Understanding the disability that took Microsoft CEO's son's life

The passing away of Microsoft CEO Satya Nadella's son who was born with cerebral palsy has put the spotlight on the condition. Despite being the most common motor disability in childhood, misconceptions about it abound. Dr Sheffali Gulati, chief of child neurology division, department of pediatrics at AIIMS, Delhi, helps us understand cerebral palsy and the care people with it need.Q: What is cerebral palsy?A: Cerebral refers to the brain and palsy means a complete or partial loss of the ability to move a body part. Cerebral palsy is an umbrella term used to describe a group of conditions that cause abnormalities of movement, posture and feel of the body (tight or loose) with or without accompanying fits, problems in speech, intelligence, vision, and hearing. It results from damage to the developing brain. The damage for faulty development in the brain usually occurs while a baby is developing in the womb. Sometimes it occurs during birth, or shortly after birth. This damage to the brain is a one-time event and so in most cases the symptoms won’t get worse with time. The symptoms may seem to progress in children with uncontrolled fits, worsening spasticity or tightness with development of stiff joints or new onset of abnormal body movements.Q: What are the causes of cerebral palsy?A: The developing brain may be damaged before birth, during delivery or during early life. The causes before birth include poor maternal nutrition; infection in the first three months manifesting in the mother as viral infection; infection of the baby sac and the fluid it contains in the mother’s womb; multiple pregnancy; exposure to smoking, alcohol, radiation, or drugs; maternal diabetes, thyroid disease, increased blood pressure; genetic disorders, inborn disorders of metabolism; abnormal structure of the developing brain or poor antenatal care.The causes around the time of delivery could be prematurity, a difficult delivery, weak babies, failure to establish breathing at birth manifesting as a weak cry, jaundice after birth, infections in the brain, low blood sugar levels of the baby or prolonged fits.Brain infections, brain hemorrhage, head injury or prolonged fits in early childhood could also be possible causes of cerebral palsy.Q: Since the first signs appear in infancy or early childhood, they may be difficult for parents to spot. What are the symptoms that parents should watch out for?A: Most children with cerebral palsy are diagnosed between the ages of six months and two years. The first thing that is usually noticed is that a child is not developing at the normal rate. Also, the muscle tone may feel abnormal, or the baby may start to have abnormal movements. Children can also have associated co-morbidities like decreased intelligence, fits, speech and language problems, hearing problems, visual problems and squint or behavioral problems. Epilepsy is seen in almost 25-30% of children with cerebral palsy and co-occurring autistic traits in 7-9% of children with cerebral palsy.Delay in seeking medical care can prevent the child in reaching his or her full potential. Though cerebral palsy is not completely curable, early rehabilitative measures can improve the quality of life to a significant extent. With timely and appropriate intervention many patients can lead an active, self-supporting and long lives.Q: Intellectual disability is sometimes confused with cerebral palsyA: Both are different. Intellectual disability means a subnormal intelligence, inability to do daily activities and limited skills to function in society normally. On the other hand, cerebral palsy refers to impaired motor function i.e. use of muscles in the arms or legs is impaired. Someone can have cerebral palsy and have normal intelligence, and someone can have intellectual disability but have no motor impairment. However, approximately two-thirds of children with cerebral palsy have some form of intellectual disability and those with spastic quadriplegia (i.e. all four limbs are stiff) are more likely to have intellectual disability.Q: What are the types of cerebral palsy?A: The classification of cerebral palsy is based on the feel of the body, the pattern of involvement of limbs and the type of movement disorder.The most common type of cerebral palsy is spastic in which the affected muscles are more stiff than normal. Some muscles may become permanently contracted and there can be associated bone and joint deformities. This is further subdivided into three types: quadriplegic (where all four limbs are involved and the arms are more or equally involved than the legs), hemiplegic (the leg and arm on one side of the body are affected) and diplegic (both the legs are affected).Dyskinetic or dystonic is another type of cerebral palsy that is associated with abnormal twisting postures or abnormal movements of the trunk and limbs. Sometimes the tongue or face muscles are affected. The tone (stiffness) of the muscles can vary from too high to too low. There are various terms used in context of this type of cerebral palsy: dystonic (presence of abnormal twisting postures of the trunk and limbs) athetoid (presence of slow writhing movements of hands and feet) or chorea (presence of jerky, dancing, flowing movements involving both proximal and distal extremities).Then there’s ataxic cerebral palsy. Children with ataxic cerebral palsy have difficulties with balance and fine movement. This can mean being unsteady when walking or difficulty in doing fine tasks like writing.Mixed cerebral palsy is characterised by a combination of various types, most commonly spastic with dystonic or dyskinetic type.Q: How is cerebral palsy diagnosed?A: The diagnosis is essentially clinical and does not involve laboratory investigations. Brain imaging is done to find out the extent of damage to the brain and the possible cause. If some specific type of fits are to be investigated or there is doubt about the presence or absence of fits or a decision regarding discontinuation of a drug against fits is to be taken, an electroencephalogram (EEG) is performed which is essentially the recording of electrical activity in the brain. Visual and hearing evaluation is done when indicated. A GER (gastro-oesophageal reflux) scan is sometimes performed if there are choking episodes and to rule out any muscular abnormality in the upper part of the food pipeQ: What does treatment for cerebral palsy involve? Can it be prevented?A: There is no cure for cerebral palsy, but much can be done to limit the degree of disability. This requires a multidisciplinary approach involving pediatricians, neurologists, psychologists, physiotherapists, occupational therapists, special educators, speech therapists, dieticians, social workers, ophthalmologists, ENT specialists and orthopedics.The treatment can help improve the quality of life of those with cerebral palsy. Physical therapy, which includes exercises or use of walkers or wheelchairs, for instance, aims at achieving physical independence and mobility for patients. Occupational therapy can increase independence in activities of daily living like feeding, seating or going to the toilet.Early developmental stimulation involves stimulating preceptory units of the body like vision, touch, hearing by talking, singing or playing music. Causes of cerebral palsy largely remain unknown and therefore, by extension, it is not preventable. However, about 10% of causes result from perinatal factors like delay at the time of birth, hence supervised delivery by trained professionals can prevent these. Q: Has there been any new research that has the potential to help patients with cerebral palsy?A: In the past decade, there has been a rapid surge in the use of technology-based interventions in the field of neuro-rehabilitation in cerebral palsy. Technology can play a vital role in facilitating mobility, optimizing communication, and enhancing self-care in children with cerebral palsy. Robots can perform high-quality, supervised movements while also providing joint support and real-time feedback. Multi-channel neuromuscular stimulation-assisted gait training can help achieve a more functional walking pattern. Communication aide designs for children can range from manual signs and graphic symbols to more complex devices that synthesise speech. Virtual reality, augmented reality, brain computer interface, artificial intelligence and trans-cranial neuro-stimulation are upcoming interventional modalities.Health on your mind? Subscribe to our weekly health newsletter here