Wednesday, March 31, 2010

Pediatric Neurological Pediatric Epilepsy

Pediatric neurological surgery refers to neurosurgical conditions that afflict children. These pediatric neurosurgery conditions vary from tumors affecting children to congenital malformations which present in childhood.

Congenital disorders are diseases with which a person is born. They include genetic disorders and malformations which occur in development or childbirth.


Follow each link below for more information about each specific condition:

  • Pediatric Brain Tumors
    • Medulloblastoma - Medulloblastoma is of the most common tumors that occur in children.
  • Pediatric Epilepsy - While epilepsy can affect people of any age, certain causes of epilepsy are more common to present in the pediatric population.
    • Febrile Seizures
    • Cortical Dysplasia
    • Hemimegalencephaly
    • Infantile Spasms
    • Sturge-Weber Syndrome
    • Tuberous Sclerosis
  • Congenital Hydrocephalus
  • Spina Bifida and Spinal Dysraphism
    • Spina Bifida Occulta
    • Meningocele
    • Myelomeningocele
    • Syringomyelia
    • Tight Filum Terminale
    • Diastematomyelia
    • Spinal Dermal Sinus
    • Spinal Lipoma

If you cannot find a particular type of pediatric neurological disease, use our Site Map and Search function to search this site. If you still cannot find it, it is possible we do not yet have an article for that specific condition. If that is the case, please Contact Us and let us know. We work hard to post new material often so that we can meet the needs of all of our readers.

Pediatric Epilepsy

What Is It?

Pediatric epilepsy is epilepsy, a disorder characterized by recurrent seizures, which occurs in a young patient. There are several potential causes of seizures and epilepsy in children.

Seizures are abnormal, rhythmic brain activity which can occur due to several pathologies. The types of seizures and presentation of epilepsy varies depending on the cause and the locations in the brain which are affected by each seizure. While the typical convulsions that most people associate with seizures can occur with some forms of epilepsy, other types can present with very different types of seizures.

Some possible causes of pediatric epilepsy:

  • Fevers: Febrile seizures are one of the most common causes of seizures in pediatric patients. They typically occur during a high fever when the child is sick with a viral illness. However, this is generally not considered epilepsy as most of these seizures are benign and most of these patients do not go on to have a lasting epileptic disorder.
  • Brain Malformations: Some malformations of the brain which occur during brain development can lead to epileptic conditions that present in childhood. For example, cortical dysplasia orhemimegalencephaly can present with pediatric epilepsy.
  • Brain Tumors: Certain brain tumors which can occur in children, such as some low grade gliomas, ganglioglioma or ganglioneuroma, can be associated with pediatric epilepsy.
  • Infection: Certain infections in or around the brain can lead to seizures. Some will only cause seizures during the infection but occasionally can lead to long-standing epileptic conditions. Examples include meningitis, cerebral abscess and encephalitis.
  • Idiopathic: In idiopathic condition is one for which we do not know the cause. Some children who present with epilepsy do not have a known cause or the cause eludes detection until extensive work-up is performed.

  • Other Causes: Other potential causes of seizures and epilepsy in children include traumatic brain injury, brain hemorrhage,arteriovenous malformation, Moyamoya disease, cerebral palsy or cerebral infarction.


What Types of Symptoms Are Typical?

The symptoms of epilepsy vary depending on the type and cause of the seizures. Generally, epilepsy is characterized by the recurrent occurrence of seizures. The frequency of seizures can vary greatly from patient to patient. In mild cases, seizures may only occur rarely while in severe cases seizures may occur many times a day.

One of the most common forms of seizure and the one with which most people are familiar is called a generalized tonic-clonic seizure. These are the seizures that present with an increase in muscle tone followed by muscle convulsions and loss of consciousness. However, there are many other possible presentations of seizure. Absence seizures do not have motor convulsions and often present with staring spells with a dissociation of consciousness and automatic movements like smacking of the lips or others. Focal seizures may not lead to loss of consciousness and may only result in twitching or convulsions of one part of the body.


How Is The Diagnosis Typically Made?

The diagnosis of epilepsy can be complex as there are many causes and types of seizures. However, one of the most commonly employed tests is an electroencephalogram or EEG. This test allows physicians to non-invasively monitor the electrical activity at the surface of the brain. In many cases this can help confirm the presence of abnormal, seizure-like activity.

Additionally, many patients undergo imaging studies such as CT scan orMRI scan. These help to evaluate if there is any abnormality of the brain such as a brain tumor or brain malformations, for example. Finally, other more advanced tests may be required to help localize the source of the seizures in the brain. These can include PET scans or invasive monitoring (placing electrodes on or in the brain to monitor brain activity).

All of these aspects of diagnosis for pediatric epilepsy vary considerably depending on the type and cause of epilepsy in each patient.


What Are Some Common Treatments?

Treatment for epilepsy varies considerably from patient to patient.

Treatment generally consists of medical treatments or surgical treatments. In general, patients with forms of epilepsy that are not due to a tumor or other surgical condition are started with medical treatment with anti-epileptic drugs. There are many such drugs which help to suppress seizure activity in the brain. If a patient's seizures are well controlled only with these medications then this may be the only treatment that is needed.

However, in cases where the seizures are not controlled adequately with medication, surgical treatments may be necessary. These vary but can include surgical removal of the part of the brain responsible for the onset of seizures, the seizure "focus". Likewise, if there is a known lesion in the brain such as a tumor or malformation, surgical removal of the lesion may be necessary both to treat the lesion itself as well as preventing further seizures.

Some pediatric epilepsy is adequately treated with these various treatment options while other severe cases may be very difficult to control. Because there is such variability from patient to patient, each child with epilepsy or any history of seizures should be worked up fully by their own physician who can recommend the best diagnostic and treatment options for their specific condition.

For more information about epilepsy and seizures in general, see ourEpilepsy section.

Febrile Seizure

What Is It?

A febrile seizure is a seizure or convulsions which is brought on by a fever in an infant or young child. Typically these occur with a temperature above 102 degrees F (about 38.9 degrees C). They occur most commonly in children between the ages of 6 months and about 5 years. In general, they are not uncommon, occurring in as many as one in 25 children. If a child has one febrile seizure, they are more likely than others to have additional febrile convulsions before they outgrow the tendency which is usually by around 5 years of age.

The seizures are generally characterized by a loss of consciousness with convulsions (shaking) of both sides of the body. However, some convulsions can include only part of the body, less commonly. They generally only last a minute or two but can be even more brief or longer in rare cases.


Will My Child Have Another Seizure?

The younger age a child has a first febrile seizure the more likely they will have more in their childhood. Additionally, children with frequent fevers are at higher risk. There also appears to be some genetic predisposition as a child with immediate family members with a history of these convulsions are at higher risk.


Are The Seizures Harmful and How are They Treated?

Most of these seizures are harmless, causing no long term injury to the patient or their brain. However frightening the convulsions may be to parents, there is no evidence that a small number of febrile convulsions in childhood significantly impairs the patient in any way later in life.

In rare cases a child may sustain injuries from falling or may choke on food or saliva in the mouth during the seizure.

Most patients with a seizure of any type will be worked up to be sure the patient does not have other reasons to have a seizure condition besides the fever. If the patients physician suspects another cause, such as a brain tumor, meningitis or some developmental disease, additional testing will be done to diagnose these conditions. Generally, uncomplicated seizures of this type do not lead to hospitalization or any specific treatment other than supportive care for the child's febrile illness.

General care of patients with febrile illnesses, such as fluids and medications to reduce the fever (acetaminophen or ibuprofen, for example) are generally recommended, although it is unknown if these measures help prevent these seizures in susceptible children. Most of these children are not proscribed anti-seizure medications. However, in severe cases in children who have recurrent convulsions, some anti-seizure medications may be prescribed at the first onset of illness to try to prevent these seizures in the future.

Each case is different, so each patient and their parents should consult their own treating physicians about the appropriate treatment options in their case.


Do Febrile Seizures Predict Future Seizures and an Epilepsy Condition?

The vast majority of patients who experience these seizures do not go on to develop ongoing epilepsy. However, a small percentage, probably less than 2 to 5 percent, are at increased risk for developing a long-term seizure condition. This is most common in children who have long seizures, seizures affecting only part of the body, frequent seizures within a 24 hour period or other neurological conditions such as cerebral palsy, delayed developement or other neurological disease. If these conditions are not present, the risk appears very small that an given child will develop epilepsy after experiencing a febrile convulsion. Again, each child is different and specific recommendations and prognosis information should be discussed with each patient's doctor.

Cortical Dysplasia

What Is It?

Cortical dysplasia is a malformation of the cortex of the brain which can lead to pediatric epilepsy conditions. It is considered congenital, meaning that it is present at birth, occurring during development of the embryo and fetus.

Normally, the brain cells, the neurons, are born during development in the innermost part of the brain, near the ventricles, the fluid-filled spaces within the brain. As they develop, they travel outwards to populate the outermost grey matter of the brain, the cerebral cortex. The normal cortex has a very specific and complex architecture with 6 primary layers of neurons. In some children this process of development does not occur properly in some parts of the brain. As a result the cortex in that area develops abnormally, often without the normal layers, architecture and connections that are required for the normal functioning of the brain.

This abnormal development is termed cortical dysplasia. It can occur in small, discrete parts of the cortex or can involve large areas of the brain. For example, cortical dysplasia involving an entire cerebral hemisphere is termed hemimegancephly.

While it can occur sporadically without any obvious cause, in some disorders it may be a part of a larger genetic abnormality of the child. An example of this are the tubers of Tuberous Sclerosis which resemble other forms of cortical dysplasia in many ways.


What Types of Symptoms Are Typical?

The most typical presenting symptom of these malformations is pediatric epilepsy, recurrent seizures in an infant or young child. The specific types of seizures and the neurological development of the child varies depending on many factors and the extent of involvement of the cortex. It is rare for seizures due to this disorder to present first in later childhood or adulthood.


How Is The Diagnosis Typically Made?

After presenting with seizures, a child will often undergo a thorough work-up which includes a neurological examination, imaging studies, such as aCT scan or MRI scan, and an electroencephalogram.

Cortical dysplasia can be subtle on MRI and practically invisible on CT. Other imaging studies such as PET scanning sometimes helps to find the abnormal location in the brain.

Electroencephalograms are performed by placing electrodes on the scalp and recording the electrical activity of the brain. Sometimes this can help localize where the seizures are coming from. If not, occasionally more invasive monitoring studies are performed to confirm the location of seizure origin.


What Are Some Common Treatments?

The treatment of epilepsy is complex and varies considerable from patient to patient. In general, treatment begins with anti-epileptic medications to help control the seizures. In some patients, this works well and is all that is needed.

In those patients who fail multiple medications or who have a very severe malformation, surgical treatment may be necessary. Generally, surgery is only performed if the neurologists and neurosurgeons can convince themselves that the seizures are in fact coming from one cortical dysplasia location. In such a case, some patients will be offered surgical treatment which involves identifying the malformation in the operating room and removing that part of abnormal brain. Success rates of these procedures vary considerably depending on the specific disease, the localization of seizures and many other factors.

As with all diseases of the nervous system, each patient is different.Epilepsy is a particularly complex example. Therefore, treatment suggestions and decisions should be made with the patient's own treating physician or physician team.


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