Disease Information


Shingles (Herpes Zoster)

From the CDC

http://www.cdc.gov/nip/diseases/varicella/faqs-gen-shingles.htm

Contents:

  1. What is shingles?
  2. Is shingles contagious?
  3. What should I do if I get shingles?
  4. Can someone who has been vaccinated for chickenpox develop shingles?
  5. Is there a vaccine to prevent it?

 

  1. What is shingles?

Shingles, or herpes zoster, is caused by the chickenpox virus that remains in the nerve roots of all persons who had chickenpox and can come out in your body again years later to cause illness. 

Shingles is more common after the age of 50 and the risk increases with advancing age. Shingles causes numbness, itching or severe pain followed by clusters of blister-like lesions in a strip-like pattern on one side of your body. The pain can persist for weeks, months or years after the rash heals and is then known as post-herpetic neuralgia.

  1. Is shingles contagious?

Yes, people with shingles are contagious to persons who have not had chickenpox. Therefore, people who have not had chickenpox can catch chickenpox if they have close contact with a person who has shingles. However, you can not catch shingles itself from someone else. Shingles is caused by the chickenpox virus which has been dormant (staying quiet) in your body ever since you had chickenpox. So, you get shingles from your own chickenpox virus, not from someone else.

  1. What should I do if I get shingles?

Contact your health care provider as soon as possible to discuss treatment with antiviral medications. These medications are most effective if given as soon as possible after rash onset.

  1. Can someone who has been vaccinated for chickenpox develop shingles?

Yes. However, a study conducted among children with leukemia determined that after receiving the vaccine these children were much less likely to develop shingles than children who had prior natural chickenpox. Available information from healthy children and adults suggest that shingles is less common in vaccinated healthy persons compared with persons who have had natural chickenpox.

  1. Is there a vaccine available to prevent or modify shingles?

No; however, a study is currently underway with a new formulation of the chickenpox vaccine to determine whether vaccination of persons older than 55 years of age will reduce the frequency and/or severity of shingles in adults. Results from this study will be available in about 5 years.


 Vagus Nerve Stimulator- Depression

 Article From:   Neuropsychopharmacology 25: 713-728, 2001 

Biological Psychiatry
Prepublication Article Abstract

Vagus Nerve Stimulation (VNSTM) for Treatment-Resistant Depressions: A Multicenter Study

A. John Rush, Mark S. George, Harold A. Sackeim, Lauren B. Marangell, Mustafa M. Husain, Cole Giller, Ziad Nahas, Stephen Haines, Richard K. Simpson, Jr., and Robert R. Goodman
Accepted 12/6/99

Abstract

Background: Vagus nerve stimulation (VNSTM), as delivered by the NeuroCybernetic Prosthesis (NCP®) System, is an approved therapy for treatment-resistant epilepsy. We examined the safety and potential antidepressant effects of VNS for treatment-resistant, major depressive episodes (MDEs).

Methods: Adult outpatients ( n = 30) with nonpsychotic, treatment-resistant, major depressive ( n = 21) or bipolar I ( n = 4) or II ( n = 5) (depressed phase) disorder, whose current MDE was >2 years in duration or who had >4 MDEs in a lifetime and who also had failed at least 2 robust medication trials in the current MDE were eligible. Each participant completed up to a 4-week baseline (preimplantation) period, while on stable antidepressant regimens or while not taking antidepressants ( n = 5). Thereafter, the NCP System was implanted, followed by a 2-week, single-blind, recovery period (no stimulation). For the next 2 weeks, stimulation parameters were adjusted. Thereafter, VNS was provided at a fixed (maximum comfortably-tolerated) dose for 8 weeks.

Results: In the current MDE, patients had failed to respond to 2 ( n = 9), 3 ( n = 2), 4 ( n = 6), or >5 ( n = 13) robust antidepressant medication trials; 57% had also received ECT. Mean length of the current MDE was 10.3 years (median = 4.7) (range = 0.3-49.5). Baseline 28-item Hamilton Depression Rating Scale (HDRS28) scores averaged 38.0 >5.5. Response rates (>50% reductions in baseline scores) were 40% by HDRS28, 50% by the Montgomery-Åsberg Depression Rating Scale (MADRS), or 40% by the Clinical Global Impressions-Improvement (CGI-I) (1 or 2). Several subscales of the Medical Outcomes Survey (MOS) Short-Form (SF-36) revealed substantial functional improvement in responders. As of this writing, improvements obtained acutely have been sustained in acute study responders seen in longer-term follow-up ( n = 10).

Conclusions: These positive open trial results in a severe, treatment-resistant patient group suggest that VNS is a safe and effective treatment for a significant proportion of these patients.


© 1999 by the Society of Biological Psychiatry
Published by
Elsevier Science Inc.

bpswebmaster@elsevier.com


 Vagus Nerve Stimulator- Seizure Disorders

by Sheryll Collins, RN   

The Vagus Nerve stimulator is a device to control seizures for all ages that have seizure disorders that are not well controlled with seizure medications. It has been a God send for many people.

 The Vagus Nerve Stimulator or VNS is a device much like a pace maker that is surgically placed in the skin flap just under the left clavicle in the chest wall. The device has a lead wire that is attached to the left vagus nerve. All under the skin. This device is battery operated and is set by computer to deliver an electrical charge at different intervals and intensities to the vagus nerve. The electrical impulse stops seizure activity or reduces the frequency and number of seizures a patient may have in a day. It makes it possible for the person to reduce or eliminate
the need for medication.

See Cyberonics, Inc for more info on vagus nerve stimulators


GeneReviews: Angelman Syndrome

Angelman syndrome (AS) is characterized by: 1) severe developmental delay or mental retardation; 2) severe speech impairment; 3) gait ataxia and/or tremulousness of the limbs; and 4) a unique behavior with an inappropriate happy demeanor that includes frequent laughing, smiling, and excitability. In addition, microcephaly and seizures are common.  

Phenylketonuria

Phenylketonuria (PKU) can be defined as a rare metabolic disorder caused by a deficiency in the production of the hepatic (liver) enzyme phenylalanine hydroxylase (PAH). PKU is the most serious form of a class of diseases referred to as "hyperphenylalaninemia," all of which involve above normal (elevated) levels of phenylalanine in the blood. The primary symptom of untreated PKU, mental retardation, is the result of consuming foods that contain the amino acid phenylalanine, which is toxic to brain tissue.

Mental retardation Disability Info: Mental Retardation Fact Sheet (FS8)

Mental retardation means having lower than normal intellectual functioning with limitations in adaptive functioning. Mental retardation occurs in people of all races and ethnicities and is the most common

developmental disorder, affecting about 1 percent of the general population. People with mental retardation score low on intelligence (IQ) tests, but the effects on day-to-day functioning depend on the severity of the retardation and associated impairments.

Autism is a developmental disorder that is characterized by impaired development in communication, social interaction, and behavior. Autism is classified as a Pervasive Developmental Disorder (PDD), which is part of a broad spectrum of developmental disorders affecting young children and adults--the Autistic Spectrum Disorders (ASD). The range of these disorders varies from severely impaired individuals with autism to other individuals who have abnormalities of social interaction but normal intelligence--Asperger's syndrome. The ways in which autism is exhibited can differ greatly. Additionally, autism can be found in association with other disorders such as mental retardation and certain medical conditions. The degree of autism can range from mild to severe. Mildly affected individuals may appear very close to normal. Severely afflicted individuals may be extremely retarded and unable to function in almost any setting.

Treatments for toxoplasmosis in pregnancy (Cochrane Review) http://www.update-software.com/abstracts/AB001684.htm
Toxoplasmosis is a widespread parasitic disease and usually causes no symptoms. However, infection of pregnant women may cause congenital infection, resulting potentially in mental retardation and blindness in the infant. The objective of this review was to assess whether or not treating toxoplasmosis in pregnancy reduces the risk of congenital toxoplasma infection.

 

Alcoholism - Alcoholism is common, serious, and expensive. Physicians confront cirrhosis, cardiomyopathy, pancreatitis, and gastrointestinal bleeding due to alcoholism on a daily basis. Wernicke encephalopathy and Korsakoff psychosis are important causes of chronic disability as well. Fetal alcohol syndrome is a leading cause of mental retardation. In addition, suicide and homicide are important consequences of alcoholism. Alcohol-related diseases are discussed in separate articles. This article focuses on screening, diagnosis, and treatment and discusses new research findings on the natural history and….

Mental Retardation - Mental retardation (MR) and other neurodevelopmental disabilities are seen often in a general pediatric practice. Approximately 10% of children are learning impaired, while as many as 3% manifest some degree of MR. The population prevalence of these combined disorders of learning rivals that of the common childhood disorder asthma. MR originates during the developmental period (i.e., conception through age 18 years) and results in significantly subaverage general intellectual function with concurrent deficits in functional life skills. The diagnosis...

eMedicine - Epilepsy in Children with Mental Retardation : Article ... 
Epilepsy in Children with Mental Retardation - Traditionally the expression mental retardation (MR) was applied to individuals with significant cognitive deficiencies, and psychometric testing was the main instrument in the definition as well as in the determination of the degree of mental retardation. More modern definitions, like the one presented by the American Association on Mental Retardation, emphasize the importance of functioning in a social context and levels of needed supports. Most of the information presented in this article is ...

Adaptive and Assistive Technology @ RehabTool.com http://www.rehabtool.com/
Leading-edge assistive and adaptive technology products and services for children and adults with disabilities. Free product searches and referrals. Augmentative communication devices, speech software, computer access equipment, cognitive rehabilitation aids for the
special needs of the disabled.

Exceptional Parent Magazine - Eparent.com - parenting those with disabilities or http://www.eparent.com/
Exceptional Parent is the leading publication for the
special needs community. Empowering parents and professionals by sharing ideas, support, products and resources in the care and parenting of children with disabilities and special healthcare needs.

Siblings factsheet: Contact a Family - for families with disabled ...
Below we highlight some of the issues that often crop up for siblings of a child with
special needs, and some examples of the ways parents have found of responding to these:


http://groups.msn.com/4OurKidsSupportforfamiliesofspecialneedskids Many articles have come from this group! Many thanks.

http://www.epilepsyfoundation.org/answerplace/Medical/treatment/medications/typesmedicine

Lennox-Gastaut Syndrome

Lennox-Gastaut Syndrome
  • Atypical absense
  • Tonic seizures
  • Drop attacks
  • Mental retardation
  • Slow spike wave
  • Onset before age 5

Lennox-Gastaut syndrome (also known as myoclonic-astatic epilepsy) is a combination of seizures usually including atypical absense seizures (starting with automatic behavior without conscious control); tonic seizures (stiffening) atonic or astatic seizures (drop attacks); mental retardation; a distinct slow spike-and-wave EEG; and onset between 1 and 5 years of age.

Some children are developmentally normal when the syndrome begins, but then lose skills, sometimes dramatically, in association with uncontrolled seizures. By age 6 most children with Lennox-Gastaut have some degree of mental retardation.

Children with Lennox-Gastaut syndrome typically have more than one type of seizure. The atonic-astatic (drop attack) seizures are most troubling because of the injuries caused by repeated falls. Many children wear protective helmets. The tonic (stiffening) seizures are most common during sleep, including nap time, whereas generalized tonic-clonic seizures (convulsions) occur most often on awakening.

Some of these children are prone to develop non-convulsive status epilepticus (a continuous seizure state that is associated with a change in the child's level of awareness. This requires medical intervention to bring it to an end).

As children with Lennox-Gastaut syndrome grow older, the types of seizures change. In most cases, the drop seizures abate. They are replaced by partial, complex partial, and secondarily generalized convulsions. Among teenagers with Lennox-Gastaut, complex partial seizures are the most common form.

This seizure syndrome is difficult to treat and often does not respond to the usual seizure medications. Other treatment choices may include the ketogenic dietvagus nerve stimulation therapy or occasionally corpus callosotomy surgery.

Medications for Epilepsy

http://www.epilepsyfoundation.org/answerplace/Medical/treatment/medications/typesmedicine

The following information is designed to help people with epilepsy become more familiar with seizure medications. It is not for use by health or other professionals to identify drugs, nor is it an exhaustive list.

The pictures shown are of brand name drugs. Their appearance differs from generic versions, and occasionally drug appearances change. The list of side effects provided is not exhaustive. For a complete list, consult your doctor, nurse, or pharmacist. More detailed sources of information on side effects include the drug's prescribing information sheet, or the Physician's Desk Reference.

Report any change in physical or mental health in someone taking these medications to the doctor. Safety in pregnancy has not been established for any of these medications and should be discussed with the doctor.

Treating Partial Seizures

Partial seizures are usually treated with medication or, in some cases, surgery or electrical stimulation of the brain.

Medication: Several drugs are available to treat partial seizures.They may be prescribed as a single drug, or in combination. Medication for epilepsy is designed to prevent seizures. It does not cure the underlying problem.

To get the best possible seizure control, the medicine has to be taken every day, on time, as prescribed. Stopping the medicine suddenly for any reason may cause serious rebound seizures.

Sometimes seizures continue even though the medication is being taken exactly as prescribed. Partial seizures, unfortunately, are often more difficult to control with medication than other types of epilepsy.

When medicines do not work and seizures are frequent, surgery may be considered.

Surgery: The most common form of surgery for partial seizures identifies and then removes a small area of the brain where the seizures are taking place.

Sometimes seizures stop completely after surgery; sometimes they continue at a reduced level; and sometimes the surgery does not help at all.

Medicine may be needed to maintain control, even after surgery.

If partial seizures are coming from many sites on one side of the brain and are spreading to cause sudden drop attacks, surgery to cut the connections between the two sides of the brain may be considered.

This operation, called a corpus callosotomy, is designed to prevent the drop attacks. The partial seizures are not likely to be affected. If the seizure site is in an area of the brain that cannot be removed, a surgical technique to cut certain connections between nerve cells (multiple sub-pial transection) may be recommended.

Vagus Nerve Stimulation: A third treatment option may be electrical stimulation of the brain via the vagus nerve in the neck. This new therapy is called vagus nerve stimulation (VNS). A small battery is implanted in the chest wall and programmed to deliver short bursts of energy to the brain.

Complex Partial Seizures

First Aid for Complex Partial Seizures

  • Do not restrain the person.
  • Remove dangerous objects from the person's path.
  • Calmly direct the person to sit down and guide him or her from dangerous situations. Use force only in an emergency to protect the person from immediate harm, such as walking in front of an oncoming car.
  • Observe, but do not approach, a person who appears angry or combative.
  • Remain with the person until he or she is fully alert.

Complex partial seizures affect a larger area of the brain than simple partial seizures and they affect consciousness.

During a complex partial seizure, a person cannot interact normally with other people, is not in control of his movements, speech, or actions; doesn't know what he's doing; and cannot remember afterwards what happened during the seizure.

Although someone may appear to be conscious because he stays on his feet, his eyes are open and he can move about, it will be an altered consciousness, a dreamlike, almost trancelike state.

A person may even be able to speak, but the words are unlikely to make sense and he or she will not be able to respond to others in an appropriate way.

Although complex partial seizures can affect any area of the brain, they often take place in one of the brain's two temporal lobes. Because of this, the condition is sometimes called "temporal lobe epilepsy."

"Psychomotor epilepsy" is another term doctors may use to describe complex partial seizures.

Simple Partial Seizures

A simple partial seizure

A simple partial seizure with motor symptoms.
Here the neuronal discharge begins in the motor strip
in the right hemisphere of the brain, affecting first one
muscle then another on the left side of the body as it spreads.
In (a) first the fingers then the hand and arm are jerking.
In (b) it has spread to the upper shoulder.
In (c) the woman's head is drawn towards her shoulder.
In (d) the leg is drawn up. The woman remains conscious but
unable to prevent her muscles' response to the excessive stimulation
they are receiving from her brain.

People who have simple partial seizures do not lose consciousness during the seizure. However, some people, although fully aware of what's going on, find they can't speak or move until the seizure is over.

They remain awake and aware throughout. Sometimes they can talk quite normally to other people during the seizure. And they can usually remember exactly what happened to them while it was going on.

However, simple partial seizures can affect movement, emotion, sensations, and feelings in unusual and sometimes even frightening ways.

Movement: Uncontrolled movements can occur in just about any part of the body. Eyes may move from side to side; there may be blinking, unusual movements of the tongue, or twitching of the face.

Some simple partial seizures start out with shaking of a hand or foot which then spreads to involve an arm or a leg or even one whole side of the body.

Emotions: A sudden feeling of fear or a sense that something terrible is about to happen may be caused by a simple partial seizure in the part of the brain which controls those emotions.

In rare cases, partial seizures can produce feelings of anger and rage, or even sudden joy and happiness.

Sensations: All five senses -- touch, hearing, taste, smell, and sight -- are controlled by various areas of the brain.

Simple partial seizures in these areas can produce odd sensations such as a sense of a breeze on the skin; unusual hissing, buzzing or ringing sounds; voices that are not really there; unpleasant tastes; strange smells (also usually unpleasant); and, perhaps most upsetting of all, distortions in the way things look.

For example, a room may suddenly seem narrower, or wider, than it really is. Objects may seem to move closer or get farther away. Part of the body may appear to change in size or shape.

If the area of the brain involved with memory is affected, there may be disturbing visions of people and places from the past.

Sudden nausea or an odd, rising feeling in the stomach is quite common. Stomach pain also may, in some cases, be caused by simple partial seizures.

Episodes of sudden sweating, flushing, becoming pale, or having the sensation of gooseflesh are also possible.

Some people even report having out of body experiences during this type of seizure. Time may seem distorted as well.

In many ways, our usual, comfortable sense of familiar things and places may be disrupted by a simple partial seizure.

Well-known places may suddenly look unfamiliar. On the other hand, new places and events may seem familiar or as if they've happened before, a feeling called déjà vu.

Simple partial seizures can also produce sudden, uncontrolled bursts of laughter or crying.

Absence Seizures (also called Petit Mal)

What the Seizure Looks Like:

A blank stare, beginning and ending abruptly, lasting only a few seconds, most common in children. May be accompanied by rapid blinking, some chewing movements of the mouth. The child or adult is unaware of what's going on during the seizure, but quickly returns to full awareness once it has stopped. It may result in learning difficulties if not recognized and treated.

absence seizure

An absence seizure. In (a) the child is eating normally. In (b) the seizure has affected his whole awareness;

his eyes have rolled back in his head and he is briefly immobilized. In (c) he is immediately awake and aware again.

What to Do:

No first aid necessary, but if this is the first observation of the seizure(s), medical evaluation should be recommended.

Temporal Lobe Epilepsy

temporal lobe

The temporal lobes are the site of one of the most common forms
of epilepsy, characterized by complex partial seizures.

The temporal lobes, one on each side of the head, just above the ears, are the sites of one of the most common forms of epilepsy. Complex partial seizures with automatisms (unconscious actions), such as lip smacking or rubbing the hands together, are the most common seizures in temporal lobe epilepsy.

Seventy-five percent of patients also experience simple partial seizures which may include such features as: a mixture of thoughts, emotions, and feelings that are hard to describe; sudden emergence of old memories or feelings of strangeness in familiar surroundings; hallucinations of voices, music, smells, or tastes, and feelings of unusual fear or joy. While partial seizures dominate, approximately half the people with temporal lobe epilepsy have generalized tonic-clonic seizures as well.

The seizures characteristic of temporal lobe epilepsy often begin in the deeper parts of the temporal lobe (part of the limbic system) which control emotions and memory. Memory problems may develop over time in people with this syndrome. Treatment is with medication or surgery, or in some cases, VNS therapy.

Landau-Kleffner Syndrome

Landau-Kleffner Syndrome
  • Begins between 3 to 7 years old
  • Progressive loss of speech
  • Seizures during sleep
  • Loss of IQ

Landau-Kleffner syndrome, a rare disorder, causes children to develop the inability to speak. They have trouble understanding speech and may seem not to hear or understand what is said to them. Many children will also have seizures; these seizures vary in type. Speech in children with the syndrome slowly declines over time. There are epilepsy-related abnormalities on the EEG, even though some of the children do not have seizures.

The syndrome typically begins in children between 3 to 7 years old. Understanding spoken language is usually affected, but the children may also lose the ability to speak as well. Seizures often occur while the child is asleep and may be quite infrequent. Simple partial and tonic-clonic seizures may occur. Treatment with standard anti-epileptic drugs is not very effective; treatment with steroids has been tried with some success. Multiple subpial transection, a form of surgery designed to spare the speech areas, has also been tried.

Language for many of these children will improve slowly over time, but may not return to a normal level for age. EEGs may continue to be abnormal, even when the speech has improved.

Symptoms and Signs of Cerebral Palsy If present in Infants and Toddlers

Source: Dzinkowkski, Rene C. and others. Cerebral Palsy a Comprehensive Review.  Nurse Practitioner. Feb. 1996. 45-59

Early Infancy (0-3 Months)

  • Excessive lethargy or irritability
  • High pitched cry
  • Poor head control
  • Weak suck/tongue thrust/tonic bite
  • Oral hypersensitivity
  • Decreased interest in surroundings
  • Stiff or floppy posture
  • Abnormal or prolonged reflexes (see information on abnormal reflexes)
    • Moro’s reflex
    • Asymmetric tonic neck reflex
    • Placing reflex
    • Landau reflex

Later Infancy

Inability to perform motor skills as indicated:
  • Control hand grasp by 3 months
  • Rolling over by 5 months
  • Independent sitting by 7 months
Abnormal Developmental Patterns:
  • Hand preference by 12 months
  • Excessive arching of back
  • Log rolling
  • Abnormal or prolonged parachute response
Abnormal Developmental Patterns after 1 year of age:
  • “W sitting” – knees flexed, legs extremely rotated
  • “Bottom shuffling” Scoots along the floor
  • Walking on tip toe or hopping

REFLEX REVIEW

Moor’s Reflex

A reaction to a startle stimulus (loud noise, sudden touch, etc.) in which arms elevate and then clasp to midline.  Normally present till 4-5 months of age.  Absence, asymmetry, or delay of this reflex may indicate motor deficits.  Persistence of this reflex past age 4-5 months is also an indicator.

Tonic Neck Reflex

Involves extension of the arm and leg on the side to which the head is passively turned and flexion of the limbs on the oppisiot side.  An asymmetric reflex is abnormal and indicates damage to the motor pathways if present constantly after the age of 6 weeks.

Parachute Response/Reflex

When held around the waist in a horizontal prone position and then lowering the infant slowly, head first to the surface.  By age 6 to 8 months the infant should respond by extending the arms and hands to break the “fall”.  If this response is asymmetrical it indicates an unilateral motor abnormality.

Placing Reflex

When the dorsal (back) side of the hand or foot is placed on the edge of a surface, such as a table, the infant will lift the extremity and place it on the flat surface.   This reflex as well as sucking, rooting, and grasping are present at birth and reflect the function of the spinal cord, brain stem , and possibly the basal ganglia (damage to the basal ganglia results in athetoid cerebral palsy—see types of CP).

Landau Reflex

When the infant is held in a horizontal prone position, the infant will lift head and extend the neck and trunk.  When the neck is passively flexed, the entire body will flex.  This reflex is present by 6 months and hypotonicity (low tone)  indicates motor system deficits.  


More great information on surgery for seizure disorders

Epilepsy Surgery
A Guide For Patients

                                                    

Brain surgery is a way of treating certain kinds of epilepsy that cannot be controlled with medication.

Brain Surgery

Epileptic seizures are produced by abnormal electrical activity in the brain. Surgical removal of seizure-producing areas of the brain has been an accepted form of treatment for over 50 years.

However, because of new surgical techniques and new ways of identifying areas to be removed, more of these operations are being done now than ever before, and with greater success.

Surgery can be performed on both children and adults. However, it is not a suitable treatment for everyone who has epilepsy, or for everyone with poor seizure control.


Introduction

  • Brain surgery is a way of treating certain kinds of epilepsy that cannot be controlled with medication.
  • Risks and benefits of surgery should be carefully discussed in advance with the doctors who are going to perform the operation.
  • Certain testing is necessary before the operation. In some cases, surgery for epilepsy requires two operations.
  • Not all patients are good candidates for surgery.
  • Having brain surgery does not guarantee that a person will be free of seizures or won't have to take medicine anymore. However, chances are good that most people will have fewer seizures after surgery and many will become seizure-free.
  • Not all epilepsy-related surgery is performed on the brain. Therapy which delivers pulses of energy to the brain through a large nerve in the neck (VNS therapy) requires a different type of surgical procedure to set the system in place.
  • Brain surgery and VNS implants are accepted treatments for relief of seizures and are covered by most health insurance plans.

Decisions

In trying to decide whether an adult or child will benefit from brain surgery, doctors want to know:

  • Is the problem really epilepsy?
  • Is it the kind of seizure that can be helped by an operation?
  • Have we tried hard enough to control the seizures with medicine, diet, or other treatment?
  • Might the condition get better without surgery?
  • Might it get worse without surgery?
  • Do the benefits outweigh the risks?
  • Can surgery be done safely in the affected area of the brain?

These are very individual questions with different answers for each person based on the medical history of the patient or his family; physical examinations; medical records; and a whole battery of pre-surgical tests.


Types of Surgery

All epilepsy surgery involves the brain. However, different types of operations may be done. In general they fall into two main groups:

  • Removal of the area of the brain that is producing the seizures.
  • Interruption of nerve pathways along which seizure impulses spread.


Lobectomy
Seizures that begin in one or more areas of the brain are known as simple or complex partial seizures. The seizures can take on different forms, depending on where they originate in the brain. The brain is divided into areas called lobes. There are temporal lobes, frontal lobes, parietal lobes and occipital lobes. There are two of each lobe on either side of the head. An operation to remove all or part of these areas is called a lobectomy. This type of surgery may be performed when a person has seizures that start in the same lobe every time. It is sometimes possible to stop the seizures by removing the seizure-producing area if it can be safely done without damaging vital functions.

Hemispherectomy
A lobectomy removes a fairly small area of the brain. However, in rare cases a child may have severe brain disease on just one side of the brain which produces uncontrollable seizures and paralysis on the opposite side of the body.

When this happens, a much more extensive operation may be considered.

It is called a hemispherectomy. It removes all or almost all of one side (hemisphere) of the brain.

It seems impossible that someone could function with only half a brain, but in children the half that is left may take over some of the functions of the part that was removed. However, there will be weakness and loss of some movement on the opposite side of the body. There will also be a loss of peripheral (side) vision.

Corpus Callosotomy
Another kind of surgery for epilepsy is called a corpus callosotomy (split brain surgery).

The corpus callosotomy operation does not take out brain tissue. Instead, it interrupts the spread of seizures by cutting the nerve fibers connecting one side of the brain to the other. This nerve bridge is called the corpus callosum.

The seizures which may respond to this type of surgery include uncontrolled generalized tonic clonic (grand mal) seizures, drop attacks, or massive jerking movements.

These seizures affect both sides of the brain at once and there is usually no one area which can be removed to stop them from happening.

Seizures are usually not stopped entirely by the operation. Some type of seizure activity on one side of the brain or the other is likely to continue, but the effects are generally less severe than the repeated drop attacks or convulsions.

The corpus callosotomy operation is often done in two steps. The first operation partially separates the two halves of the brain but leaves some connections in place.

If the generalized seizures stop, no further surgery is done. If they continue, the doctors may recommend a second step that completes the separation.

Multiple Subpial Transection
Some seizures originate in or spread to parts of the brain that are responsible for functions such as movement or language. Removing these areas would lead to paralysis or loss of language function.

A surgical technique called multiple subpial transection (MST) may be performed in these situations. It involves making small incisions in the brain which interfere with the spread of seizure impulses.

This technique may be used alone or in addition to a lobectomy.


Pre-Surgical Testing

Before any operation for epilepsy can be performed, there has to be a period of careful testing and evaluation.

These tests are done to make sure the surgery has a good chance of being successful and won't affect any of the important functions of the brain.

Most of the tests are used to pinpoint the area of the brain where seizures begin or to locate other areas, like speech and memory, that have to be avoided.

How many tests have to be done depends on the kind of operation that is being planned and how much information each test produces.

The following tests are most often used before a decision to operate is made:

  • Electroencephalography (EEG) tests record electrical activity in the brain and identify areas of the brain where seizures occur
  • Magnetic resonance imaging (MRI) scans take pictures of the inside of the brain. MRI scans may show tumors, abnormal blood vessels, cysts, and areas of brain cell loss or other brain damage.
  • Simultaneous video (TV) monitoring and EEG recording help identify the type of seizure that is taking place.
  • Neuropsychological tests, including IQ, memory, and speech tests, tell doctors more about where the seizures (or the brain damage which is causing the seizures) are located.
  • An intracarotid sodium amobarbital test locates speech and memory centers. A drug is injected into an artery leading to the brain. It puts half of the brain to sleep for a short period of time. The doctors then check speech and memory on the side of the brain not put to sleep.
  • Positron emission tomography (PET) scans may be used in certain cases to help identify where seizures are taking place. PET measures how intensely different parts of the brain use up glucose, oxygen, or other substances.
  • Single photon emission computed tomography (SPECT) scans also help identify where seizures are taking place by measuring blood flow.

Tests Using Implants
Even after all the previously described tests are done, additional information may be needed to identify the epileptic area in the brain. This is because the area of seizure activity sometimes can't be found by electrodes attached to the surface of the head.

To obtain that additional information, two separate operations may be required.

The first operation places electrodes in or on the brain itself. These special electrodes are called depth or subdural electrodes.

After they are placed, the patient remains in the hospital with the head wrapped in a large dressing, with wires attached to the electrodes coming out of the dressing. Seizures are then recorded directly from the brain, often on simultaneous video and EEG. This process is called electrocorticography.

Both kinds of recording instruments may be kept in place for some time while doctors monitor signals from within the brain during seizures.

The brain may be stimulated with mild electrical impulses via the electrodes to identify special areas controlling speech, movement and sensation. In addition, further electrical recording to map out the seizure focus (the exact area to be removed) may be done.

If the tests show that there is a single epileptic area and it can be removed safely, a second operation is performed to remove the affected area. If not, surgery is done only to remove the electrodes.

Sometimes all the tests and procedures rule out surgery as a suitable treatment. Other times the tests may fail to give enough information and the doctors may decide not to recommend surgery.


The Operation

Successful epilepsy surgery depends on careful selection of patients and a skilled medical and surgical team.

The operation may take several hours to perform, as surgeons first locate and then remove the area of the brain identified in pre-testing as the source of the seizure activity, or carefully sever the nerve fibers between the two halves of the brain if a split brain operation is being performed, or make the incisions required by the MST procedure.

EEG recordings during the surgery help the physicians map out the exact area of brain to be removed.

The brain may be stimulated with mild electrical impulses during the operation itself to identify special areas controlling speech, movement and sensation.

Sometimes the whole operation is done with the patient awake but under local anesthetic. This is possible because brain tissue is not sensitive to pain.

Recovery
After the operation, the patient stays up to a week in the hospital and then goes home and continues to recuperate. After about three to eight weeks he or she can usually go back to normal activities.

Doctors usually recommend that surgery patients stay on antiepileptic medicines for up to two years after the operation. Some people may have to continue with medication indefinitely to retain seizure control.


Benefits and Risks

Lobectomies
While there are risks in all surgical procedures, including the placement of depth electrodes and grids, most brain surgery for epilepsy appears to be relatively safe. The success rate for epilepsy surgeries depends on the type of operation performed and can usually be predicted after all the test results are available.

For temporal lobectomies, 65 to 85% of patients will be seizure-free.

Complications occur in about 4 out of every 100 of these operations. Depending on the kind of surgery that's performed, possible complications include: partial losses of vision, motor ability, memory or speech. Infection or temporary swelling of the brain may also sometimes happen.

Corpus Callosotomies
Among patients having a corpus callosotomy (split brain operation), risks of major and minor complications after surgery are around 20 per 100 operations. Generalized seizures may stop or happen less often than before the operation. Partial seizures (that is, changes in movement, feeling or emotion without loss of consciousness) will probably continue and may even get worse. Still, the uncontrolled drop attacks and generalized tonic clonic seizures that the operation is designed to treat have risks of their own. Decisions to operate take all these possibilities into account.

Hemispherectomies
Excellent results for this operation, which involve removal of one half or almost one half of the brain, are being reported by the small number of very specialized centers doing these operations. However, there are more risks with hemispherectomies than with other types of epilepsy surgery.

Children who have hemispherectomy operations will continue to have loss of function on the side of the body opposite the side where the brain was removed.


Preparing For Surgery

In preparation for surgery you should do the following:

  1. Write down any questions you may have. You will see the neurosurgeon for a presurgical visit before you come to the hospital and you will be seen again in the hospital, the day before your operation. The neurosurgeon will explain the details of the operation and answer any questions you have about risks, complications and benefits of surgery. It is important that you fully understand the operation.
  2. Take your medication in the usual dose.
  3. You will be admitted to the hospital the day before your operation to prepare you for surgery. Your head will be shaved. You may want to bring a head covering to wear home from the hospital.
  4. If your surgery involves the side of the brain which controls language (usually the left), brain mapping is done to preserve language function. The procedure involves reading short phrases or naming items presented on slides. The day before the surgery, you will go through a training session to be sure you are familiar with the mapping procedure. You will see the same slides that will be used during the operation so that you will know what to expect and can fully cooperate with the doctors during surgery.

Recovering From Surgery

Most patients are able to be out of bed two days after surgery. Plan to be in the hospital for one to two weeks. However, it will be one to three months before you will be ready to return to work or school.

After surgery you may experience some of these post-operative symptoms. Usually, these symptoms go away spontaneously.

  1. Numbness of the scalp
  2. Clicking in your ears when you open and close your jaws
  3. Difficulty opening your mouth fully
  4. Headaches
  5. Feeling depressed or tired for several weeks after surgery
  6. Auras - Some patients find that auras continue for weeks or months after surgery. This does not necessarily mean that your seizures will come back.
  7. Post-operative seizures. Some patients have a few seizures for 7-10 days after surgery. This does not necessarily mean that your seizures are back permanently.
  8. Memory or word-finding difficulties - For 2-6 weeks, patients who have had surgery on the dominant or left side of their brain may have difficulty remembering names or words. This problem usually goes away.
  9. Immediately after surgery, your hair will begin to grow back at a normal rate (approximately 3/4 of an inch per month).

More Info & Links

Theodore H. Schwartz, MD - Epilepsy Surgery
Theodore H. Schwartz, MD - EPILEPSY-SURGERY.COM
Timothy D. Nichols, M.D.: Gamma - Knife: A Tool for Brain Surgery
Johns Hopkins Medicine - Gamma Knife

Johns Hopkins Epilepsy Center
Epilepsy Foundation of America Answer Place - Surgery
Cyberonics Vagus Nerve Stimulator
Epilepsy Foundation of America Answer Place - Vagus Nerve Stimulation
The VNS from a Patient's Point of View

Information came from these sources above- can be published


Down Syndrome and Autistic Spectrum Disorder: A Look at What We Know

This article looks at Down's Syndrome with ASD.

SEX EDUCATION FOR PEOPLE WITH AUTISM

This article talks about sex education at different levels.

Breaking down Barriers: Finding the right way to discuss sexuality

Tuesday, May 25, 2004

By Tina Calabro

Having a straightforward talk about sexuality is an awkward task for many parents, but even more challenging for parents of children with developmental disabilities.

Despite physical, emotional and/or intellectual differences, people with developmental disabilities are sexual beings who have a right to experience the richness of life, including love and intimacy. Many of us do not know how to help our children grow up in this important way.

I look at my delightful 9-year-old son who has cerebral palsy and I have trouble picturing how he, with his significant physical limitations, will be able to have an intimate relationship. When he falls in love, will his affections be reciprocated or understood?

Even more troubling is the fear that one's child may be taken advantage of sexually. Statistics show that people with developmental disabilities are victims of sexual abuse in disproportionately high numbers.

Is it any wonder the subject of sexuality is so overwhelming?

Fortunately, this topic is gaining more attention. On May 27, Temple University Institute on Disabilities, in collaboration with several local groups, is bringing internationally known author and sexuality educator David Hingsburger to Pittsburgh for a workshop entitled "Sexuality and People with Disabilities" (See the More Info box for details.)

Hingsburger is considered an authority on this issue.

"He transmits a very powerful message that people with disabilities are sexual beings just like the rest of us," said Celia Feinstein, assistant director of the Institute on Disabilities. "He's right in your face and he tells it like it is."

Hingsburger's visit highlights the important work of sexuality educators. Pittsburgh is fortunate to have some outstanding resources in its own back yard.

For the past seven years, Magee-Womens Hospital has offered classes for young people with disabilities. Entitled "The Social You, The Sexual You," these promote healthy self-esteem and sexuality in teens. The classes also review what kinds of talk and touch are appropriate in different relationships.

Magee's program was developed in response to requests from parents and disability advocates who recognized that insufficient sex education during the teenage years was producing a generation of confused and misunderstood adults. Jim DelBianco, a sexuality educator, and Elaine Beck, a nurse practitioner, teach the course.

The classes cover topics from puberty to sexually transmitted diseases to contraception and decision-making.

Another organization, Pittsburgh Action Against Rape (PAAR), offers programs focused on reducing risk of sexual assault.

"You don't want to scare people, but it does happen at alarming rates," explained Tricia Underwood, community trainer for PAAR. Children with special needs are two to five times more likely than other children to be victimized, studies show. Also, women with disabilities were more likely to experience sexual abuse from personal care attendants than are men.

PAAR offers psycho-education workshops for older teens and young adults with developmental disabilities. It also offers trainings for professionals who work with people with disabilities.

Underwood points out that adults with developmental disabilities often are unclear about appropriate sexual behavior. Unwittingly, parents and other well-meaning caretakers may set the stage for confusion when children are young. For example, a child who is considered "cute" when he or she hugs people indiscriminately may have a difficult time understanding why such behavior is inappropriate for an adult.

Parents and caregivers have an important role to play in helping children grow up to be healthy sexual adults.

"The sexuality of people with disabilities is not a technical issue," explained DelBianco, the sexuality educator at Magee. "It's not about body parts.

"Sexuality is what connects one person to another. It's what makes your life full and complete. Think about how important relationships are in your life. Why would it be any different for a person with a disability?"


(Tina Calabro of Highland Park is a freelance writer who focuses on disability issues. Her e-mail is tccalabro@aol.com)



 

Page last updated 5/31/04