Cerebral Palsy Clinical Presentation
- Author: Hoda Z Abdel-Hamid, MD; Chief Editor: Amy Kao, MD more...
The child with cerebral palsy can present after failing to meet expected developmental milestones or failing to suppress obligatory primitive reflexes. The 2003 American Academy of Neurology (AAN) practice parameter suggests screening for the following potential cerebral palsy–associated deficits at the initial assessment :
Ophthalmologic and hearing impairments
Speech and language disorders
The diagnosis begins with a history of gross motor developmental delay in the first year of life. Cerebral palsy frequently manifests as early hypotonia for the first 6 months to 1 year of life, followed by spasticity.
Abnormal muscle tone is the most frequently observed symptom. The child may present as either hypotonic or, more commonly, hypertonic with either decreased or increased resistance to passive movements, respectively. Children with cerebral palsy may have an early period of hypotonia followed by hypertonia. The longer the period of hypotonia before hypertonia, the greater the likelihood that the hypertonia will be more severe.
Definite hand preference before age 1 year is a red flag for possible hemiplegia. Asymmetric crawling or failure to crawl may also suggest cerebral palsy. Growth disturbance is often noted in children with cerebral palsy, especially failure to thrive.
The general medical history should include a review of systems to evaluate for the multiple complications that can occur with cerebral palsy (see Complications under Prognosis).
The prenatal history should include information on the mother's pregnancy, such as prenatal exposure to illicit drugs, toxins, or infections; maternal diabetes; acute maternal illness; trauma; radiation exposure; prenatal care; and fetal movements.
A history of early frequent spontaneous abortions, parental consanguinity, and a family history of neurologic disease (eg, hereditary neurodegenerative disease) is also important.
The perinatal history should include the child's gestational age (ie, degree of prematurity) at birth, presentation of the child and delivery type, birth weight, Apgar score, and complications in the neonatal period (eg, intubation time, presence of intracranial hemorrhage on neonatal ultrasonogram, feeding difficulties, apnea, bradycardia, infection, and hyperbilirubinemia).
The child's developmental history should review his/her gross motor, fine motor, language, and social milestones from birth until the time of evaluation.
Gross motor milestones of concern with cerebral palsy include head control at age 2 months, rolling at age 4 months, sitting at age 6 months, and walking at age 1 year. Infants with cerebral palsy may have significantly delayed gross motor milestones or show an early hand preference when younger than 1.5 years, suggesting the relative weakness of one side (eg, reaching unilaterally).
The presence of an unexplained regression would be more suggestive of a hereditary neurodegenerative disease than cerebral palsy.
Current social skills, academic performance, and participation in an early intervention program (if < 3 y) or school support (if >3 y) should be reviewed, including resource room assistance; physical, occupational, and speech and language therapy; and adaptive physical education.
Standardized cognitive and educational testing and a current individualized education plan can be used to determine whether speech therapy, occupational therapy, and physical therapy are in place or whether referrals for these are needed.
Review the patient's equipment or need for equipment such as adaptive and communication devices (eg, computer-assisted speech programs), orthotics (eg, ankle-foot orthoses, walkers, wheelchair), and/or seating (may require straps to keep in place). See Rehabilitation and Cerebral Palsy.
Physical indicators of cerebral palsy include joint contractures secondary to spastic muscles, hypotonic to spastic tone, growth delay, and persistent primitive reflexes.
The initial presentation of cerebral palsy includes early hypotonia, followed by spasticity. Generally, spasticity does not manifest until at least 6 months to 1 year of life. The neurologic evaluation includes close observation and a formal neurologic examination.
Before the formal physical examination, observation may reveal abnormal neck or truncal tone (decreased or increased, depending on age and type of cerebral palsy); asymmetric posture, strength, or gait; or abnormal coordination.
Patients with cerebral palsy may show increased reflexes, indicating the presence of an upper motor neuron lesion. This condition may also present as the persistence of primitive reflexes, such as the Moro (startle reflex) and asymmetric tonic neck reflexes (ie, fencing posture with neck turned in same direction when one arm is extended and the other is flexed). Symmetric tonic neck, palmar grasp, tonic labyrinthine, and foot placement reflexes are also noted. The Moro and tonic labyrinthine reflexes should extinguish by the time the infant is aged 4-6 months; the palmar grasp reflex, by 5-6 months; the asymmetric and symmetric tonic neck reflexes, by 6-7 months; and the foot placement reflex, before 12 months. Cerebral palsy may also include the underdevelopment or absence of postural or protective reflexes (extending arm when sitting up). For a good discussion of this topic, see Capute AJ, Accardo PJ, eds. Developmental Disabilities in Infancy andChildhood. 2nd ed.2001;95-100.
The overall gait pattern should be observed and each joint in the lower extremity and upper extremity should be assessed, as follows:
Hip – Excessive flexion, adduction, and femoral anteversion make up the predominant motor pattern. Scissoring of the legs is common in spastic cerebral palsy.
Knee – Flexion and extension with valgus or varus stress occur.
Foot – Equinus, or toe walking, and varus or valgus of the hindfoot is common in cerebral palsy.
Gait abnormalities may include the crouch position with tight hip flexors and hamstrings, weak quadriceps, and/or excessive dorsiflexion.
Spastic (pyramidal) cerebral palsy
Patients with spastic (pyramidal) cerebral palsy evidence spasticity (ie, a velocity-dependent increase in tone) and constitute 75% of patients with cerebral palsy. Patients have signs of upper motor neuron involvement, including hyperreflexia, clonus, extensor Babinski response, persistent primitive reflexes, and overflow reflexes (crossed adductor). This may be observed by the child's tendency to keep the elbow in a flexed position or the hips flexed and adducted with the knees flexed and in valgus, and the ankles in equinus, resulting in toe walking.
Dyskinetic (extrapyramidal) cerebral palsy
Dyskinetic (extrapyramidal) cerebral palsy is characterized by extrapyramidal movement patterns, abnormal regulation of tone, abnormal postural control, and coordination deficits. Abnormal movement patterns may increase with stress or purposeful activity. Muscle tone is usually normal during sleep. Intelligence is normal in 78% of patients with athetoid cerebral palsy. A high incidence of sensorineural hearing loss is reported. Patients often have pseudobulbar involvement, with dysarthria, swallowing difficulties, drooling, oromotor difficulties, and abnormal speech patterns. Thus, the classic physical presentations of dyskinetic cerebral palsy include the following:
Early hypotonia with movement disorder emerging at age 1-3 years
Arms more affected than legs
Deep tendon reflexes usually normal to slightly increased
Risk of deafness in those affected by kernicterus
These patients with dyskinetic cerebral palsy may have decreased head and truncal tone and defects in postural control and motor dysfunction such as athetosis (ie, slow, writhing, involuntary movements, particularly in the distal extremities), chorea (ie, abrupt, irregular, jerky movements) or choreoathetosis (ie, combination of athetosis and choreiform movements), and dystonia (ie, slow, sometimes rhythmic movements with increased muscle tone and abnormal postures, eg, in the jaw and upper extremities)
Spastic hemiplegic cerebral palsy
Hemiplegia is characterized by weak hip flexion and ankle dorsiflexion, an overactive posterior tibialis muscle, hip hiking/circumduction, supinated foot in stance, upper extremity posturing (that is, often held with the shoulder adducted, elbow flexed, forearm pronated, wrist flexed, hand clenched in a fist with the thumb in the palm), impaired sensation, impaired 2-point discrimination, and/or impaired position sense. Some cognitive impairment is found in about 28% of these patients. Thus, spastic hemiplegic cerebral palsy includes the following classic physical presentations:
One-sided upper motor neuron deficit
Arm generally affected more than leg; possible early hand preference or relative weakness on one side; gait possibly characterized by circumduction of lower extremity on the affected side
Specific learning disabilities
Possible unilateral sensory deficits
Visual-field deficits (eg, homonymous hemianopsia) and strabismus
Spastic diplegic cerebral palsy
Patients with spastic diplegia often have a period of hypotonia followed by extensor spasticity in the lower extremities, with little or no functional limitation of the upper extremities. Patients have a delay in developing gross motor skills. Spastic muscle imbalance often causes persistence of infantile coxa valga and femoral anteversion. Cognitive impairment is present in approximately 30% of spastic diplegic patients. Spastic diplegic cerebral palsy includes the following classic physical presentations:
Upper motor neuron findings in the legs more than the arms
Scissoring gait pattern with hips flexed and adducted, knees flexed with valgus, and ankles in equinus, resulting in toe walking
Learning disabilities and seizures less commonly than in spastic hemiplegia
Spastic quadriplegic cerebral palsy
Most patients with spastic quadriplegic cerebral palsy have some cognitive impairment and demonstrate the following classic physical presentations:
All limbs affected, either full-body hypertonia or truncal hypotonia with extremity hypertonia
Increased risk of cognitive difficulties
Multiple medical complications (see Complications under Prognosis)
Legs generally affected equally or more than arms
Categorized as double hemiplegic if arms more involved than legs
Simpson DM, Gracies JM, Graham HK, Miyasaki JM, Naumann M, Russman B, et al. Assessment: Botulinum neurotoxin for the treatment of spasticity (an evidence-based review): report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2008 May 6. 70(19):1691-8. [Medline].
Scholtes VA, Dallmeijer AJ, Knol DL, Speth LA, Maathuis CG, Jongerius PH, et al. The combined effect of lower-limb multilevel botulinum toxin type a and comprehensive rehabilitation on mobility in children with cerebral palsy: a randomized clinical trial. Arch Phys Med Rehabil. 2006 Dec. 87(12):1551-8. [Medline].
Dai AI, Wasay M, Awan S. Botulinum toxin type A with oral baclofen versus oral tizanidine: a nonrandomized pilot comparison in patients with cerebral palsy and spastic equinus foot deformity. J Child Neurol. 2008 Dec. 23(12):1464-6. [Medline].
Yang EJ, Rha DW, Kim HW, Park ES. Comparison of botulinum toxin type A injection and soft-tissue surgery to treat hip subluxation in children with cerebral palsy. Arch Phys Med Rehabil. 2008 Nov. 89(11):2108-13. [Medline].
Pascual-Pascual SI, Pascual-Castroviejo I. Safety of botulinum toxin type A in children younger than 2 years. Eur J Paediatr Neurol. 2009 Nov. 13(6):511-5. [Medline].
Hoving MA, van Raak EP, Spincemaille GH, Palmans LJ, Becher JG, Vles JS. Efficacy of intrathecal baclofen therapy in children with intractable spastic cerebral palsy: a randomised controlled trial. Eur J Paediatr Neurol. 2009 May. 13(3):240-6. [Medline].
Trost JP, Schwartz MH, Krach LE, Dunn ME, Novacheck TF. Comprehensive short-term outcome assessment of selective dorsal rhizotomy. Dev Med Child Neurol. 2008 Oct. 50(10):765-71. [Medline].
Mutch L, Alberman E, Hagberg B, Kodama K, Perat MV. Cerebral palsy epidemiology: where are we now and where are we going?. Dev Med Child Neurol. 1992 Jun. 34(6):547-51. [Medline].
Bax M, Goldstein M, Rosenbaum P, Leviton A, Paneth N, Dan B, et al. Proposed definition and classification of cerebral palsy, April 2005. Dev Med Child Neurol. 2005 Aug. 47(8):571-6. [Medline].
Shevell MI, Bodensteiner JB. Cerebral palsy: defining the problem. Semin Pediatr Neurol. 2004 Mar. 11(1):2-4. [Medline].
Stanley F, Blair E, Alberman E. Cerebal Palsies: Epidemiology and Causal Pathways. London, United Kingdom: MacKeith Press; 2000.
Jacobsson B, Hagberg G. Antenatal risk factors for cerebral palsy. Best Pract Res Clin Obstet Gynaecol. 2004 Jun. 18(3):425-36. [Medline].
Odding E, Roebroeck ME, Stam HJ. The epidemiology of cerebral palsy: incidence, impairments and risk factors. Disabil Rehabil. 2006 Feb 28. 28(4):183-91. [Medline].
Russman BS, Ashwal S. Evaluation of the child with cerebral palsy. Semin Pediatr Neurol. 2004 Mar. 11(1):47-57. [Medline].
Doyle LW, Crowther CA, Middleton P, Marret S, Rouse D. Magnesium sulphate for women at risk of preterm birth for neuroprotection of the fetus. Cochrane Database Syst Rev. 2009 Jan 21. CD004661. [Medline].
Rouse DJ, Hirtz DG, Thom E, Varner MW, Spong CY, Mercer BM, et al. A randomized, controlled trial of magnesium sulfate for the prevention of cerebral palsy. N Engl J Med. 2008 Aug 28. 359(9):895-905. [Medline]. [Full Text].
Conde-Agudelo A, Romero R. Antenatal magnesium sulfate for the prevention of cerebral palsy in preterm infants less than 34 weeks' gestation: a systematic review and metaanalysis. Am J Obstet Gynecol. 2009 Jun. 200(6):595-609. [Medline].
Volpe JJ. Neurology of the Newborn. 4th ed. Philadelphia, Pa: WB Saunders; 2001. 4.
Moster D, Wilcox AJ, Vollset SE, Markestad T, Lie RT. Cerebral palsy among term and postterm births. JAMA. 2010 Sep 1. 304(9):976-82. [Medline].
Papile LA, Burstein J, Burstein R, Koffler H. Incidence and evolution of subependymal and intraventricular hemorrhage: a study of infants with birth weights less than 1,500 gm. J Pediatr. 1978 Apr. 92(4):529-34. [Medline].
Nelson KB. Can we prevent cerebral palsy?. N Engl J Med. 2003 Oct 30. 349(18):1765-9. [Medline].
American College of Obstetricians and Gynecologists, American Academy of Pediatrics. Neonatal Encephalopathy and Cerebral Palsy: Defining the Pathogenesis and Pathophysiology. Washington, DC: American College of Obstetricians and Gynecologists; 2003. [Full Text].
Capute AJ, Accardo PJ, eds. Developmental Disabilities in infancy and Childhood. 2nd ed. Baltimore, Md: Brookes Publishing; 2001. Vol 2.:
Majnemer A, Mazer B. New directions in the outcome evaluation of children with cerebral palsy. Semin Pediatr Neurol. 2004 Mar. 11(1):11-7. [Medline].
Vincer MJ, Allen AC, Joseph KS, Stinson DA, Scott H, Wood E. Increasing prevalence of cerebral palsy among very preterm infants: a population-based study. Pediatrics. 2006 Dec. 118(6):e1621-6. [Medline].
Ancel PY, Livinec F, Larroque B, Marret S, Arnaud C, Pierrat V, et al. Cerebral palsy among very preterm children in relation to gestational age and neonatal ultrasound abnormalities: the EPIPAGE cohort study. Pediatrics. 2006 Mar. 117(3):828-35. [Medline].
Dolk H, Pattenden S, Johnson A. Cerebral palsy, low birthweight and socio-economic deprivation: inequalities in a major cause of childhood disability. Paediatr Perinat Epidemiol. 2001 Oct. 15(4):359-63. [Medline].
Strauss D, Shavelle R, Reynolds R, Rosenbloom L, Day S. Survival in cerebral palsy in the last 20 years: signs of improvement?. Dev Med Child Neurol. 2007 Feb. 49(2):86-92. [Medline].
Hemming K, Hutton JL, Colver A, Platt MJ. Regional variation in survival of people with cerebral palsy in the United Kingdom. Pediatrics. 2005 Dec. 116(6):1383-90. [Medline].
Hemming K, Hutton JL, Pharoah PO. Long-term survival for a cohort of adults with cerebral palsy. Dev Med Child Neurol. 2006 Feb. 48(2):90-5. [Medline].
Verrall TC, Berenbaum S, Chad KE, Nanson JL, Zello GA. Children with Cerebral Palsy: Caregivers' Nutrition Knowledge, Attitudes and Beliefs. Can J Diet Pract Res. 2000 Autumn. 61(3):128-134. [Medline].
Bax M, Tydeman C, Flodmark O. Clinical and MRI correlates of cerebral palsy: the European Cerebral Palsy Study. JAMA. 2006 Oct 4. 296(13):1602-8. [Medline].
Woodward LJ, Anderson PJ, Austin NC, Howard K, Inder TE. Neonatal MRI to predict neurodevelopmental outcomes in preterm infants. N Engl J Med. 2006 Aug 17. 355(7):685-94. [Medline].
Wyatt K, Edwards V, Franck L, Britten N, Creanor S, Maddick A, et al. Cranial osteopathy for children with cerebral palsy: a randomised controlled trial. Arch Dis Child. 2011 Jun. 96(6):505-12. [Medline].
Edwards P, Sakzewski L, Copeland L, Gascoigne-Pees L, McLennan K, Thorley M, et al. Safety of Botulinum Toxin Type A for Children With Nonambulatory Cerebral Palsy. Pediatrics. 2015 Nov. 136 (5):895-904. [Medline].
Blackmore AM, Boettcher-Hunt E, Jordan M, Chan MD. A systematic review of the effects of casting on equinus in children with cerebral palsy: an evidence report of the AACPDM. Dev Med Child Neurol. 2007 Oct. 49(10):781-90. [Medline].
Delgado MR, Hirtz D, Aisen M, et al. Practice parameter: pharmacologic treatment of spasticity in children and adolescents with cerebral palsy (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2010 Jan 26. 74(4):336-43. [Medline].
Muthusamy K, Recktenwall SM, Friesen RM, Zuk J, Gralla J, Miller NH, et al. Effectiveness of an anesthetic continuous-infusion device in children with cerebral palsy undergoing orthopaedic surgery. J Pediatr Orthop. 2010 Dec. 30(8):840-5. [Medline].
Du RY, McGrath CP, Yiu CK, King NM. Oral health behaviors of preschool children with cerebral palsy: a case-control community-based study. Spec Care Dentist. 2014 Nov-Dec. 34 (6):298-302. [Medline].
Anderson P. FDA Clears Stimulation System for Foot Drop in Children. Medscape Medical News. Jan 25 2013. Available at http://www.medscape.com/viewarticle/778221. Accessed: February 5, 2013.
Dabney KW, Lipton GE, Miller F. Cerebral palsy. Curr Opin Pediatr. 1997 Feb. 9(1):81-8. [Medline].
Girard S, Kadhim H, Roy M, Lavoie K, Brochu ME, Larouche A, et al. Role of perinatal inflammation in cerebral palsy. Pediatr Neurol. 2009 Mar. 40(3):168-74. [Medline].
Jones MW, Morgan E, Shelton JE, Thorogood C. Cerebral palsy: introduction and diagnosis (part I). J Pediatr Health Care. 2007 May-Jun. 21(3):146-52. [Medline].
Mattern-Baxter K. Effects of partial body weight supported treadmill training on children with cerebral palsy. Pediatr Phys Ther. 2009 Spring. 21(1):12-22. [Medline].