Cerebral Palsy


Article Author:
Jamika Hallman-Cooper


Article Editor:
William Gossman


Editors In Chief:
Michael Labanowski


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Trevor Nezwek
Radia Jamil
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Steve Bhimji
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
7/18/2019 5:27:16 PM

Introduction

Cerebral palsy is a group of permanent disorders affecting the development of movement and causing a limitation of activity. Non-progressive disturbances that manifest in the developing fetal or infant brain lead to cerebral palsy.[1] It is the most common cause of childhood disability. The degree and type of motor impairment and functional capabilities vary depending on the etiology. Cerebral palsy may have several associated comorbidities, including epilepsy, musculoskeletal problems, intellectual disability, feeding difficulties, visual abnormalities, hearing abnormalities, and communication difficulties. Treatment of cerebral palsy should take an interprofessional approach.

Etiology

Abnormal development or damage to the fetal or infant's brain causes cerebral palsy. The brain insult/injury causing CP is non-progressive (“static”) and can occur in the prenatal, perinatal or postnatal periods. The etiology in an individual patient is often multifactorial.

Prenatal Causes[2][3]

  • Congenital brain malformations
  • Intrauterine infections
  • Intrauterine stroke
  • Chromosomal abnormalities

Perinatal Causes[2][3]

  • Hypoxic-ischemic insults
  • Central nervous system (CNS) infections
  • Stroke
  • Kernicterus

Postnatal Causes[2][3]

  • Accidental and non-accidental trauma
  • CNS infections
  • Stroke
  • Anoxic insults

Prematurity is a significant risk factor for cerebral palsy. Complications of prematurity that can cause cerebral palsy include[2][3]:

  • Periventricular leukomalacia
  • Intraventricular hemorrhage
  • Periventricular infarcts.

Other risk factors associated with cerebral palsy are multiple gestation, intrauterine growth restriction, maternal substance abuse, preeclampsia, chorioamnionitis, abnormal placental pathology, meconium aspiration, perinatal hypoglycemia, and genetic susceptibility.[4][5]

Epidemiology

Cerebral palsy is the most common cause of childhood disability. It occurs in 1.5 to 2.5/1000 live births.[6] The prevalence is significantly higher in infants born prematurely than infants born at term. The risk of developing cerebral palsy increases with declining gestational age with infants born at less than 28 weeks gestational age being at most risk.[6] The prevalence is also higher in low birthweight infants. Very low birth weight (less than 1500 grams) infants born are at greatest risk; 5% to 15% of infants born weighing less than 1500 grams develop cerebral palsy.[6] Prenatal events cause approximately 80% of cerebral palsy cases, and postnatal events cause about 10% of cases.

History and Physical

Cerebral palsy is a clinical diagnosis based mostly on information gathered from the patient’s history and physical exam. The clinical history should focus on identifying risk factors and likely etiologies of the patient’s cerebral palsy. The history should include a detailed prenatal, birth, and developmental history. Developmental history should pay particular attention to motor development. In cerebral palsy motor development is delayed. A history of developmental regression is not consistent with cerebral palsy. Family history is important as well. Multiple family members with similar delayed development or neurologic disorders as the patient should prompt consideration of a genetic etiology of cerebral palsy or a disorder that mimics CP.[7][8] Clinical history should also focus on screening for co-morbid disorders including epilepsy, musculoskeletal abnormalities, pain, visual and hearing difficulties, feeding problems, communication disorders, and behavioral disorders.

The physical exam should focus on identifying clinical signs of cerebral palsy. Head circumference, mental status, muscle tone and strength, posture, reflexes (primitive, postural, and deep tendon reflexes), and gait should be evaluated. Clinical signs and symptoms of cerebral palsy can include micro- or macrocephaly, excessive irritability or diminished interaction, hyper- or hypotonia, spasticity, dystonia, muscle weakness, a persistence of primitive reflexes, abnormal or absent postural reflexes, incoordination, and hyperreflexia.

The physical exam is also used to identify the cerebral palsy type. Cerebral palsy is characterized by the type of tone abnormality and distribution of motor abnormalities. The subtypes of cerebral palsy are[9]:

  • Spastic diplegic: The patient has spasticity and motor difficulties affecting the legs more than the arms
  • Spastic hemiplegic: The patient has spasticity and motor difficulties affecting one side of the body, the arms are often involved more than the legs
  • Spastic quadriplegic: The patient has spasticity and motor difficulties affecting all four extremities, often the upper extremities are more involved than the legs
  • Dyskinetic/hyperkinetic (choreoathetoid): The patient has excessive, involuntary movements characterized as a combination of rapid, dance-like contractions of muscles and slow writhing movements
  • Dystonic: The patient has involuntary, sustained muscle contractions causing twisting and repetitive movements  
  • Ataxic: The patient has unsteadiness and incoordination, they are often hypotonic

Evaluation

Clinical history and physical exam combined with neuroimaging and standardized developmental assessments are used to make a diagnosis of cerebral palsy. Brain MRI is the preferred imaging modality for evaluating the cause of cerebral palsy. MRI has a higher diagnostic yield than CT and provides better detail of the brain’s anatomy. MRI has an 86% to 89% sensitivity for detecting abnormal neuroanatomy in the motor areas of the brain.[10] A cranial ultrasound performed in the neonatal/early infancy period can be useful in identifying intraventricular hemorrhage, ventriculomegaly, and periventricular leukomalacia.

For early detection of cerebral palsy standardized developmental assessments along with neuroimaging should be used. The General Movements Assessment (GM) is a standardized motor assessment used in children under age 5 months corrected age.[10] The GM observes the quality of spontaneous movements in infants while lying supine. Cramped-synchronized general movements and the absence of fidgety movements between 9 to 20 months reliably predict cerebral palsy. It has a 98% sensitivity and 89% to 93% inter-rater reliability.[10] The Hammersmith Infant Neurological Exam (HINE) is a standardized neurological assessment that can be administered between the ages of 2 and 24 months. It consists of 37 items and is subdivided into 3 sections: physical exam, documentation of motor development, and evaluation of the behavior state. The HINE has a 90% sensitivity for detecting cerebral palsy.[10]

An EEG should be obtained in patients suspected of having seizures. Patients with stroke as a cause of their cerebral palsy should undergo thrombophilia screening. Pro-thrombotic coagulation abnormalities are seen in 50% to 60% of patients with a history of stroke.[8]

The clinical signs and symptoms of cerebral palsy can be seen in many other conditions. Slowly progressive disorders can be mistaken for cerebral palsy, and sometimes these disorders have a specific treatment that halts the progression, prevents complications, or treats the primary underlying pathophysiology of the condition. Therefore, it is essential to screen for disorders that mimic cerebral palsy when the clinical history, physical exam, and neuroimaging are atypical for cerebral palsy. The following historical features are concerning for an alternative diagnosis: family history of cerebral palsy or other neurologic disorders, no known risk factor for cerebral palsy, developmental regression, hypotonia associated with weakness, rapid loss of neurologic skills, worsening during fasting or illness, oculomotor abnormalities, or sensory loss.[8][11] A metabolic work up to screen for inborn errors of metabolism should be obtained in patients who have a progressive course or decompensation during periods of catabolism.[11] Genetic workup should be obtained in those with dysmorphic features, brain malformations, family history of cerebral palsy, or if there is a history of consanguinity. Lumbar puncture should be obtained in patients with unexplained refractory seizures or movement disorders to screen for neurotransmitter disorders and glucose transporter deficiency.[8][11]

Treatment / Management

Treatment of cerebral palsy takes a interprofessional team approach. The team includes physicians (primary care, neurologists, physiatrists, orthopedists, and other specialists needed based on co-existing conditions), therapists (physical, occupational, and speech), behavioral health specialists, social workers/case managers, and educational specialists. Interventions should focus on maximizing the quality of life and decreasing disability burden. The patient, family, and team should set functional goals that are realistic and periodically reevaluated.[10]

Oral and injectable (e.g., botulinum toxin) medications are used to treat tone abnormalities, pain, and comorbid conditions such as epilepsy, sialorrhea, gastrointestinal disturbances, and behavior disorders. Medications used for spasticity include benzodiazepines, baclofen, dantrolene, tizanidine, cyclobenzaprine, botulinum toxin, and phenol.[12] Dystonia is often treated with trihexyphenidyl, gabapentin, carbidopa-levodopa, and benztropine. Sialorrhea is treated with glycopyrrolate, atropine drops, and scopolamine patches. Anti-seizure medications are used in patients with epilepsy. Constipation is a frequent complication of cerebral palsy and is treated with stool softeners and pro-motility agents. Anti-inflammatories are used for pain and antidepressants for depression and anxiety.

Surgical management options include placement of a baclofen pump, selective dorsal rhizotomy,[13] tendon releases, hip derotation/rotation surgery, spinal fusion, strabismus repair, and deep brain stimulation.[12]

Differential Diagnosis

Conditions that can mimic cerebral palsy include neurodegenerative disorders, inborn errors of metabolism, developmental abnormalities of the spinal cord, neuromuscular disorders, movement disorders, and neoplasms. Below is a list of differential considerations based on the predominant clinical feature.[3][7][11] 

Spasticity

  • Hereditary spastic paraplegia
  • Tethered cord
  • Spinal cord tumor
  • Adrenoleukodystrophy
  • Arginase deficiency
  • Pyruvate dehydrogenase deficiency
  • Rett syndrome
  • Lesch-Nyhan syndrome
  • Pelizaeus-Merzbacher
  • Glut 1 transporter deficiency

Dystonia

  • Dopa-responsive dystonia
  • Glutaric aciduria type 1
  • Pyruvate dehydrogenase deficiency
  • Lesch-Nyhan syndrome
  • Leigh's disease
  • Niemann-Pick type C
  • Glut 1 transporter deficiency

Hypotonia

  • Holocarboxylase synthetase deficiency
  • Zellweger syndrome
  • Infantile Refsum disease
  • Pontocerebellar hypoplasias
  • Metachromatic leukodystrophy

Ataxia

  • Ataxia-telangiectasia
  • X-linked spinocerebellar ataxia
  • Angelman's syndrome
  • Glut 1 transporter deficiency
  • Leigh disease
  • Joubert syndrome

Choreoathetosis

  • Pelizaeus-Merzbacher
  • Lesch-Nyhan syndrome

Weakness

  • Muscular dystrophies
  • Metachromatic leukodystrophy
  • Pontocerebellar hypoplasias

Prognosis

Most children with cerebral palsy will survive into adulthood.[14] Life expectancy is reduced in those who are severely affected. The most common cause of early death is respiratory disease, usually aspiration pneumonia.

The prognosis of motor abilities depends on the cerebral palsy subtype, the rate of motor development, ascertainment of developmental reflexes, and cognitive abilities. Children who walk independently typically achieve this milestone by 3 years of age. Those who walk with support may take up to age 9 years to reach this milestone.[15] A child that is not walking by age 9 years is unlikely to walk even with support. Children with hemiplegic, choreoathetoid, and ataxic cerebral palsy are likely to achieve walking. Good prognostic indicators for independent walking are sitting by age 24 months and crawling by 30 months.[15] Poor prognostic indicators for walking include no having achieved head balance by 20 months, primitive reflexes retained or nor postural reflexes by age 24 months, and not crawling by age 5 years.[15]

Complications

A variety of complications  can accompany cerebral palsy including[9]:

  • Pain- occurs in 50% to 75%
  • Intellectual disability occurs in 50%
  • Epilepsy occurs in 25% to 45%
  • Orthopedic disorders (hip subluxation/dislocation (30%), foot deformities, and scoliosis)
  • Speech impairment occurs in 40% to 50%
  • Hearing impairment occurs in 10% to 20%
  • Blindness occurs in 10%
  • Strabismus occurs in 50%
  • Neurobehavioral disorders occur in 25%
  • Growth failure
  • Pulmonary disease
  • Osteopenia occurs in 77% of those moderate-severely affected
  • Urologic disorders (incontinence, neurogenic bladder)- occurs in 30-60%
  • Sleep disturbances occur in 23%
  • Dental abnormalities

Deterrence and Patient Education

Cerebral palsy is a term used to describe a group of disorders that are caused by a non-progressive brain abnormality which results in difficulty with movement, tone, and/or posture. There are several factors during pregnancy, around the time of birth, and after birth that play a role in the development of cerebral palsy. The major risk factors for cerebral palsy are prematurity and low birth weight. Other causes of cerebral palsy include stroke, lack of oxygen to the brain, infections of the brain, and abnormal development of the brain. Cerebral palsy is the most common cause of childhood disability. Cerebral palsy is a clinical diagnosis and made by obtaining a detailed prenatal and birth history, physical exam, and neuroimaging. Treatment is aimed at achieving the best functional outcomes and takes an interprofessional team approach. Routine prenatal care and measures to reduce preterm birth lower the risk of cerebral palsy.

Enhancing Healthcare Team Outcomes

Clinicians should aim to recognize the signs of cerebral palsy and make the diagnosis of cerebral palsy or “high risk of cerebral palsy” as early as possible. Early diagnosis can improve functional outcomes and reduce disease burden because starting interventions early can optimize neuroplasticity. Historically the diagnosis of cerebral palsy was made between the ages of 12 to 24 months. Experts now feel the diagnosis can be made or accurately predicted before age 6 months corrected age.[10] Delays in diagnosis can be harmful to parent and caregiver’s well-being. A diagnosis allows parents to receive psychological support and resources. Patients with known risk factors for cerebral palsy should be referred for further diagnostic testing including neuroimaging and standardized developmental assessments.[10] (Level I)  Despite the best treatment, the lifespan of most individuals with cerebral palsy is significantly reduced. [16] (Level II)


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Cerebral Palsy - Questions

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Which of the following will cause a scissor gait?



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In explaining what cerebral palsy is to the parents of an 8-year-old daughter, why would the condition be described as a static encephalopathy?



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Which disease is responsive to neurodevelopmental treatment?



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Which is the best stabilizer for jaw control in a patient with athetoid cerebral palsy?



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A 15-month-old boy with a history of prematurity crosses his legs when lifted from behind rather than pulling them up. He has stiffness at his hips bilaterally, making diaper changing difficult. He has not started crawling yet. What is the likely diagnosis?



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Where is the most frequent contracture in cerebral palsy?



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Which of the following findings is least likely to be associated with cerebral palsy?



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Which of the following is most commonly related to cerebral palsy?



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Which of the following statements is not true about cerebral palsy?



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Which of the following is not considered a risk factor for the development of cerebral palsy (CP)?



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Which of the following types of cerebral palsy is most likely to be seen in preterm infants?



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Which of the following medications is often used to reduce spasticity in patients with cerebral palsy (CP)?



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A child with severe spastic quadriplegic cerebral palsy is never expected to ambulate. He has no cognitive impairment and has the desire to be independent for mobility. What kind of wheelchair would be best?



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A child with cerebral palsy has good head control and fair sitting stability. There is fluctuation of lower extremity extensor tone. What position would be best for feeding?



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While working with children in an Early Intervention group the occupational therapist starts a floor program. One of the children has cerebral palsy (CP) and has a problem sitting independently. What is the best solution?



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What should be done first prior to feeding as an initial intervention for a child with cerebral palsy (CP) who exhibits tongue thrusting?



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A patient with spastic cerebral palsy wants to use a computer. What should the OT assess first?



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A patient with cerebral palsy has gait ataxia and is cognitively intact. During a home evaluation, what should be the major focus for the occupational therapist?



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A child with cerebral palsy (CP) has as a short-term goal of improving his ability to manipulate objects through the inhibition of hand flexor spasticity. Which activity would be most effective?



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Which of the following types of cerebral palsy is most common?



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Which of the following is the best description of cerebral palsy?



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A 3-year-old child is receiving therapy for spastic cerebral palsy (CP). During the session, the patient becomes unresponsive for a few seconds and the only movement is eye rolling. What is the most likely explanation?



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A patient with cerebral palsy has severe spasticity of the lower limbs only. What type of CP is present?



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Which of the following is the exception to the four types of cerebral palsy?



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What percentage of cerebral palsy is caused by a recognized brain injury such as infection, bleeding into the brain, and damage caused by lack of oxygen?



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Which of the following is an exception to what might be observed in a newborn with cerebral palsy?



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Which of the following types of cerebral palsy is the most likely diagnosis for a six-year-old boy whose parents report his sporadic difficulty when lifting his toys onto the toy shelf but the ability to write his name with a crayon?



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Which of the following is true of spastic cerebral palsy?



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Which of the following is the type of cerebral palsy, also referred to as dyskinetic cerebral palsy, caused by damage to the basal ganglia?



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Which of the following is the most common form of cerebral palsy, affecting 70 to 80 percent of patients?



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Which of the following is false related to the risk of cerebral palsy?



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Which is not associated with cerebral palsy?



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Which is the least common cause of cerebral palsy?



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Which is not true regarding patients with cerebral palsy?



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Which of the following is not a risk factor for developing cerebral palsy?



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Certain cranial sonographic findings in preterm infants increase the risk of cerebral palsy (CP). Which one the following findings does not increase this risk?



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A 12-month-old is being evaluated for motor disabilities with increased tone suspicious for cerebral palsy (CP). MRI of the head is done. Which of the abnormalities is least likely to explain the findings?



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A 12-month-old infant has motor delays and is felt to have cerebral palsy. Developmental skills are measured using the Bayley scales. The MDI is 75 and the PDI is less than 50 with a normal range of 100 plus or minus 15. Select the correct statement.



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A 15-month-old child with motor delays secondary to cerebral palsy is tested using the Battelle Developmental Inventory. The patient's receptive language skills are at 12 months, her motor skills are at 6 months, her expressive language skills are at 12 months, and her emotional and social skills are at 15 months. Which of the following would the child not be able to do?



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A 15-month-old child with motor delays secondary to cerebral palsy is tested using the Battelle developmental scales. His motor skills are at the 6-month level. Which of the following skills is he unlikely to be able to do?



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A five-year-old with cerebral palsy has been wheelchair-bound his whole life. The child cannot sit unsupported, communicates using a touch talker, and has never been able to crawl or pull to a stand. She likes to have books read to her and responds appropriately to jokes. Which of the following is not a realistic short-term goal?



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A 5-year-old with cerebral palsy has been wheelchair-bound his whole life. The child cannot sit unsupported, communicates using a touch talker and has never been able to crawl or pull to a stand. The child is also unable to feed his/herself. The child is able to use the direction switch on his power wheelchair. Which of the following goals is not realistic for kindergarten?



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A 12-month-old female is brought in for a routine health visit. The mother is concerned that the child is not at the same level as a sibling was at the same age. Birth history and medical history are unremarkable. The child's Moro reflex at birth was normal. She has favored her left hand since the age of 6 months. She smiles symmetrically, sits without support, but falls if pushed to the right. Her right hand is slightly smaller than the left. In the prone position, the infant does not push up with her right arm. Leg exam and reflexes are normal. What is the most likely diagnosis?



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A patient is suspected of having congenital hemiplegic cerebral palsy. EEG shows no epileptiform activity and MRI shows a left-sided small frontal porencephalic cyst. There are no depigmented macules on Wood's light exam and urine for CMV is negative. Select the correct statement.



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A 1-year-old child is diagnosed with congenital hemiplegic cerebral palsy with a small left-sided porencephalic cyst. Which of the following should be done?



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An 18-month-old female presents with her parents for an initial evaluation. She was born prematurely at 28 weeks and had an intraventricular hemorrhage. She started sitting at 12 months and recently started crawling. Her provider suggested she be evaluated for cerebral palsy. The parents would like to know what exactly is cerebral palsy. What is the definition of cerebral palsy?



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A 22-month-old girl presents for evaluation of motor delay. She was born at 27 weeks gestational age and has a history of periventricular leukomalacia. On exam, she has spasticity of the bilateral lower extremities. Which of the following is the most common risk factor for her clinical presentation?



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Cerebral Palsy - References

References

The Definition and Classification of Cerebral Palsy. Developmental medicine and child neurology. 2007 Feb;     [PubMed]
Nelson KB, Causative factors in cerebral palsy. Clinical obstetrics and gynecology. 2008 Dec;     [PubMed]
MacLennan AH,Thompson SC,Gecz J, Cerebral palsy: causes, pathways, and the role of genetic variants. American journal of obstetrics and gynecology. 2015 Dec;     [PubMed]
McMichael G,Bainbridge MN,Haan E,Corbett M,Gardner A,Thompson S,van Bon BW,van Eyk CL,Broadbent J,Reynolds C,O'Callaghan ME,Nguyen LS,Adelson DL,Russo R,Jhangiani S,Doddapaneni H,Muzny DM,Gibbs RA,Gecz J,MacLennan AH, Whole-exome sequencing points to considerable genetic heterogeneity of cerebral palsy. Molecular psychiatry. 2015 Feb;     [PubMed]
van Eyk CL,Corbett MA,Maclennan AH, The emerging genetic landscape of cerebral palsy. Handbook of clinical neurology. 2018;     [PubMed]
Oskoui M,Coutinho F,Dykeman J,Jetté N,Pringsheim T, An update on the prevalence of cerebral palsy: a systematic review and meta-analysis. Developmental medicine and child neurology. 2013 Jun;     [PubMed]
Morgan C,Fahey M,Roy B,Novak I, Diagnosing cerebral palsy in full-term infants. Journal of paediatrics and child health. 2018 Oct;     [PubMed]
Whelan MA, 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 Nov 23;     [PubMed]
Graham HK,Rosenbaum P,Paneth N,Dan B,Lin JP,Damiano DL,Becher JG,Gaebler-Spira D,Colver A,Reddihough DS,Crompton KE,Lieber RL, Cerebral palsy. Nature reviews. Disease primers. 2016 Jan 7;     [PubMed]
Novak I,Morgan C,Adde L,Blackman J,Boyd RN,Brunstrom-Hernandez J,Cioni G,Damiano D,Darrah J,Eliasson AC,de Vries LS,Einspieler C,Fahey M,Fehlings D,Ferriero DM,Fetters L,Fiori S,Forssberg H,Gordon AM,Greaves S,Guzzetta A,Hadders-Algra M,Harbourne R,Kakooza-Mwesige A,Karlsson P,Krumlinde-Sundholm L,Latal B,Loughran-Fowlds A,Maitre N,McIntyre S,Noritz G,Pennington L,Romeo DM,Shepherd R,Spittle AJ,Thornton M,Valentine J,Walker K,White R,Badawi N, Early, Accurate Diagnosis and Early Intervention in Cerebral Palsy: Advances in Diagnosis and Treatment. JAMA pediatrics. 2017 Sep 1;     [PubMed]
Leach EL,Shevell M,Bowden K,Stockler-Ipsiroglu S,van Karnebeek CD, Treatable inborn errors of metabolism presenting as cerebral palsy mimics: systematic literature review. Orphanet journal of rare diseases. 2014 Nov 30;     [PubMed]
Nahm NJ,Graham HK,Gormley ME Jr,Georgiadis AG, Management of hypertonia in cerebral palsy. Current opinion in pediatrics. 2018 Feb;     [PubMed]
Park TS,Dobbs MB,Cho J, Evidence Supporting Selective Dorsal Rhizotomy for Treatment of Spastic Cerebral Palsy. Cureus. 2018 Oct 19;     [PubMed]
Strauss D,Brooks J,Rosenbloom L,Shavelle R, Life expectancy in cerebral palsy: an update. Developmental medicine and child neurology. 2008 Jul;     [PubMed]
da Paz Júnior AC,Burnett SM,Braga LW, Walking prognosis in cerebral palsy: a 22-year retrospective analysis. Developmental medicine and child neurology. 1994 Feb;     [PubMed]
Morgan P,McGinley JL, Cerebral palsy. Handbook of clinical neurology. 2018     [PubMed]

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