Procedure

Cerebral Palsy Management- Neurology

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Cerebral palsy is a neurological disorder that is caused due to injury to the brain during its development. The brain’s development begins in the third week of pregnancy and extends until late adolescence. A brain injury may occur at any stage of the development: before birth, during birth, or after birth. Of these, ~75% of cerebral palsy cases are caused due to factors associated with pregnancy or birth.

This disease affects the cognitive and motor skills (movement of muscles) in children which affects their ability to perform basic day to day activities, impeding their quality of life.

Epidemiology

For every 1000 live births, 2 infants develop cerebral palsy. About 10,000 infants and preschool children are diagnosed with cerebral palsy every year in the US. It is more commonly observed in males than in females.

Etiology

The exact cause of the disease is unknown. The presence of risk factors can interrupt the oxygen supply to the brain of the developing fetus or infant during birth. This causes brain damage leading to cerebral palsy. The most common risk factors or events associated with cerebral palsy are:

Risk factors before birth:

  • Preterm delivery (birth in less than 32 weeks of gestation)
  • Birth weight less than 5 pound and 8 ounces (2.5 kgs)
  • Retarded growth in the uterus (intrauterine life)
  • Trauma
  • Bleeding in the fetus’ brain (intracranial hemorrhage)
  • Stress or untreated hypothyroidism in mothers
  • Genetics
  • Multiple pregnancies (Twins or triplets)

Risk factors at the time of birth:

  • Decreased oxygen supply to the brain and other organs (Asphyxia)
  • Infections

Risk factors after birth

  • Bacterial meningitis (Inflammation of the protective membrane of the brain)
  • Viral encephalitis (Inflammation of the brain)
  • Hyperbilirubinemia (Increase in bilirubin levels in the blood)
  • Brain damage due to accidents or child abuse
  • Lack of nourishment

Classification and clinical features

Cerebral palsy is classified into three main types: Spastic, Athetoid, and Ataxia.

  • Spastic: This type constitutes ~80% of cerebral palsy cases. It is characterized by stiffness or tightness of muscles, wiggling movements of hands, tremors, muscle weakness, scissors gait (thighs and knees are pressed together), and walking on toes. It can be further divided into various subcategories such as diplegia (stiffness of legs or hands), hemiplegia (one side of the body is affected), quadriplegia (both hands and legs are involved).
  • Athetoid or dyskinetic: This is found in 10-15% of cerebral palsy cases. It is characterized by abnormally slow, jerky, and uncontrolled movements of hands or legs. These uncontrolled movements intensify during stress and are absent during sleep. This may also affect the muscles of the face and tongue, causing difficulty in talking, eating, and swallowing.
  • Ataxia: This is seen in 5-10% of cerebral palsy cases. It affects individuals’ sense of balance and coordination. They could develop tremors that may complicate their daily activities such as writing, walking steadily, holding things, or bringing hands together. They also have a wide-spaced gait (wide distance between feet while walking).

In general, intellectual and learning abilities of affected individuals are impaired, they develop seizures (fits), and lose their reflexes. Vision and hearing abilities are also impaired and affected individuals experience abnormal sensations of touch and pain, respiratory problems, bowel and bladder problems, and bone related abnormalities.

Diagnosis

In the early stages, doctors track the growth and development of the child over some time. The slow development of motor skills, abnormal muscle movements, and postures can indicate presence of the disease. In infants, head lag (backward drooping of head) can be observed due to poor head and neck control. Stiffness in the body is also exhibited, along with inability to roll, bring hands together, or crawl.

Several laboratory tests with imaging of the brain (MRI, CT scan, ultrasound) can help in the diagnosis. Other morbidities such as vision and hearing loss, seizures, and cognitive impairment can also point to the disease.

Management

There is no cure for cerebral palsy. A multidisciplinary intervention may help improve symptoms and the quality of life of the individual. The treatment includes a combination of physical and neurodevelopmental therapies, medications, surgical treatments, mechanical aids, and management of comorbidities.

Physical and neurodevelopmental therapies:

In cerebral palsy, muscles fail to relax due to muscle spasm. Poor muscle balance means that affected individuals can perform little movement. In these individuals, physical exercise such as muscle strengthening and fitness programs can improve the strength of muscle, speed of walking, and several other motor functions.

Neurodevelopmental therapy involves task-specific activities which enable individuals to perform day-today activities, improving their quality of life.

Medications

  • Intake of botulin toxin (Botox) can help in treating the stiffness of muscles. This medication relaxes muscles by blocking the release of chemicals such as acetylcholine. However, these injections are expensive, work temporarily (~3-6 months), and can only delay the required surgical intervention for a small period of time.
  • Many muscle-relaxing drugs such as baclofen, diazepam, and dantrolene can help in reducing muscle stiffness and wiggling movement of hands or legs. However, long term usage of these medications can have severe side effects.

Surgical approach:

  • Selective dorsal rhizotomy includes cutting part of the spinal cord segment from L1 (lumbar) to S2 (sacral) (lower regions of spinal cord) to decrease muscle stiffness.
  • In individuals with cerebral palsy, the prevalence of hip dislocation is very high. In such individuals, orthopedic surgeries can be performed to correct the deformity.

External aids

  • Orthoses are external devices or splints to support legs, hands, and spine, used along with other interventions to prevent inappropriate movements.

Management of comorbidities:

  • Horseback riding (hippotherapy) can help improve speech, language, and other motor functions in children with cerebral palsy.
  • A consultation with an ophthalmologist is recommended for vision-related disorders.
  • In most affected children, mental health is hindered as they are unable to perform basic functions, suffer from chronic pain, remain secluded from society, and face rejection from peers. So, it is important to educate parents regarding the challenges and ways to help their children overcome them.

Prevention of cerebral palsy

There are three main methods that could help reduce the rate of cerebral palsy:

  • Prior prescription of progesterone in females during pregnancy can reduce preterm births. In some, the use of prosthetic device (pessary) in the vagina or cervical stitch (cerclage) can reduce preterm births.
  • Research suggests that supplementation of magnesium sulfate in mothers reduces the risk of cerebral palsy in infants who were at risk of premature birth.
  • In affected newborns, a cooling treatment (Neonatal Therapeutic Hypothermia) can reduce the risk of birth asphyxia (decreased oxygen supply to the brain and other organs) by lowering the temperature of the newborn after birth.

Sources:

  • Krigger KW. Cerebral palsy: an overview. American family physician. 2006 Jan 1;73(1):91-100
  • Stavsky M, Mor O, Mastrolia SA, Greenbaum S, Than NG, Erez O. Cerebral Palsy-Trends in Epidemiology and Recent Development in Prenatal Mechanisms of Disease, Treatment, and Prevention. Front Pediatr. 2017; 5:21
  • Stiles J, Jernigan TL. The basics of brain development. Neuropsychol Rev. 2010;20(4):327-348. doi:10.1007/s11065-010-9148-4
  • Marret, S., Vanhulle, C., & Laquerriere, A. (2013). Pathophysiology of cerebral palsy. Pediatric Neurology Part I, 169–176.
  • Eunson, P. (2012). Aetiology and epidemiology of cerebral palsy. Paediatrics and Child Health, 22(9), 361–366.
  • Rosen, M. G., & Dickinson, J. C. (1992). The incidence of cerebral palsy. American Journal of Obstetrics and Gynecology, 167(2), 417–423.
  • Pakula, A. T., Van Naarden Braun, K., & Yeargin-Allsopp, M. (2009). Cerebral Palsy: Classification and Epidemiology. Physical Medicine and Rehabilitation Clinics of North America, 20(3), 425–452.
  • Sharan, D. (2005). Recent advances in management of cerebral palsy. The Indian Journal of Pediatrics, 72(11), 969–973.
About the Author:
Dr. Shilpy Bhandari is an experienced dental surgeon, with specialization in periodontics and implantology. She received her graduate and postgraduate education from Rajiv Gandhi University of Health Sciences in India. Besides her private practice, she enjoys writing on medical topics. She is also interested in evidence-based academic writing and has published several articles in international journals.
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