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Cerebral Palsy

Cerebral palsy (CP) describes a heterogenous group of motor impairment syndromes.


Presentation

General

Infants are slow to start normal activities like sitting up, crawling, smiling or walking. They may favour one side when sitting up or maintain an abnormal posture. Their limbs may appear to be stiff and the child while growing up may seem excessively clumsy. While in some cases, the child may seem excessively floppy with flaccid, low toned muscles. Movements may be uncoordinated.

Eyes

42% of children suffering from CP have visual defects.

Musculoskeletal

Contractures may occur, especially in spastic CP. There may be dysarthria. The muscles are either hypertonic or hypotonic, or sometimes both. Bones are thin and weak, with the metaphyses appearing to be wide and ballooning while the diaphysis appear to be very narrow. Joint deformities may also occur. Stature is short due to improper bone development.

Neurological

Seizures, with or without epilepsy are common, occurring in about 28% of cases. 31-88% children suffer from speech defects due to hypertonia or hypotonia of muscles of the larynx, respiratory tree and oro-facial muscle defects. Children are both unable to articulate well, due to muscle abnormality as well as unable to learn the language in time due to mental impairment.

Psychiatrical

Mental impairment is present and the child has great difficulty in learning and performing simple activities like holding a pencil, catching a ball, etc in 23-56% of cases. This may lead to depression while growing up.

Systemic

Urinary and fecal incontinence may be present.

Complications

Complications are very rare since the disease is non progressive. However, some include:

  • Breathing problems due to respiratory muscle involvement
  • Persistent pain due to bone and joint deformities
  • Severe seizures and epileptic fits
  • Motor dysfunction leading to trauma
  • Severe deformity
Epilepsy
  • We describe the first clinical case with a 1.607 Mb duplication at 3q29 (chr3: 195,731,956-197,339,329), accompanied by severe intellectual disability, epilepsy, and cerebral palsy.[ncbi.nlm.nih.gov]
  • A large proportion has some kind of cognitive impairment; the prevalence varies with the type of CP and especially increases when epilepsy is present. Epilepsy is present in 20-40%; it is most common among the hemi- and tetraplegics.[ncbi.nlm.nih.gov]
  • A large proportion has some kind of cognitive impairment; the prevalence varies with the type of CP and especially increases when epilepsy is present. Epilepsy is present in 20 – 40%; it is most common among the hemi- and tetraplegics.[doi.org]
  • METHOD: Population CP registry data were used to retrospectively assess the frequency of intellectual disability and strength of associations between intellectual disability and mobility, epilepsy, vision, hearing, communication, and neuroimaging patterns[ncbi.nlm.nih.gov]
  • Thirty children (17.3%) had epilepsy. A total sleep problem score and six factors indicative of the most common areas of sleep disorder in childhood were obtained.[ncbi.nlm.nih.gov]
Difficulty Walking
  • People with cerebral palsy may have difficulty walking. They may also have trouble with tasks such as writing or using scissors. Some have other medical conditions, including seizure disorders or mental impairment.[medlineplus.gov]
  • They may have difficulty walking and performing fine motor functions, such as grasping objects and writing. Mixed cerebral palsy Some people have a combination of symptoms from the different types of CP. This is called mixed CP.[healthline.com]
  • The most common symptoms include: Seizures Stiffness of the arms and/or legs Difficulty walking Developmental delays Proper nutrition is highly important for children with cerebral palsy.[rileychildrens.org]
  • Each of them were having difficulty walking, with their knees starting to slope inwards. But a video of them walking in the exoskeleton suit showed noticeable improvements.[dailymail.co.uk]
Abdominal Obesity
  • Mark D Peterson, Heidi J Haapala, Ashish Chaddha and Edward A Hurvitz, Abdominal obesity is an independent predictor of serum 25-hydroxyvitamin D deficiency in adults with cerebral palsy, Nutrition & Metabolism, 10.1186/1743-7075-11-22, 11, 1, (22), ([doi.org]
Failure to Thrive
  • The individual had a history of failure to thrive, bowel and bladder incontinence, reduced bone mineral density (BMD) for age, and spinal deformity at the time exercise was initiated.[ncbi.nlm.nih.gov]
  • Other associated problems include: Eating, drinking and swallowing difficulties and poor nutrition: failure to thrive and malnourishment. Bladder problems: incontinence and infections are common.[patient.info]
  • Obesity, less frequently than failure to thrive Gastroesophageal reflux and associated aspiration pneumonia Constipation Dental caries Dental problems also include enamel dysgenesis, malocclusion, and gingival hyperplasia.[emedicine.com]
Malocclusion
  • We looked for a possible association between overall functional impairment and malocclusion in this population.[ncbi.nlm.nih.gov]
  • This article reports the treatment of a 12-year-old girl with ataxic cerebral palsy; she had a dental and skeletal Class II malocclusion, maxillary transverse deficiency, and severe crowding in both arches.[ncbi.nlm.nih.gov]
  • 13-year old boy with spastic quadriplegia cerebral palsy visited dental clinic with chief complaints of mouth breathing and malocclusion. His mouth was constantly open at the resting position, with his mandible and tongue displaced downward.[ncbi.nlm.nih.gov]
  • A 13-year-old boy with cerebral palsy and spastic quadriplegia complained of an undesirable oral appearance because of his malocclusion. He had a Class II molar relationship, with severe maxillary and moderate mandibular anterior crowding.[ncbi.nlm.nih.gov]
  • A case report describing the successful orthodontic treatment of a 10-year-old boy with the dyskinesia type of CP and severe malocclusion is presented.[ncbi.nlm.nih.gov]
Xerostomia
  • Adverse side effects include xerostomia (dry mouth) with resulting impact on mastication and dental health; external scarring; and risk of facial and hypoglossal nerve damage ( Burton 1991 ). (e) Sublingual gland excision.[doi.org]
Hearing Impairment
  • Although the main focus is commonly the motor disorder, medical management also encompasses far wider issues including the management of multiple co-morbidities (e.g. epilepsy, visual and hearing impairment, gastro-oesophageal reflux and constipation,[discovery.ucl.ac.uk]
  • Because children with CP may have associated deficits of mental retardation, ophthalmologic and hearing impairments, speech and language disorders, and oral-motor dysfunction, screening for these conditions should be part of the initial assessment (Level[ncbi.nlm.nih.gov]
  • If your child is diagnosed with CP, a multidisciplinary team of TIRR Memorial Hermann affiliated physicians and specialists will perform additional tests to identify vision and hearing impairment, speech delays or impairments, intellectual disabilities[tirr.memorialhermann.org]
  • For vision and hearing impairments, there was a lack of consistency in coding of impairments between authors and registries, as some authors used the term “some impairment.”[doi.org]
  • Other complications, such as intellectual impairment, seizures, and vision or hearing impairment also commonly accompany Cerebral Palsy.[cerebralpalsy.org]
Esotropia
  • Kolling, Best age for surgery for infantile esotropia, European Journal of Paediatric Neurology, 15, 3, (205), (2011).[doi.org]
Muscle Spasticity
  • RESULTS: Significant changes were observed in the children's gross motor function and muscle spasticity, as evidenced by the GMFM-88 total score, scores for each of its domains, the GMFM-66 percentile and the muscle tone (P CONCLUSION: The QOL of the[ncbi.nlm.nih.gov]
  • Anti-cholinergic drugs to treat involuntary movements, drugs like diazepam to help with muscle spasticity, etc can be administered. Physical therapy is very useful in case of with muscle spasticity/flaccidity.[symptoma.com]
  • Common motor disorders in cerebral palsy that may appear in different combinations include co‐contraction of antagonist muscles, spasticity and related signs such as clonus and reflex irradiation, mirror movements, impaired ability for rapid and selective[doi.org]
  • We aim to minimize muscle spasticity and improve range of motion and mobility.[columbiadoctors.org]
  • Selective dorsal rhizotomy (SDR) Selective dorsal rhizotomy (SDR) is a surgical procedure that can help children with severe muscle spasticity in their legs to improve their walking.[web.archive.org]
Scissoring Gait
  • gait; and muscle tone that is either too stiff or too floppy.[ninds.nih.gov]
  • Physical indicators of spastic cerebral palsy include spastic paresis of multiple limbs and joint contractures , scissors gait, and persistence of primitive reflexes .[amboss.com]
  • The affected individuals have a wide, staggering gait, known as the scissors gait. Ataxic cerebral palsy In this type, instead of muscle rigidity and stiffness, hypotonia is present. Tremors are also found.[symptoma.com]
  • A scissors gait and toe walking are typical. In mild cases, impairment may occur only during certain activities (eg, running).[merckmanuals.com]
  • gait; variations in muscle tone, either too stiff or too floppy; excessive drooling or difficulties swallowing or speaking; shaking ( tremor ) or random involuntary movements; delays in reaching motor skill milestones; and difficulty with precise movements[web.archive.org]
Anterior Knee Pain
  • Patella alta is another relatively common condition in ambulatory adults with CP, often associated with longstanding anterior knee pain or a crouch gait pattern.[doi.org]
Knee Extension Limited
  • Children with knee extension limited by 10 - 30 degrees,especially those with cerebral palsy, exhibit a stance compatible with walking. Walking is difficult and the gait pattern, "crouch gait", is considered typical for this degree of limitation.[ncbi.nlm.nih.gov]
Dystonia
  • We rated the GRADE quality of the evidence as low.We found no difference in mean follow-up scores for change in dystonia as measured by the Barry Albright Dystonia Scale (BADS), which assesses eight body regions for dystonia on a 5-point scale (0 none[ncbi.nlm.nih.gov]
  • We describe the importance of the order of dual neuromodulation surgery for dystonia and deafness.[ncbi.nlm.nih.gov]
  • Screening for methemoglobinemia in children with dystonia, microcephaly, and progressive cerebellar atrophy can be helpful in identifying more cases.[ncbi.nlm.nih.gov]
  • Our findings suggest that ITB therapy may be useful for CP patients with uncontrollable spasticity, dystonia, or opisthotonus who are not able to take a lateral position for pump implantation due to deformities of their extremities and spine.[ncbi.nlm.nih.gov]
  • Uncontrolled dystonia with concomitant rhabdomyolysis was considered. Subsequent aggressive hydration and administration of muscle relaxant afforded abrupt resolution of symptoms.[ncbi.nlm.nih.gov]
Tremor
  • He had nystagmus and psychomotor delay since infancy and tremor with ataxia developing gradually. Brain MRI revealed demyelination over white matter of the cerebral hemispheres and posterior limbs of the internal capsules.[ncbi.nlm.nih.gov]
  • […] development milestones – for example, not sitting by eight months or not walking by 18 months seeming too stiff or too floppy (hypotonia) weak arms or legs fidgety, jerky or clumsy movements random, uncontrolled movements muscle spasms shaking hands (tremors[nhs.uk]
  • The following symptoms may occur in other types of cerebral palsy: Abnormal movements (twisting, jerking, or writhing) of the hands, feet, arms, or legs while awake, which gets worse during periods of stress Tremors Unsteady gait Loss of coordination[nlm.nih.gov]
Ataxia
  • BACKGROUND: Ataxia-telangiectasia (A-T) is an autosomal recessive disease that consists of progressive cerebellar ataxia, variable immunodeficiency, sinopulmonary infections, oculocutaneous telangiectasia, radiosensitivity, early aging, and increased[ncbi.nlm.nih.gov]
  • He had nystagmus and psychomotor delay since infancy and tremor with ataxia developing gradually. Brain MRI revealed demyelination over white matter of the cerebral hemispheres and posterior limbs of the internal capsules.[ncbi.nlm.nih.gov]
  • This report describes the modulation effect of rTMS to functional connectivity, functional network connectivity, motor, and cognitive ability following treatment in a child with mild ataxia CP.[ncbi.nlm.nih.gov]
  • Although gangliocytoma is a well-differentiated benign tumor, the almost total replacement of the medulla oblongata by the tumor cells was assumed to result in ataxia via the olivocerebellar tract and secondary cerebellar atrophy, followed by central[ncbi.nlm.nih.gov]
  • The most common are a lack of muscle coordination when performing voluntary movements (ataxia); stiff or tight muscles and exaggerated reflexes (spasticity); walking with one foot or leg dragging; walking on the toes, a crouched gait, or a “scissored”[ninds.nih.gov]
Dysarthria
  • This study describes the use of a neuroplasticity-principled speech treatment approach (LSVT( )LOUD) with children who have dysarthria secondary to cerebral palsy.[ncbi.nlm.nih.gov]
  • Children affected with athetoid cerebral palsy may be perceptive and intelligent; however, because of the involuntary movements and dysarthria, they are often unable to communicate by intelligible words or signs.[britannica.com]
  • Patients with non- spastic cerebral palsy present with dysarthria and abnormal involuntary movements (choreoathetoid, dystonic, or ataxic ) that worsen with stress. Seizure disorders and intellectual disability are associated with all types.[amboss.com]
  • Dysarthria occurs and is often severe. Ataxic syndromes occur in 5% of cases and result from involvement of the cerebellum or its pathways.[merckmanuals.com]
  • Tremors Unsteady gait Loss of coordination Floppy muscles, especially at rest, and joints that move around too much Other brain and nervous system symptoms may include: Learning disabilities are common, but intelligence can be normal Speech problems (dysarthria[nlm.nih.gov]
Hyperactivity
  • Verónica Schiariti, Soheil Mahdi and Sven Bölte, International Classification of Functioning, Disability and Health Core Sets for cerebral palsy, autism spectrum disorder, and attention‐deficit–hyperactivity disorder, Developmental Medicine & Child Neurology[doi.org]
  • According to the Scientific World Journal, children with cerebral palsy often suffer from attention deficit hyperactivity disorder (ADHD), anxiety, worry, conduct problems such as temper tantrums and disobedience, and socialization problems such as preferring[birthinjuryguide.org]
  • Interventional neuropediatrics: treatment of dystonic and spastic muscular hyperactivity with botulinum toxin A. Neuropediatrics. 1997 ; 28 : 307 – 313. 25. Use of botulinum toxin injection in 17 children with spastic cerebral palsy.[doi.org]
  • There is also an increased prevalence of challenging behaviours (may be triggered by pain, discomfort or sleep disturbances), problems with peer relationships, neurodevelopmental disorders, including autism spectrum disorder (ASD) and attention deficit hyperactivity[patient.info]

Workup

A definite test for cerebral palsy does not exist. Diagnosis is made based on the history and physical examination of the motor system [6]. A CT scan and an MRI may be performed to check for CNS abnormalities [5].

Streptococcus Pneumoniae
  • Make sure your child is vaccinated against infections that can cause meningitis and encephalitis, including Haemophilus influenzae type B (HiB vaccine) and Streptococcus pneumoniae ( pneumococcal vaccine).[cdc.gov]

Treatment

There is no cure for this disease. However, treatment helps improve the quality of life. Anti-convulsant drugs to prevent fits may be given. Anti-cholinergic drugs to treat involuntary movements, drugs like diazepam to help with muscle spasticity, etc can be administered [8].

Physical therapy is very useful in case of with muscle spasticity/flaccidity. Special exercises are performed by affected children that help improve motor coordination [10]. Speech therapy is undertaken to help the child to articulate better. It helps both in overcoming learning disabilities as well as speaking. Surgical intervention is needed to treat any bone or joint deformity, if present as well as muscle contractures.

Counselling is also a very important part if treatment as it helps the family understand how to support the affected child in the best way.

Prognosis

This disease is non progressive, so the symptoms do not worsen over time. With the right guidance and counselling, parents are able to help and support their child suffering from this disease.

Etiology

Cerebral palsy occurs due to abnormalities in the developing brain. These abnormalities lie in the motor cortex hence the resulting motor dysfunction.

Structural defects

80% of CP are due to structural defects that occur in intrauterine life or due to hypoxia occurring during obstructed or difficult labor. These structural abnormalities occur mostly in the white matter and are of unknown cause.

Genetics

About 2% of cerebral palsy cases are due to genetic abnormalities which are either inherited (rare) or occur due to acquired mutations. No particular gene has been isolated as the sole culprit yet. However, it is believed that defects or deficiency in the enzyme glutamate carboxylase-1 may be involved. In inherited cases, the pattern of inheritance is autosomal recessive.

Causative factors

  • Prematurity

Premature infants are at a much higher risk of developing CP. Around 40-50% infants born premature develop the disease. Out of these, 70-90% cases are due to abnormalities occurring in intrauterine life. In infants born before the 28th gestational week, chances of developing CP are as high as 11% [2].

  • Birth weight

Infants with a low birth weight are more at risk [4]. Neonates having a birth weight of only 1-1.5 Kg, or less than 3.3 pounds, have a 6% chance of becoming a victim of cerebral palsy [1].

  • Trauma

Trauma of the neonate, during or after labor and delivery may result in brain damage, either due to direct trauma, or due to other causes like hypoxia, exposure to toxins, etc. this may lead to CP.

  • Rh-Incompatibility

Rh factor incompatibility between mother and her baby may result in excessive hemolysis leading to hypoxia and this brain injury.

  • Others

Other causes include obstructed labor, complications during delivery, exposure to radiation, teratogens, lead poisoning, severe infections during pregnancy, severe jaundice, meningitis, etc.

Epidemiology

Race

Black infants and children are at a higher risk of developing cerebral palsy than white infants. This may be attributed to a low level of maternal health care and low socioeconomic status in most black countries [7].

Age

Incidence is much higher in preterm than term infants. Majority of the cases occur at birth, however, some preterm infants may develop CP between 3-5 years of age [3].

Sex

Cerebral palsy is much more common in males.

Incidence

Studies reveal that 2.1 to 4 out of every 1,000 individuals in the United States suffer from Cerebral Palsy. It is estimated than more than 764,000 people suffer from Cerebral Palsy currently in the US. Around 8,000-10,000 children are likely to develop this disease every year.

Sex distribution
Age distribution

Pathophysiology

Cerebral palsy occurs due to structural or genetic defects leading to abnormalities in the white matter of the motor cortex which results in motor dysfunction [2]. The type of palsy present is classified according to the degree and type of motor impairment present.

Spastic cerebral palsy

This is by far the most common type of cerebral palsy accounting for around 70% of cases. It presents with stiff or spastic muscles. Lower limbs are mostly involved, resulting in diplegia or just single leg paralysis. Upper limbs may also be involved, along with the leg of that side resulting in hemiplegia. In severe cases, both arms and legs may be spastic, making the patient quadriplegic. The affected individuals have a wide, staggering gait, known as the scissors gait.

Ataxic cerebral palsy

In this type, instead of muscle rigidity and stiffness, hypotonia is present. Tremors are also found. Visual and auditory functioning is commonly disturbed. Since in this type, the abnormality lies mainly in the cerebellum, patients present with movement and balance discoordination, unstable gait, writing and speech disabilities. It occurs in 5-10% of cases.

Dyskinetic or athetoid cerebral palsy

10% of cases of CP are of this type. In this type, the defect occurs in the pyramidal or extra pyramidal tracts and the basal ganglia may also be involved. Affected individuals have both hypertonia and hypotonia resulting in complete inability of fine motor movements. They are unable to maintain their posture or complete simple movements such as holding or touching a certain object.

Mixed cerebral palsy

This is the most rare type and is a heterogeneous combination of all three subtypes: Spastic, ataxic and athetoid cerebral palsies. Diagnosis is exceedingly difficult as symptoms vary greatly.

Prevention

  • Early treatment with magnesium sulphate in high risk preterm infants may prove to be beneficial in preventing cerebral palsy.
  • Full term babies that are at high risk may be protected from disabilities by cooling them immediately after birth.
  • Maintaining a healthy nutritional diet during pregnancy.
  • Managing maternal hypertension and/or diabetes.
  • Managing anemia.
  • Avoiding alcohol intake and smoking during pregnancy.
  • Avoiding exposure to harmful radiation, chemicals and intake of illegal drugs.

Summary

Cerebral palsy (CP) is a group of motor defects that result in movement disabilities along with other symptoms. The word cerebral denotes an abnormality in the cerebrum of the brain and the word palsy refers to weakness or paralysis of a group of muscles.

So due to an abnormality in the part of the brain that controls motor activity of the body, movement disorders occur. Patients with this condition suffer from immobility or difficulty in walking with an uneven gait, uncoordinated and disjointed movements, involuntary, spastic motions as well as speech and visual defects. Mental impairment and low IQs may be present.

Cerebral palsy mostly occurs at birth, but in some cases, it may develop in children between 3-5 years of age. The disease is non progressive, but unfortunately, it does not have a cure.

Patient Information

Definition

Cerebral palsy is a group of motor defects that are due to structural abnormalities in the brain occurring either before birth, or after birth up to around 5 years of age.

Cause

It is due to structural abnormalities in the part of the brain that controls movements and coordination.

Signs and symptoms

Affected children are short with weak bones, stiff, rigid or extremely floppy muscles, limb paralysis, speech defects, visual defects, seizures and mental impairment.

Treatment

There is no cure but treatment can be started to improve quality of life. It includes counselling, medication, physical therapy, and sometimes surgery.

References

Article

  1. Wyatt K, Edwards V, Franck L, et al. Cranial osteopathy for children with cerebral palsy: a randomised controlled trial. Arch Dis Child. Jun 2011;96(6):505-12.
  2. Ancel PY, Livinec F, Larroque B, et al. Cerebral palsy among very preterm children in relation to gestational age and neonatal ultrasound abnormalities: the EPIPAGE cohort study. Pediatrics. Mar 2006;117(3):828-35
  3. Capute AJ, Accardo PJ, eds. Developmental Disabilities in infancy and Childhood. Vol 2. 2nd ed. Baltimore, Md: Brookes Publishing; 2001.
  4. 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. Oct 2001;15(4):359-63.s
  5. Woodward LJ, Anderson PJ, Austin NC, Howard K, Inder TE. Neonatal MRI to predict neurodevelopmental outcomes in preterm infants. N Engl J Med. Aug 17 2006;355(7):685-94.
  6. Jones MW, Morgan E, Shelton JE, Thorogood C. Cerebral palsy: introduction and diagnosis (part I). J Pediatr Health Care. May-Jun 2007;21(3):146-52.
  7. Odding E, Roebroeck ME, Stam HJ. The epidemiology of cerebral palsy: incidence, impairments and risk factors. Disabil Rehabil. Feb 28 2006;28(4):183-91.
  8. 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. May 2009;13(3):240-6.
  9. Nelson KB. Can we prevent cerebral palsy?. N Engl J Med. Oct 30 2003;349(18):1765-9
  10. Volpe JJ. Neurology of the Newborn. 4th ed. Philadelphia, Pa: WB Saunders; 2001:4.

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Last updated: 2019-07-11 22:04