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Cerebral Palsy - Initial Diagnosis
Overview
The initial assessment of a child for possible cerebral palsy (CP) should focus on determining the cause of the abnormal signs (e.g., spasticity or delay in achievement of milestones) that prompted concern. In some cases, the diagnosis of CP may be straightforward, while in others, the diagnosis may require prolonged observation and eliminating other causes for the observed signs; essentially in these cases CP becomes a diagnosis of exclusion. For roughly 20% of children with developmental delay and non-progressive motor involvement, a clear diagnosis cannot be determined. Particularly important are ruling out a progressive disease, which might require serial examinations, and genetic conditions that might occur in subsequent pregnancies.Presentations
The manifestations of CP in a given patient will depend on the extent, timing, and location(s) of abnormal brain development or injury. The age at which a child begins to exhibit signs of CP is determined by the type and amount of brain involvement and the stage of motor development affected by the altered brain function. Because motor development is easily assessed in early infancy, most children with pre- or perinatal causes of CP can be identified between 4 and 9 months of age. Some aspects of the condition may become more apparent over time (e.g., intellectual disability, extent of increased tone, orthopedic sequelae, seizures), although the brain injury itself is not progressive.There are many types of CP and many classification schemes. Even within a given classification scheme, there may be limited agreement among clinicians regarding the specific classification of an individual. However, some classification is important for communication among various providers. (Percentages of children with the various types of CP described below are from [Himmelmann: 2005].)
- Spastic CP: increased velocity-dependent muscle tone causing stiff and awkward movement. Almost 80% of people with CP have
spasticity. Children with spastic CP may be further described by the areas of the body affected:
- spastic diplegia (approximately 35% of children with CP): Motor impairment is most significant in the lower extremities. Arm, trunk, and facial musculature are often affected, but to a lesser extent.
- spastic hemiplegia (approximately 38%): One side of the body is affected, with the arm usually more involved than the lower extremity.
- spastic quadriplegia (approximately 6%): whole body involvement (face, trunk, legs, and arms).
- Dyskinetic CP (approximately 15% of children with CP): involves involuntary movements described as athetoid (slow, writhing), choreic (quick fast), or dystonic (longer, sustained muscle contractions, causing twisting movements or postures). These movement disorders generally involve most of the body and can involve the face and tongue, affecting speech. The dyskinetic group also includes hypotonic CP, identified in children with low muscle tone as their major manifestation. Dyskinetic CP is sometimes known as extrapyramidal CP because it results from injury to the basal ganglia and/or cerebellum. A classic example is kernicterus.
- Ataxic CP (approximately 6%): Results in problems with balance and depth perception, and may often overlap with other categories.
- Mixed CP: a term used when a child has features of more than one type. Examples include the combination of low truncal tone and poor head control and seating posture but with spasticity in the lower extremities or a mixture of spasticity and dystonia (pure dystonic CP is rare).
- Intellectual disability is found overall in about 2/3 of children with CP, with a higher percentage in those children with quadriparesis.
- Seizure disorders are common, found in about half of children with CP, and are more common in those with the quadriparetic and hemiplegic forms (in this latter form, seizures may not present until 3-5 years of age).
- Hydrocephalus - Some children with CP will have hydrocephalus, either as the cause of their CP or as an associated condition, e.g., from an intraventricular hemorrhage in a child born prematurely. New or increasing hydrocephalus may cause developmental regression or, if acute, symptoms such as headache and lethargy. See Hydrocephalus and VP shunts (general).
- Impaired growth
- Vision problems (including strabismus, myopia, cortical blindness and in children with hemiplegia a homonymous visual field defect) (see also Visual Impairment)
- Hearing problems
- Drooling/swallowing problems
- Incontinence, constipation - Approximately 25% of children with CP have primary urinary incontinence, [Odding: 2006] and constipation is very common.
- Speech/language defects
- Learning problems - Even in children with normal intelligence, such as in children with hemiplegic CP, IQ is often preserved but subtle learning concerns may become evident during grade school. Attention deficit disorder (with or without hyperactivity) is prevalent in this population and should be specifically inquired about once the child has reached the appropriate age.
Pearls And Alerts
Seizures are found in about 50% of children with CP and clinicians should ask about possible seizure activity.
Some children with CP will present initially with delayed development and no other signs. The developmental quotient (DQ) can be used to determine if the child's skill development is out of the broad range of normal. To calculate the DQ, divide the "motor age" (the average age of the child's current developmental milestones by a standardized test such as the Denver II) by the chronological age (corrected for prematurity). For example, the DQ of a 1 year old born at term who just started crawling (average age of attainment is 9 months) would be 9/12=75%. In general, a DQ above 70% with a normal neurologic exam and normal quality of movements suggests that development is in the broad range of normal. Also see Developmental Screening Tools (dbpeds.org).
Practice Guidelines
Ashwal S, Russman BS, Blasco PA, Miller G, Sandler A, Shevell M, Stevenson R.
Practice parameter: diagnostic assessment of the child with cerebral palsy: report of the Quality Standards Subcommittee of
the American Academy of Neurology and the Practice Committee of the Child Neurology Society.
Neurology.
2004;62(6):851-63.
PubMed abstract / Full Text
Differential Diagnosis
The differential diagnosis for CP is becoming increasingly complex as more metabolic and genetic conditions are recognized to have clinical characteristics similar to those in CP. [Hoon: 1997] In a child with CP of unknown cause, clinicians should consider several general categories of conditions other than CP. These include:- Slowly progressive conditions may present similarly to CP, including familial paraplegia which presents as spastic diplegia, and some of the leukodystrophies. See Slowly progressive disorders sometimes misdiagnosed as CP, adapted from [Fenichel: 2005]. Brain MRI and serial observations over time will help differentiate these conditions, as signs and symptoms may develop or become more obvious as the child gets older.
- Genetic syndromes causing developmental delay may become more obvious as the child ages (e.g., developmental delay, poor feeding, and hypotonia in an infant with Prader Willi syndrome) .
- Children with metabolic disease may present with CP-like symptoms, but may also include developmental regression, periods of emesis and dehydration, and failure to thrive.
- Children with neurologic conditions may present early in life as CP but the absence of reflexes, bowel and bladder dysfunction,
and muscle weakness (which can be difficult to differentiate from low tone) should distinguish these.
History And Examination
Family History
Ask about familial spastic paraplegia, dopa-responsive dystonia, and any neurologic disease in the extended family. Familial stroke and clotting or bleeding problems may be important. Family history of CP (which may not really be CP), other developmental delay, or miscarriages/infant death may suggest a specific etiology. Ask about family members with intellectual disability. If there are possible connections, the family of the child with CP should be asked to gather more information.Pregnancy/Perinatal History
Pregnancy, labor, delivery, and perinatal course should be detailed, including prematurity, birth trauma, APGAR scores, need for oxygen, feeding ability at and around birth, etc.Medical History
Questions regarding associated features will help the primary care physician identify areas of concern.Mobility/ADLs: Is the child able to move in an age appropriate manner (roll over, crawl, walk)? (also see Musculoskeletal Exam (
102 KB)
). How is fine motor control compared to other children their age? (e.g., self feed, grasp objects, dress self, write efficiently)?
Has there been any decrease in their mobility or abilities over time?Growth and nutrition: Are there any episodes of emesis? Has nutritional status, growth, or feeding been a concern? See Nutrition and growth in children with CP.
GI: Does the child have constipation and is he/she toilet trained? Does the child have problems that suggest gastroesophageal reflux disease (GERD)?
Swallowing and drooling: Does the child cough and/or choke during eating and drinking? Does it take a very long time to feed the child? Is swallowing or drooling a problem?
Pulmonary: Is there chronic cough or wheezing? Has the child had pneumonia? Does the child have difficulty sleeping? Does the child snore or have frequent awakenings?
Neurology: A history of developmental regression should prompt a referral to neurology as it raises the question of a different diagnosis. Has the child had seizures? See the Seizure module for more information. Does the child have any unusual movements or episodes of unexplained pain or agitation? Does the child have a history of hydrocephalus and/or a VP shunt?
Vision/hearing: Does the child have normal vision and hearing? Do they wear glasses or hearing aids?
Musculoskeletal/Bone: Has the child had fractures? Are there any limitations of joint or muscle movement?
Urinary: Although not common in CP, does the child have frequency, urgency, dribbling of urine? Also see Neurogenic Bladder (general).
Other: Is chronic pain a concern?
Developmental and Educational History
Review developmental milestones and at what age they were achieved. What are the child's current abilities? Parents' estimate of child's mental age? How does the child communicate (e.g., differential cries, eye gaze, signing, augmentative device, verbal).Social and Family History
What concerns does the family have? What kinds or levels of support can the extended family offer? Does the family have experience with chronic conditions? Developmental delay?Physical Exam
General
Assess alertness and responsiveness, posture, general tone, position and use of extremities, apparent nutritional status, and use of assistive devices such as a wheelchair, hearing aids, and glasses.Growth Parameters
Height | weight | head circumference: Growth parameter measurements are critical but may be difficulty to obtain accurately. See Nutrition and growth in children with CP. Parameters of children who were born prematurely may need to be plotted on preterm growth charts; if standard growth charts are used, correct for prematurity until 18-24 months of age.HEENT
Oral exam - the presence of dental caries may signal reflux and may cause food refusal or discomfort. Also look for a high arched palate and pooling of saliva at the back of the throat. Look for visual attentiveness, unequal light reflexes, strabismus.Neurologic Exam
Look first at the pattern of development and whether it has slowed, regressed, or plateaued. Does the child have abnormal patterns of motor movements? For example, does the child army crawl (lower extremity spasticity), scissor the lower extremities, appear to have early hand dominance (possible hemiplegia), use a Gower maneuver to stand (proximal weakness), have chorea or ataxia, or persistently walk on his/her toes (habitual pattern vs. spasticity)?Then, is the child's neurologic exam normal? Evaluate tone, strength, balance, primitive reflexes, coordination, reflexes. Classify findings by the type of tonal abnormality and the area(s) affected. Dystonia and spasticity can be difficult to differentiate but it is important to do so. See Classifying types of cerebral palsy for physical exam hints for the differentiation of spasticity and dystonia and a topographical classification system for spastic CP. Low tone in infants can be very difficult to differentiate from decreased strength. Check supine posture - infants with low tone will lie in the frog-leg position. Also check if the infant will slip through the examiner's hands under the infant's arms and whether there is head lag when pulling the infant up from supine. Infants with these problems have low tone but might still be able to kick with good strength if they are angry. Also check if the infant will bear weight when held in a standing position and, conversely, if the baby wants to keeps his/her legs extended much of the time.
Musculoskeletal
Look for contractures, scoliosis, hip dislocation. Also see Musculoskeletal Assessment in the Primary Care Setting ( 99 KB)
for an assessment tool.
Testing
Sensory Testing
Vision screening should be performed with referral to pediatric ophthalmology if problems are suspected. Strabismus and myopia are common, as is cortical blindness. Children with dyskinetic CP may have eye movement problems, such as nystagmus, that interfere with vision and children with hemiplegia may have a homonymous heminanopia. See Visual Impairment.Hearing screening should be performed with referral for audiologic evaluation if problems are suspected. Hearing deficits are common in children with CP.
Laboratory Testing
Routine laboratory testing is not indicated, however some specific situations may warrant focused lab evaluation:Metabolic - Testing should be considered in a child with developmental regression, periods of emesis/dehydration, or failure to thrive. In a child with dyskinetic or ataxic CP, consider the possibility of a metabolic disorder.
Hematologic - An evaluation for clotting dysfunction should be considered in children whose CP is secondary to an intra-uterine stroke. Testing for an inherited hypercoaguable state may include Factor V Leiden (Factor V G1691A), lupus anticoagulant/antiphospholipid antibodies, antithrombin antigen/activity, prothrombin mutation (G20210A), fibrinogen, factors VIII, IX, XI, fasting homocysteine, free and total protein S antigen, activated protein C resistance, protein C activity/antigen, plasminogen, and lipoproteins. [Raju: 2007]
Nutritional - Serum markers for nutritional status are of limited value. However, in the child with malnutrition, assessment of targeted vitamin and mineral stores should be considered. In particular, a significant percentage of children with CP demonstrate a deficiency in vitamin D.
Imaging and EEG
Neuro-imaging - Imaging in children with CP is frequently abnormal (62-100% [Ashwal: 2004]), and is usually performed as part of the initial diagnostic evaluation in a child with suspected CP.
In the child with hemiplegia, MRI may identify an underlying cause (e.g., in-utero stroke or focal brain malformation such as schizencephaly). In the child with spastic quadriplegia neuroimaging can rule-out white matter disorders which can be so slowly progressive that they mimic CP. Imaging does not need to be performed after the initial diagnosis unless a new clinical question arises (for example, a regression in abilities). Many brain malformations are known to be associated with genetic and or metabolic conditions and, if found, should trigger a referral to genetics (e.g. lissencephaly-Miller Dieker syndrome and Zellweger syndrome, [Ashwal: 2004], [Lequin: 1999]). Several excellent neuroradiology texts offer more information regarding neuroimaging abnormalities in CP. [Ball: 1997] [Barkovich: 2005] [Osborn: 1994]
Electroencephalogram (EEG) - An EEG is warranted if clinical evidence of a seizure disorder exists. Seizures are more common in children with hemiplegic and quadriplegic CP. Children with CP may have many types of seizures, including generalized tonic-clonic, partial complex, and atypical absence. It can be difficult to determine if some events in children with CP are seizures or behaviors. If the event occurs frequently, a video EEG may be helpful in sorting this out. See the Seizure module for more information.
Skeletal imaging - may be helpful to evaluate orthopedic concerns, most often scoliosis or hip dislocation.
Upper gastrointestinal study (UGI) - Used for assessment of gastroesophageal reflux (although sensitivity and specificity are moderate), delayed gastric emptying, and to rule out a structural cause of GI symptoms.
Genetic Testing
Genetic testing may be indicated if a syndrome or genetic disease is suspected. Also, consider a referral to genetics for testing if there is no explanation for the child's CP, or if there are other signs such as poor growth of height, weight, and head circumference, dysmorphic features, or otherwise unusual presentations.Subspecialist Collaborations and Other Resources
A child with CP often needs access to a large number of subspecialists and the Medical Home should be the initiator and coordinator of these visits with input from the family. The goal is to have the child see the subspecialists needed but to avoid duplication of services or unnecessary appointments.
Pediatric Neurology (see Services below for relevant providers)
The primary care provider might refer to neurology or developmental peds for confirmation of a diagnosis of CP. A neurology consultant can offer expertise in management of a seizure disorder or evaluation of new neurological changes.
Pediatric Medical Genetics (see Services below for relevant providers)
An evaluation with genetics might be helpful if a syndrome or genetic disease is suspected in a child without a clear history of pre-, peri-,or postnatal brain injury. Cerebral palsy can be associated with a number of syndromes (e.g., Angelman syndrome, FG syndrome). An increasing number of focal brain malformations have identifiable genetic patterns (e.g., lissencephaly, septo-optic dysplasia). Up to 50% of children with ataxic CP have a genetic cause.
Developmental Pediatrics (see Services below for relevant providers)
Developmental pediatric specialists provide neurodevelopmental consultation for the evaluation and diagnosis of children with developmental disabilities such as CP
Pediatric Physical Medicine & Rehab (see Services below for relevant providers)
An evaluation with a pediatric physiatrist will assist in improving functional quality of life through the prescribing of assistive devices, medication, and other modalities, including physical, occupational, and speech therapy as necessary for management and treatment of spasticity, feeding and language problems, problems with activities of daily living, etc.
Pediatric Orthopedics (see Services below for relevant providers)
Most children with CP and significant tonal abnormalities should be followed by an orthopedic specialist to monitor for contractures, scoliosis, and hip subluxation.
Pediatric Gastroenterology (see Services below for relevant providers)
Children with difficulty gaining weight, feeding problems, GERD, and severe constipation may benefit from referral to gastroenterology.
Neuropsychology (see Services below for relevant providers)
It is important to understand the cognitive abilities of each child with CP since the extent of motor impairment may not correlate with the extent of cognitive impairment. However, testing cognitive skills in children with motor and/or sensory impairments requires special expertise. Attention deficit disorder is common in this population and should be specifically looked for as part of psychological testing.
Pediatric Ophthalmology (see Services below for relevant providers)
As clinically indicated, keeping in mind that visual deficits and strabismus are common in CP. This is particularly important if the child does not have the ability to participate in age appropriate visual screening during well child checks. Children with hemiplegic CP should be examined for a homonymous visual field defect.
Pediatric Otolaryngology (see Services below for relevant providers)
To evaluate and treat swallowing difficulties, as well as to consider PE tubes if there are frequent ear infections which may be affecting hearing.
Pediatric Pulmonology (see Services below for relevant providers)
To assess difficulties the child may be having with cough, breathing during sleep, and frequent respiratory infections.
Resources
Information & Support
For Professionals
Information on spasticity, choreoathetosis, more (WE MOVE)
The Worldwide Education and Awareness for Movement Disorders.
Denver II
Commercial site offers ordering of Denver II developmental testing forms.
MD Virtual University: Pediatric Movement Disorders
The information is not specific to CP but includes information about CP.
For Parents and Patients
Support
United Cerebral Palsy
This national non-profit organization, provides information on a variety of topics including advocacy, conferences, insurance,
sexuality, policy, and other items for the public and providers.
Cerebral palsy support groups (Children's Disability Information)
A list of support groups for different types of CP as well as other resources
General
CP information (MedlinePlus)
from the National Library of Medicine; basic information and links to many additional sources of reliable information
NINDS Cerebral Palsy Information Page
A webpage of the National Institute of Neurological Disorders and Strokes (NINDS) providing a thorough overview of CP plus
links to multiple organizations, publications, and education tools. For physicians as well as patients and families
Practice Guidelines
Ashwal S, Russman BS, Blasco PA, Miller G, Sandler A, Shevell M, Stevenson R.
Practice parameter: diagnostic assessment of the child with cerebral palsy: report of the Quality Standards Subcommittee of
the American Academy of Neurology and the Practice Committee of the Child Neurology Society.
Neurology.
2004;62(6):851-63.
PubMed abstract / Full Text
Evidence based summary of indicated diagnostic evaluation for children with cerebral palsy.
Tools
Musculoskeletal Assessment in the Primary Care Setting
( 99 KB)
A concise, easy to use tool to document the limitations of a child's musculoskeletal abilities and exam.
Services
Developmental Pediatrics
Child Development Clinic,
more info...
44 Mario Capecchi Drive
Salt Lake City, UT 84114
Phone: 801-584-8510
Toll Free Phone: 800-829-8200
Fax: 801-584-8579
http://health.utah.gov/cshcn/cdc/
See all Developmental Pediatrics services providers (2) in our database.
Pediatric Gastroenterology
See all Pediatric Gastroenterology services providers (2) in our database.
Pediatric Medical Genetics
See all Pediatric Medical Genetics services providers (3) in our database.
Pediatric Physical Medicine & Rehab
See all Pediatric Physical Medicine & Rehab services providers (6) in our database.
For other services related to this condition, browse our Services categories or search our database.
Helpful Articles
PubMed Search on cerebral palsy: review articles for the last 2 years
Deon LL, Gaebler-Spira D.
Assessment and treatment of movement disorders in children with cerebral palsy.
Orthop Clin North Am.
2010;41(4):507-17.
PubMed abstract
Helpful information on distinguishing spasticity, rigidity, and dystonia in children with cerebral palsy.
Authors
| Authors: | Lisa Samson-Fang MD, 3/2008 Lynne M Kerr MD, PhD, 3/2008 |
| Content Last Updated: | 5/2011 |
Page Bibliography
Ashwal S, Russman BS, Blasco PA, Miller G, Sandler A, Shevell M, Stevenson R.
Practice parameter: diagnostic assessment of the child with cerebral palsy: report of the Quality Standards Subcommittee of
the American Academy of Neurology and the Practice Committee of the Child Neurology Society.
Neurology.
2004;62(6):851-63.
PubMed abstract / Full Text
Evidence based summary of indicated diagnostic evaluation for children with cerebral palsy.
Ball, William S., Jr.
Pediatric Neuroradiology.
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Barkovich, A. James.
Pediatric Neuroimaging.
Fourth Edition ed. Lippincott Williams and Wilkins;
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Fenichel, GM.
Clinical Pediatric Neurology: A Signs and Symptoms Approach.
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Himmelmann K, Hagberg G, Beckung E, Hagberg B, Uvebrant P.
The changing panorama of cerebral palsy in Sweden. IX. Prevalence and origin in the birth-year period 1995-1998.
Acta Paediatr.
2005;94(3):287-94.
PubMed abstract
Hoon AH Jr, Reinhardt EM, Kelley RI, Breiter SN, Morton DH, Naidu SB, Johnston MV.
Brain magnetic resonance imaging in suspected extrapyramidal cerebral palsy: observations in distinguishing genetic-metabolic
from acquired causes.
J Pediatr.
1997;131(2):240-5.
PubMed abstract
Case series of children with motor disorders in which the MRI provided diagnostic clues.
Lequin MH, Barkovich AJ.
Current concepts of cerebral malformation syndromes.
Curr Opin Pediatr.
1999;11(6):492-6.
PubMed abstract
Summarizes current knowledge of the genetics of brain malformations.
Odding, E, Roebroeck, ME, and Stam, HJ.
The Epidemiology of cerebral palsy: incidence, impairments, and risk factors.
UCP Research and Education Foundation Fact Sheet; (2006)
http://www.ucpresearch.org/fact-sheets/epidemiology-cerebral-palsy.php. Accessed on August 2008.
Osborn, Anne G.
Diagnostic Neuroradiology A Text/Atlas.
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Raju TN, Nelson KB, Ferriero D, Lynch JK.
Ischemic perinatal stroke: summary of a workshop sponsored by the National Institute of Child Health and Human Development
and the National Institute of Neurological Disorders and Stroke.
Pediatrics.
2007;120(3):609-16.
PubMed abstract
A summary of the NIH sponsored workshop on ischemic perinatal stroke; causes, evaluation, and research directions.