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Pediatric Neurology

 

About Pediatric neurology

Pediatric neurology is a specialized branch and very few trained doctors are available in India. It deals with disorders of inborn error of metabolism, developmental delay, cerebral palsy, childhood stroke, epilepsy, movement disorders, headache and neurodegerative disorders.

Cerebral palsy

Cerebral palsy is an umbrella term for describing a group of chronic disorders that impair a person’s ability to control body movement and posture. These disorders result from injury to the motor areas of the brain. Cerebral palsy affects two to six infants out of every 1,000 births, and is the most common disability among children in the India. The problem causing cerebral palsy may occur while the infant is still in the womb or after birth, and the problem is not always detectable during a child’s first year of life. Many of the infants born with cerebral palsy also experience some degree of mental retardation and/or have seizures.

Cerebral palsy may affect one arm or leg, an arm and leg on the same side, only the legs, all four limbs, or any combination of arms and legs. Whatever the affected areas may be, the muscle types involved are often the same. 

Patients with cerebral palsy can have a variety of symptoms. These symptoms usually do not worsen over time and include:

  • Difficulty maintaining balance and walking
  • Learning problems
  • Vision defects, such as crossed eyes
  • Speech difficulty
  • Sucking and swallowing problems
  • Difficulty with fine motor skills, such as writing and using scissors
  • Involuntary muscle movements

Spastic cerebral palsy

Spastic cerebral palsy is the most common form and is the type seen in 75 to 80 percent of cases. Patients with this form are unable to relax their muscles, which respond by tightening further if the patient or someone else tries to stretch them. This spasticity affects the function of individual muscles, especially flexor muscles.

Spasticity in the legs also affects the adductor muscles (the inner thigh muscles). Adductor muscles pull a body part toward its midline, such as those that pull the arms to a person’s side or close a person’s legs. In patients with cerebral palsy, the inward pull of the adductor muscles is so strong that the legs cross over each other or scissor. This motion also rotates the legs inward at the hips, pulling them away from the hip sockets, which can lead to abnormal socket development and hip dislocation.

Causes- Some of the known causes or contributors to cerebral palsy include:

  • Premature birth with internal bleeding in the baby's head
  • Lack of oxygen to the baby during development or delivery
  • An infection, such as cytomegalaovirus, rubella (German measles) or toxoplasmosis, during pregnancy
  • Drugs and/or alcohol abuse during pregnancy
  • Blood type differences between mother and fetus
  • Early separation of the placenta or damage to the umbilical cord
  • Excessive bile pigment (jaundice) in the baby's brain after birth
  • A viral infection that affects the brain (encephalitis)
  • Hydrocephalus
  • An infection of the membranes surrounding the brain and spinal cord (meningitis)
  • A severe head injury in the baby

Preventive methods, such as proper prenatal care, can eliminate some causes, while others are as yet unpreventable.

Diagnosis and treatment
Unless it is severe, cerebral palsy may be difficult to diagnose in a child’s first year of life. Because much of the development in this first year is based on motor functions, observation of these developing functions is often needed to make an accurate diagnosis. Actions such as reaching for toys, rolling over, sitting, standing, and walking develop during this time, and a delay in this development will prompt a physician to look for other physical symptoms if cerebral palsy is suspected. These signs may include abnormalities in muscle tone, movements, and reflexes. A doctor may also look at an infant’s hand preference. During their first year, babies normally do not show hand preference. But infants with cerebral palsy in only one side of the body may develop a hand preference early on, using their unaffected side to reach and grab for toys even if they are closer to their opposite, affected hand.

While physical observation of motor functions is the best method for diagnosing cerebral palsy, a physician may order other tests to rule out other neurological disorders. For example, computed tomography (CT) scans, magnetic resonance imaging (MRI), and head ultrasounds all take images of the brain and surrounding areas and may be useful in detecting serious conditions such as hydrocephalus (abnormal accumulation of fluid in the brain). While these tests are not used to confirm or rule out cerebral palsy, these scans may reveal the presence of brain cysts, scars, or other abnormalities that may have caused the cerebral palsy.

Although cerebral palsy cannot be cured, in many instances it can be effectively treated and managed.

Treatment often involves a combination of approaches and varies with each individual.

Physical therapy can teach patients how to train and exercise their muscles, speech therapy can help improve speaking and other tasks involving the mouth, and eye surgery or prescription lenses can repair or compensate for vision problems.

Some patients benefit from antispasticity medicines, while others require orthopedic surgery or neurosurgery to change the position of an arm or leg, so assistance devices such as crutches or braces can be used. Newer techniques include Botox injections directly into involved muscles to decrease muscle rigidity and Baclofen infusion pumps that provide a constant stream of muscle relaxant into the spinal fluid.

Headache

Headaches are very common in children and adolescents.  20% of adult headache sufferers say their headaches started before age 10, and 50% report their headaches started before age 20  By age 15, 5 percent of all children and adolescents have had migraines and 15 percent have had frequent non-progressive or tension-type headaches. Most headaches in children and adolescents are not the result of a serious illness but many parents worry that their child’s headache is the sign of a brain tumor or serious medical condition.

Migraines— a migraine is a moderate to severe headache that lasts from 1 to 24 hours and usually occurs 2 to 4 times per month. The pain is often throbbing and affects the front or both sides of the head. Common symptoms of migraines in children and adolescents include paleness (pallor), decreased appetite, dizziness, blurred vision, fever, stomach upset, nausea and vomiting. The child may also be very sensitive to light, noise or smells and want to sleep.

Most children and adolescents (90%) who have migraines have a family history of migraines. When both parents have a history of migraines, there is a 70% chance that the child will also develop migraines. If only one parent has a history of migraines, the risk drops to 25% to 50%. Children and adolescents with migraines may also inherit the tendency to be affected by certain migraine triggers, such as fatigue, bright lights, weather changes and other triggers.

Chronic non progressive headaches or tension-type headaches — frequent or daily headaches or headaches that come and go over a prolonged period of time without causing neurological symptoms. These are the most common types of headache among adolescents. About 15% of children and adolescents suffer from tension-type headaches.

Acute headaches are usually due to an illness, infection, cold or fever. Other conditions that can cause an acute headache include sinusitis (inflammation of the sinuses), pharyngitis (inflammation or infection of the throat) or otitis (ear infection or inflammation).

Evaluation of Headache
A headache evaluation may include a CT scan or MRI if a structural disorder of the central nervous system is suspected. Both of these tests

Muscular Dystrophy

Muscular dystrophy (MD) refers to a group of genetic diseases characterized by progressive damage and weakness of facial, limb, breathing, and heart muscles. It is due to the lack of a key protein that is needed to maintain the integrity and proper function of the muscle. As the muscle tissue is damaged, the muscle bulk is reduced. Sometimes the muscle tissue can be replaced with fat and excessive scar tissue to make muscle appears larger than normal.

MD is categorized as listed below based on the clinical features, including inheritance pattern, muscles affected, and muscle biopsy features:

  • Duchenne muscular dystrophy
  • Becker muscular dystrophy
  • Myotonic dystrophy
  • Facioscapulohumeral muscular dystrophy
  • Limb-girdle muscular dystrophy
  • Oculopharyngeal muscular dystrophy
  • Congenital muscular dystrophy
  • Emery-Dreifuss

More than 30 genes have been identified to cause different types of muscular dystrophies. Many muscular dystrophies are now diagnosed through gene tests.
MD can affect people of all ages. Although some forms first become apparent in infancy or childhood, others may not appear until middle age or later. Duchenne muscular dystrophy is the most common form affecting children, while myotonic MD is the most common form affecting adults.
There are three primary types of inheritance in which the faulty gene that causes MD can be passed along to offspring:

  • X-linked recessive: Genes that are X-linked recessive are carried by the female on one of the X chromosomes that determine the sex of the child. As such, only boys will inherit conditions determined by these genes. Their mothers, known as carriers, will usually not show signs of the disease. A son of a carrier of MD has about a 50 percent chance of developing the disease, while a daughter of a carrier has a 50 percent chance of being a carrier. If a boy is unaffected, he cannot pass on MD; however, daughters from a man with an X-linked dystrophy will all be carriers. Duchenne/Becker and Emery-Dreifuss are X-linked recessive.
  • Autosomal recessive: For this type of inheritance, both parents must carry and pass on the faulty gene. Neither parent shows any symptoms, but each of their offspring, regardless of gender, will have a 25 percent chance of developing the disease. Limb-girdle type 2 MD and distal myopathy are autosomal recessive.
  • Autosomal dominant: In the case of autosomal dominant inheritance, an affected person will have MD even though only one faulty gene has been passed along. This faulty gene can come from either parent, and it can affect either sex. Each child of an affected parent will have a 50 percent chance of developing MD. For this type of inheritance, the severity of MD can vary greatly. It can be so mild that it is not recognized, but it can also be severe. Myotonic dystrophy, facioscapulohumeral dystrophy (FSHD), and oculopharyngeal muscular dystrophy (OPMD) are autosomal dominant.

Diagnosing muscular dystrophy

After carefully evaluating a patient’s medical history, the doctor will perform a thorough physical exam to rule out other causes. If muscular dystrophy is suspected, there are a variety of laboratory tests that can be used to solidify a diagnosis. These tests may include:

  • Blood tests: When blood tests are performed to test for MD, the doctors are looking for an enzyme called creatine kinase (CK). This enzyme rises in the blood due to muscle damage or deterioration and may reveal some forms of MD before any physical symptoms appear.
  • Muscle biopsy: During a muscle biopsy, a small piece of muscle tissue is removed and then examined under a microscope. If MD is present, changes in the structure of muscle cells and other characteristics of the different forms of MD can be detected. The sample can also be stained to detect the presence or absence of particular proteins.
  • Electromyogram (EMG): An EMG is a test that measures the muscle’s response to stimulation of its nerve supply (nerve conduction study) and the electrical activity in the muscle (needle electrode examination). This test will confirm that the muscle weakness is due to a muscle disease, not a nerve disease.
  • Genetic tests: Many muscular dystrophies can be definitively diagnosed by testing for the mutated genes. The gene tests can spare muscle biopsies in these MD, including Duchenne, Becker, myotonic dystrophy, FSHD, OPMD, Distal, several forms of Limb-girdle, and Emery-Dreifuss dystrophies.

Treating MD
There is no cure for muscular dystrophy, although some drugs still in the trial stage have shown promise in slowing or delaying the progression of the disease. The only FDA-approved drug for Duchenne is a steroid, which may prolong ambulation by 2 years. For the time being, treatment is aimed at preventing complications due to the effects of weakness, decreased mobility, contractures, scoliosis, heart defects, and respiratory weakness.

Physical therapy: Physical therapy, especially regular stretching, is important in helping to maintain the range of motion for affected muscles and to prevent or delay contractures. Strengthening other muscles to compensate for weakness in affected muscles may be of benefit also, especially in earlier stages of milder MD. Regular exercise is important in maintaining good  overall health, but strenuous exercise may damage muscles further. For patients whose leg muscles are affected, braces may help lengthen the period of time that they can walk independently.

Surgery: If a patient’s contractures have become more pronounced, surgery may be used to relieve the tension by cutting the tendon of the affected muscle, then bracing it in a normal resting position while it regrows.
Other surgeries are used to compensate for shoulder weakness in facioscapulohumeral muscular dystrophy, and to keep the breathing airway open for people with distal MD who sometimes experience sleep apnea. Surgery for scoliosis is often needed for patients with Duchenne muscular dystrophy.

Occupational therapy: Occupational therapy involves employing methods and tools to compensate for a patient’s loss of strength and mobility. This may include modifications at home, dressing aids, wheelchair accessories, and communication aids.

Nutrition: Nutrition has not been shown to treat any conditions of MD, but it is essential to maintaining good health.

Cardiac care: Arrhythmias are often a symptom with Emery-Dreifuss and Becker MD and may need to be treated with special drugs. Pacemakers may also be needed in some cases, and heart transplants are becoming more common for men with Becker MD.

Respiratory care: When the muscles of the diaphragm and other respiratory muscles become too weak to function on their own, a patient may require a ventilator to continue breathing deeply enough. Air may also be administered through a tube or mouthpiece. It is therefore very important to maintain healthy lungs to reduce the risk of respiratory complications.

Like many other disorders, understanding and education about muscular dystrophy is the most important tool with which to manage and prevent complications. The following organizations can provide more information about muscular dystrophy:

Pediatric Sleep Disorders

Sleep disturbances are often due to temporary or chronic medical problems and are seen in as many as 25 to 30 percent of infants and children. They may range from insufficient sleep, bedtime settling problems and sleepwalking to sleep apnea and narcolepsy.

Sleep disorders in children may lead to daytime moodiness, irritability, lack of focus in class, sleepiness in school, inability to get up in time for school, and significant behavioral and learning problems. Some sleep disorders are serious enough to cause adverse cardiovascular and metabolic effects as well as failure to thrive.

In some cases it may be necessary to observe your child's sleep in the sleep laboratory. Here, in a comfortable bedroom-like setting, and with a parent nearby, children are able to sleep without much difficulty.

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