1. Cerebral Palsy Margie Ream MD, PhD Assistant Professor, Pediatric Neurology

2. Objectives: stuff to know about cerebral palsy  Definition, incidence and timing of insult  Diagnosis, exam findings  Classification of CP  Differential Diagnosis and comorbidities  Treatment  Prognosis  Quiz

3. Cerebral Palsy - definition  The diagnosis requires three conditions: 1) Motor difficulty 2) Not progressive 3) Injury to the immature brain (prenatal up to ≈2 yr) “Non-progressive disorder of posture or movement caused by a lesion in the developing brain.” Notice that the definition does not comment on intellectual ability.

4. Incidence and prevalence  2-5/1,000 births  At 12 months old 5/1000  At 7 years old 2/1000  Some kids “grow out of it” and some kids don’t get diagnosed until 2-3 years old

5. Timing of insult leading to CP Term babiesPretermExamples Prenatal24%6%Maternal and placental factors Perinatal20%50%HIE, trauma, cerebral hemorrhage, hyperbili Postnatal8%2%CNS infection, accident, abuse combination20%23% No known cause30%17% Taft 1995 <32 weeks, <2500g

6. Diagnosis  Delayed motor development.  May present as “floppy baby” and later develop increase tone.  Increased tendon reflexes  sustained ankle clonus – always abnormal  any ankle clonus after the first few months  increased sensory input zone for reflexes (percuss over deltoid and get biceps)  cross adduction at the patellar reflexes.

7. Normal Developmental Milestones

8. Exam findings  Persistent primitive reflexes:  Asymmetric tonic neck – should never be obligatory (held >30 sec) and should disappear by 6 months.  Crossed extensor reflex – stimulate foot of extended leg. Contralateral leg will flex then extend and adduct. Normal up to 4 months.  Positive supporting reaction – baby looks like he is stepping, should disappear by 4 months.  Moro should disappear by 6 months. Crossed extensor reflex. Intranet.tdmu.edu Stepping reflex. Artship.org Asymmetri c tonic neck reflex. Taft, 1995 Moro reflex. library.med. utah.edu/

9. Classification of CP  Spastic (70-80%)  Diplegia (legs) - #1  Hemiparesis  Quadriparesis  Dyskinetic (10-15%)  Athetosis, chorea, ballismus, tremor  Ataxic (1%)  Mixed (10-15%)  Rigid (5%) Di = 2 Hemi = half Quad = four Paresis = weak Plegia = paralyzed Di = 2 Hemi = half Quad = four Paresis = weak Plegia = paralyzed

10. Spastic Diplegia  Most common form. Affects legs >>> arms.  Delayed sitting and crawling. May prefer to “army crawl”  Leg scissoring with increased tone, increased reflexes in legs.  May not be obvious until 1 year old  Risk of hip subluxation or dislocation Studyblue.com

11. Upper body strength/coordination far exceeds that in her lower body. www.youtube.com/watch?v=mHXNjZS74s Spastic diplegia video

12. Child with spastic diplegia undergoing gait analysis using a walker. Notice toe walking. www.youtube.com/watch?v=TP37l54UqTE

13. Spastic Diplegia  Common cause is IVH in premature babies leading to periventricular leukomalacia affecting the motor tracks for legs.

14. Spastic Quadriplegia  All limbs affected, legs > arms  Double hemiplegia if arms>legs  Hypotonia early after injury  Profound motor delay  Dysphagia/dysarthria common  Seizures in 50% Abc.net.au

15. Hemiplegic Cerebral Palsy  Affects one side of the body, arm>leg.  First noticed around 4 months as hand fisting and contralateral hand preference.  Increased tone and reflexes noted by 15 months  tight elbow flexors, wrist pronators, gastrocnemius  Causes:  Stroke  IVH  Cerebral malformations Physio-pedia.com

16. Hemiplegic CP video Listen to narration then turn down volume and play video – we weren’t able to remove the sound. Notice the upper motor neuron pattern of weakness in which she keeps right arm flexed and has decreased arm swing. Also she doesn’t flex at the hip as much on the right when walking. https://www.youtube.com/watch?v=3w2IhGqIPAU

17. Less common forms of CP  Athetoid dystonic CP:  Prior to Rhogam was caused by hyperbilirubinemia/kernicterus. Basal ganglia and auditory centers are sensitive to bilirubin toxicity. Often comorbid hearing loss. Typical course was initial extensor posturing/opisthotonis, then hypotonia with obligatory tonic neck reflex. Athetosis and dystonia at 12-18 months, then rigidity by 2 yrs.  Now most commonly caused by hypoxia- ischemia in basal ganglia (HIE) Quizlet.com

18. Athetoid CP video Athetoid movements are slow and writhing, usually distal. https://www.youtube.com/watch?v=NXuwX7UBOlc

19. Less common forms of CP  Ataxic-spastic CP  Low tone with normal DTR, then develop wide based support and hyperreflexia.  Can be due to hydrocephalus. Ataxia can represent early signs of metabolic disorder so watch for progression and be suspicious.

20. Differential diagnosis of CP

21. Comorbidities of CP  Intellectual disability in 50-60%  Seizures in up to 1/2  Vision, hearing, sensory impairments  Feeding/secretion difficulty  Constipation, incontinence  Pain and dislocation from contractures  Osteopenia  Communication difficulties even with normal IQ  Spastic bladder  Depression  Skin breakdown

22. Treatment of Spasticity  Medical management  Oral meds (Baclofen, Benzodiazepines)  Botulinum toxin injections  Baclofen pumps  Surgical management  Dorsal rhizotomy (cut dorsal root from L1-S2)  Bracing or osteotomy for hip dislocation  Tendon release Rch.org.au

23. Treatment of comorbidities  Monitor feeding difficulty and airway protection  Consider g-tube in some patients  Assistive communication devices  Antiepileptics if needed  PT, OT, ST  Educational and social support Uakron.edu

24. Prognosis of CP  It is difficult to give prognosis, especially based on <12 mo exam  20-30% of children with CP at 12 months outgrow the diagnosis.  Some deficits may not be apparent until 2-3 years old.  Good prognosis for ambulation if sitting independently by 1.5-2 years  If can sit by 2-4 years, most can walk.  Not sitting by 4 years  no ambulation later.

25. References  Taft, Lawrence. Cerebral Palsy. Pediatrics in Review. 1995.  Krigger, Karen. Cerebral Palsy: An Overview. American Family Physician. 2006.  Clinical Pediatric Neurology: A Signs and Symptoms Approach, Gerald Fenichel  eMedicine  Medscape Bailer and White. Nature Reviews Drug Discovery 9, 68-82 (January 2010)

27. Thank you! For questions please contact Dr. Ream at Margie.Ream@nationwidechildrens.org

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