outcomes),4 and there are only a handful of clinical trials currently being conducted in this field. Intuitively these interventions should work best on the least impaired and Granild-Jensen et al. suggest that CP in these infants is being identified the latest. This paper is a timely reminder that we must reconsider our practices, use evidence-based assessments, and identify infants at high risk of CP as soon as possible, to provide an opportunity to answer the research question: does early neurorehabilitation reduce severity of motor impairment? Simultaneously, CP registers must stay vigilant after a high risk of CP label is given, and confirm CP at age 5 to accurately report birth prevalence trends and severity for total populations.
REFERENCES Meulengracht
4. Spittle A, Orton J, Anderson P, Boyd R, Doyle LW.
Report of the International Survey of Cerebral Palsy
Flachs E, Uldall P. Predictors of early diagnosis of cere-
Early developmental intervention programmes post-hos-
Registers and Surveillance Systems 2015. Sydney, NSW,
bral palsy from national registry data. Dev Med Child
pital discharge to prevent motor and cognitive impair-
Australia: Cerebral Palsy Alliance, 2015. Available from:
Neurol 2015; 57: 931–35.
ments in preterm infants. Cochrane Database Syst Rev
1. Goldsmith S, McIntyre S, Smithers-Sheedy H, et al.
2. Granild-Jensen
JB,
Rackauskaite
G,
http://impact.cerebralpalsy.org.au/wp-content/uploads/
3. Bosanquet M, Copeland L, Ware R, Boyd R. A system-
2015/04/International-Survey-CP-Registers-Report-2015.
atic review of tests to predict cerebral palsy in young
pdf (accessed 19 March 2015).
children. Dev Med Child Neurol 2013; 55: 418–26.
2012; 12: CD005495.
Commentaries
891
Cerebral palsy (CP) is an umbrella term for motor impairment due to a non-progressive cerebral lesion acquired early in life. The majority of CP registers exist to report trends in birth prevalence for their region.1 Now that neuroprotective strategies are moving from research into clinical settings, CP registers are a powerful tool to evaluate the real world efficacy of such treatments, so an accurate count is extremely important. As such, CP registers do not report birth year prevalence until children’s CP is confirmed, typically at 5 years of age. The article by Granild-Jensen et al.2 reports that the median age at which CP was diagnosed in Denmark was 11 months. This suggests that the Danish national system of seven free health examinations and referral of all infants with suspected neurological conditions is working well. The predictors of an earlier diagnosis were increased motor severity, abnormal cognition, and epilepsy. It is not surprising that the more severe the outcome the earlier the CP label was given. But as the authors suggest, where does this leave us and can we do better? CP is not strictly a diagnosis but a clinical description, and families want the answers that a true diagnosis provides: a definitive test to rule it in or out, the cause, the prognosis, and the most appropriate treatment. The CP label is not able to do this. Clinical signs of CP are not present at the time of birth for almost all, and indeed may not appear for 6 to 18 months for approximately 50%. However, the good
news is that clinicians no longer need to wait for a child to be old enough to observe the clinical signs of CP to confidently label them ‘high risk of CP’.3 There are now motor and neurological assessments and imaging modalities, with high sensitivity and specificity to predict CP.3 Infants with moderate to severe encephalopathy (hypoxic-ischaemic or not) and those born extremely preterm receive specialist care and follow-up, and can be assessed with these instruments in the first 3 months of life.3 A high risk of CP label should entitle these infants to enter early intervention trials and programs, and concurrently allow time for metabolic and genetic testing to eliminate differential and degenerative disorders essential for a CP register confirmation. However, for half of all infants with CP their first clinical signs are missing motor milestones and/or hypertonicity. Further research is required to test the predictive properties of instruments that can be used up to age 2 in low-risk infants.4 There is limited evidence to support the established concept that the earlier we intervene with neurorehabilitation the better the motor outcome (much more exists for cognitive outcomes),4 and there are only a handful of clinical trials currently being conducted in this field. Intuitively these interventions should work best on the least impaired and Granild-Jensen et al. suggest that CP in these infants is being identified the latest. This paper is a timely reminder that we must reconsider our practices, use evidence-based assessments, and identify infants at high risk of CP as soon as possible, to provide an opportunity to answer the research question: does early neurorehabilitation reduce severity of motor impairment? Simultaneously, CP registers must stay vigilant after a high risk of CP label is given, and confirm CP at age 5 to accurately report birth prevalence trends and severity for total populations.
REFERENCES Meulengracht
4. Spittle A, Orton J, Anderson P, Boyd R, Doyle LW.
Report of the International Survey of Cerebral Palsy
Flachs E, Uldall P. Predictors of early diagnosis of cere-
Early developmental intervention programmes post-hos-
Registers and Surveillance Systems 2015. Sydney, NSW,
bral palsy from national registry data. Dev Med Child
pital discharge to prevent motor and cognitive impair-
Australia: Cerebral Palsy Alliance, 2015. Available from:
Neurol 2015; 57: 931–35.
ments in preterm infants. Cochrane Database Syst Rev
1. Goldsmith S, McIntyre S, Smithers-Sheedy H, et al.
2. Granild-Jensen
JB,
Rackauskaite
G,
http://impact.cerebralpalsy.org.au/wp-content/uploads/
3. Bosanquet M, Copeland L, Ware R, Boyd R. A system-
2015/04/International-Survey-CP-Registers-Report-2015.
atic review of tests to predict cerebral palsy in young
pdf (accessed 19 March 2015).
children. Dev Med Child Neurol 2013; 55: 418–26.
2012; 12: CD005495.
Commentaries
891
