The Cause of Most Cases of Cerebral Palsy - Case Study Example

Cerebral Palsy Definition Cerebral palsy is a static encephalopathy that may be defined as a non-progressive disorder of posture and movement, often associated with epilepsy and abnormalities of speech, vision, and intellect resulting from a defect or lesion of the developing brain (Haslam, 1996). The damage may occur in utero, during the birth process, or during the first few years of life. Incidence and prevalence The incidence is about 2 per 1000 live births. It is more prevalent in more deprived socio-economic populations (Odding, 2006). Etiology and risk factors The cause of most cases of cerebral palsy is not known. Babies who have congenital malformations in systems such as the heart, kidneys, or spine are also more likely to develop CP, probably because they also have malformations in the brain (Wilmington, "Cerebral palsy program"). Some of the risk factors that have been identified are prematurity, low-birth weight (Haslam, 2000), seizures in newborn, alcohol abuse and cocaine consumption  by the mother during pregnancy, intrauterine infections like rubella, toxoplasmosis, and cytomegalovirus (Wilmington, "Cerebral palsy program"), multiple births and neonatal asphyxia (Sharma, 1999). In premature and low-birth weight babies, the increased incidence is probably due to intracerebral haemorrhage and periventricular leukomalacia (Haslam, 2000). Cerebral palsy can occur in children who were born normally but have suffered some kind of insult to brain later. Asphyxia, shaken baby syndrome and severe meningitis or encephalitis can lead to CP (Wilmington, "Cerebral palsy program"). Clinical features There are five major types of cerebral palsy (CP): Spastic cerebral palsy: This type accounts for over 90% of all CP cases (Witchers, 2005). It is the most severe form of CP. In this type, the child typically has hypertonia, which makes movement difficult. It can affect both legs (spastic diplegia), or one side of the body (spastic hemiplegia) or involve all four limbs and the trunk (spastic quadriplegia). Spastic hemiplegia is more common than spastic diplegia in low birth weight infants (Haslam, 2000). Infants with spastic hemiplegia have decreased spontaneous movements on the affected side and show hand preference at a very early age. The upper limb is more involved than the lower limb and the child has difficulty in the motor movements of the hand. Motor development is delayed. The gait is circumductive type. There may be growth arrest in the affected limbs. The child can have mental retardation and seizures also along with motor symptoms. In those with spastic diplegia, there is bilateral spasticity of the legs. These children, while crawling, use the arms in a normal reciprocal fashion but tend to drag the legs behind as a rudder (Haslam, 2000). This is known as commando crawl and this may be the first sign of CP in these infants. In severe cases, excessive adduction of the legs makes application of the diaper difficult. The child may adopt scissoring posture when suspended by the axillae. Seizures are rare in these children and most of them have normal intellectual development (Haslam, 2000). The most severe form of CP is spastic quadriplegia. Children who are affected with this type of CP have motor impairment of all the limbs including the trunk. They have recurrent seizures and marked mental retardation. They have supranuclear bulbar palsies because of which they have swallowing difficulties leading to aspiration pneumonia. They eventually develop flexor contractures of the knees and elbow, developmental disabilities and speech and visual abnormalities (Haslam, 2000). Athetotic cerebral palsy: This is also referred to as extrapyramidal CP or dyskinetic CP. It affects the entire body and often causes uncontrolled, slow movements. It is seen in children who have suffered from kernicterus in the new born period (Haslam, 2000). Its incidence has diminished now owing to the aggressive management of neonatal hyperbilirubinemia these days. Children suffering from this type of CP are usually hypotonic with marked head lag and poor head control. These children may have difficulty in feeding. They have drooling of saliva due to tongue thrust. The athetoid movements become evident at the age of one year. Involvement of oropharyngeal muscles leads to speech difficulties. Seizures and mental retardation are rare (Haslam, 2000). Ataxic cerebral palsy: This is the least common of the major types of cerebral palsy. It affects depth perception, balance and coordination. Hypotonic cerebral palsy: In this form of CP, the child is hypotonic. This form of CP eventually turns into either spastic or athetotic form. Mixed forms of CP: Children may have more than one of the 4 forms. Differential diagnosis CP needs to be differentiated from other conditions like spinal cord dysfunction, temporary motor problems resulting from closed head injuries, seizures, drug overdoses, or some brain tumors, muscular dystrophy, peripheral neuropathies such as Charcot-Marie- Tooth disease, osteogenesis imperfecta, progressive neurologic disorders (including Retts syndrome, leukodystrophy, and Tay-Sachs disease), chromosomal anomalies ( trisomy 13 and 18) and congenital disorders (hereditary spastic paraplegia) (Wilmington, "Cerebral palsy program"). Diagnosis The diagnosis of CP is purely clinical. CP may be suspected in a child in whom development of mile stones is delayed and the child has abnormal muscle tone, abnormal movements, abnormal reflexes and persistent infantile reflexes. Infact, making a definite diagnosis of cerebral palsy is not always easy, especially before the childs first birthday (Wilmington, "Cerebral palsy program"). Investigations CT Scan and MRI are usually asked for to rule out differential diagnosis. However, these investigations may show some abnormalities in children with CP like hydrocephalus, cysts, etc. In those with spastic hemiplegia, CT Scan or MRI may show an atrophic cerebral hemisphere with a dilated ventricle contralateral to the side of the affected extremities. In neonates in whom focal seizures have occurred, these tests may show evidence of cerebral infarction, probably secondary to intrauterine thromboembolism (Haslam, 2000). CT/MRI in neonates with spastic diplegia may show periventricular leukomalacia, particularly in the area where fibers innervating the legs course through the internal capsule (Haslam, 2000). In those with spastic quadriplegia, cystic cavities may be seen in the brain. Prognosis It is difficult to predict the outcome of a child with CP, at an age less than one year, when it is difficult to establish the diagnosis itself. Ideally, it is only when the child is two years old that the physician can determine whether the child has hemiplegia, diplegia, or quadriplegia (Wilmington, "Cerebral palsy program"). Children with cerebral palsy do not stop doing activities once they have begun to do them, in the sense; there is no regression of developed skills. It is generally assumed that if a child is not sitting up by himself by age 4 or walking by age 8, he will never be an independent walker. It is also difficult to predict intellectual capacity even at the age of 2 years. Those who do not have mental retardation do much better than others who have (Wilmington, "Cerebral palsy program"). Complications Children with CP develop many complications which add to their disability. These include epilepsy, mental retardation, learning disabilities, attention deficit-hyperactivity disorder, swallowing problems, speech and hearing problems, visual deficits, gastroesophageal reflux, aspiration pneumonia, etc. Management 1. Role of the pediatrician: The pediatricians initial role consists of making a correct diagnosis, determining the etiology, identification of the type, evaluation of the extent and severity of the neuromotor deficit as well as of associated problems and disclosure of the diagnosis to the parents. The parents must be elaborated about the nature of the disease in their own language. The pediatrician also has a role in preparing a plan of management suited to the child, implement treatments and follow the progress of the child (Singhi, 2004). 2. Comprehensive approach: Because of the multiplicity of problems, a multidisciplinary team comprising of a neuro-developmental pediatrician as the team-leader, physiotherapist, occupational therapist, clinical psychologist, speech pathologist, orthopedic surgeon, otorhinolaryngologist, ophthalmologist, teacher, play therapist and social worker is required, preferably under one roof. 3. Evaluation: All children with CP must have evaluation of their motor, sensory and cognitive functions. Hearing and speech disorders, feeding problems, visual problems and behaviour problems should be routinely looked for (Singhi, 2004). 4. Physiotherapy (PT): PT, especially when started early in life, is helpful in promoting normal motor development, and preventing deformity and contractures. It reduces abnormal patterns of movement and posture and promotes the normal ones so as to enable the child to gain maximal functional independence. The most common technique used is the neurodevelopmental Bobath technique which consists of guiding the child through normal sequences of motor development, inhibition of primitive and abnormal reflexes, reinforcement of normal postural reflexes and facilitation of normal movements (Singhi, 2004). 5. Occupational therapy (OT): Ideally, both OT and PT should be done together. OT helps in day-to-day activities like feeding, bathing, dressing, toilet training, brushing, combing, etc. Co-ordination and sensory-perceptual integration can be taught and multisensorial stimulation provided through peg board, blocks and other toys of different colours, textures, sizes and shapes, and producing different sounds. Play therapy is one form of OT which uses natural activity in a young child, to help him consolidate the levels of development that he has reached and encourage him to move on, to the next level. It is important to teach the parents to manage the child properly at home. Play therapy advocates home- based management programme in which the parents are taught correct methods of handling and lifting the child and carrying out specific exercises (Singhi, 2004). 6. Role of assistive and adaptive devices: Children with CP may need these devices to help them go on with day-to-day activities. These include angled spoons, two handled cups, stiff cloth collar with velcro adjustment, cloth hammocks, a good stable seat with a back-support, abductor wedge, foot rest, and a detachable table, programmed wheel chairs, electronic feeding devices, various access systems, computerized speech systems, cochlear implants, specially designed shoes, ankle-foot orthoses and calipers (Singhi, 2004). 7. Medications: Drugs are mainly given to reduce spasticity. These must be started only if physiotherapy has failed to reduce spasticity. The drugs commonly used are baclofen, diazepam, tizanidine and dantrolene sodium. In severe cases, Botulinum Toxin may need to be given. Levo-dopa for severe athetosis and carbamezepine or trihexyphenidyl for dystonia may be helpful. 8. Role of surgery: Surgery may be considered in some children with spasticity, especially where mainly the lower limbs are involved. Tendon lengthening and transfer and arthrodesis are some of the procedures commonly performed. These procedures are undertaken after 8 years of age. Dorsal rhizotomy which involves selective resection of posterior nerve roots from L 2 to S 2 may be helpful in children with severe lower limb spasticity, with sufficient trunk control and some form of forward locomotion. Thalamotomy for athetoid CP, stereotactic dentatomy and chronic cerebellar stimulation via implanted electrodes have been tried in some cases but are still experimental procedures (Singhi, 2004). 9. Management of associated problems: Seizures may need to be managed with anti-epileptics with the advice of a neurologist. Those with hearing problems may need to use hearing aids. Many children have strabismus and refractory errors which require early corrective intervention by an Ophthalmologist. Augmented communication and use of information technology like speech synthesizers may be helpful in those with learning disabilities. Special feeding techniques and prokinetics may be necessary in those with feeding problems. Oral exercises may be necessary in those with excessive salivation. Behaviour modification techniques, anti-psychotics and parental counseling may be useful in those with hyperactivity and destructive behaviour. Sedatives may be given in those with sleep problem (Singhi, 2004). 10. Rehabilitation: Education, prevocational and vocational training need to be individualized according to the childs abilities, as determined by detailed assessment. References Haslam, R.H.A. (2000). Encephalopathies. In: Nelson Textbook of Pediatrics, 16th edn. Eds. Berhman RE, Kliegman RM, Arvin AM. Bangalore, Prism Books Pvt Ltd, Odding, E., Roebroek, M.E., & Stam, H.J. (2006). The epidemiology of cerebral palsy: incidence, impairments and risk factors. Disabil Rehabil., 28(4), pp. 83-91. Sharma, P., Sharma, U., & Kabra, A. (1999).Cerebral palsy- Clinical profile and predisposing factors.  Indian Pediatrics, 36, pp. 1038-1042. Singhi, P.D. (2004). Cerebral palsy-management. Indian J Pediatr [serial online], 71, pp.635-639. Retrieved on October 2nd, 2007 from: http://www.ijppediatricsindia.org/text.asp?2004/71/7/635/11142 Wichers, M.J., Odding, E., Stam, H.J., & van Nieuwenhuizen, O. (2005). Clinical presentation, associated disorders and aetiological moments in Cerebral Palsy: a dutch population- based study. Disabil Rehabil., 27(10), pp.583-9. Wilmington, D. Cerebral Palsy Programme. Retrieved on October 2nd, 2007 from: http://gait.aidi.udel.edu/res695/homepage/pd_ortho/clinics/c_palsy/cpweb.htm Read More