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
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].
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
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 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.
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.
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.
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.
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
- 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.
- 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
- Capute AJ, Accardo PJ, eds. Developmental Disabilities in infancy and Childhood. Vol 2. 2nd ed. Baltimore, Md: Brookes Publishing; 2001.
- 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
- 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.
- 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.
- 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.
- 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.
- Nelson KB. Can we prevent cerebral palsy?. N Engl J Med. Oct 30 2003;349(18):1765-9
- Volpe JJ. Neurology of the Newborn. 4th ed. Philadelphia, Pa: WB Saunders; 2001:4.