Calcium, vitamin D urged for epilepsy patients

Timothy F. Kirn

SEATTLE -- Bone loss in epilepsy patients is a serious problem that has not received enough attention, experts said at the annual meeting of the American Epilepsy Society.

Some of those experts urged that all epilepsy patients, regardless of the medications they might be on, should receive calcium and vitamin D supplementation.

Among the new information presented at the meeting was a retrospective study of 50 adult, male patients. Only 18% of those men got adequate exercise and only 12% got the daily recommended amount of calcium in their diet. Of five patients who underwent a bone scan, three had osteopenia/osteoporosis; they were 32 years, 47 years, and 52 years of age, and had had epilepsy since childhood.

Another study compared young patients on a ketogenic diet with young patients about to start the diet. All of the patients had been on multiple drugs, all had low bone density, and all were significantly below their expected height and weight.

Physicians who treat epileptic patients need a "wake-up call," said Dr. Richard H. Mattson, professor of neurology, Yale University, New Haven. Reports of fractures and osteoporosis associated with drug treatment of epilepsy date back to the late 1960s, yet the significance of those reports has not been fully appreciated.

Dr. Mattson said that he has been remiss himself in taking such reports too lightly. In the mid-1980s, he attended a conference in Europe at which the problem was discussed.

Most of the data came from Northern European countries and institutionalized patients, and he assumed that the patients simply did not get enough sun. "That conclusion, I have now decided, was incorrect," he said. A survey published in 2001 found that only 27% of neurologists screened their epilepsy patients for bone loss; only 7% advised their patients to take calcium and vitamin D supplements. "In my opinion, that is not adequate," Dr. Mattson said.

There are no figures on how frequently patients experience bone loss, and the literature is deficient in prospective clinical trials that have looked at the problem, said Dr. Mattson, who is one of the authors of the largest epilepsy drug trial ever conducted, the VA Epilepsy Cooperative Study That study failed to take note of fractures as adverse events.

A Swedish study of institutionalized patients reported that 10% sustained a fracture per year. Other reports suggest that epilepsy patients are at risk for rickets or osteomalacia, and poor dentition.

One of the earliest attempts to explain why epilepsy drugs might contribute to bone loss postulated that drugs such as phenytoin, phenobarbital, and carbamazepine might interfere with vitamin D metabolism by inhibiting cytochrome P450-mediated reactions. But valproate, which does not affect that enzyme, has been shown to cause bone loss.

Newer agents may not be free of bone effects, despite hopes that the bone problem would "fade into history" as a result of greater reliance on newer antiepileptic medications-levetiracetam, topiramate, and zonisamide, Dr. Mattson said. Topiramate, for example, can interfere with oral contraceptive effectiveness, and vitamin D and estradiol are somewhat similar molecules.

The survey of 50 adult male subjects (mean age 41 years) was designed to evaluate 15 primary and secondary risk factors known to be associated with osteoporosis.

In 74% of the subjects, the onset of epilepsy came before age 25, which is the age of peak bone density attainment in males, Dr. Mercedes P. Jacobson of Temple University, Philadelphia, said in a poster presentation.
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Complementary and Alternative Therapies for Epilepsy

Steve Helms
Complementary and Alternative Therapies for Epilepsy

Orrin Devinsky, MD, Steven Schachter, MD, and Steven pacia, MD (eds) Demos Medical Publishing, 386 Park Avenue South, New York, NY 10016 ISBN 1-888799-89-7; Hardcover; 330 pages; $79.95

Complementary and Alternative Therapies for Epilepsy reports the perspective of many professionals (MD, ND, DO, DC, PhD) in treating this confounding nervous system pathology. Therapeutics are covered in 31 chapters that range in diversity from neurofeedback, meditation, acupuncture, hyperbaric oxygen, hormones, and manipulation, to music, art and pet therapies. Each chapter is organized at the author's discretion, includes a foundational review of the treatment modality, and maintains a brief position paper written by the editors (a commentary section) that describes each modality's application in modern medicine. Presenting the modalities that have scientific efficacy, their application, and for whom they best apply is the stalwart focus of Complementary and Alternative Therapies for Epilepsy.

An evidence-based approach is noted throughout the text and highlights the academic occupations of the editors. The introductory chapters clarify the distinctions between different study methods and explain the validity of scientific studies. Of note is the chapter regarding the over-reporting of double-blind studies.

The broad range of modalities are grouped into seven distinct sections. All physicians will benefit from the knowledge of the use of meditation, exercise, and the ketogenic diet in epilepsy, while incorporating other therapies into patient care may necessitate referral. The chapter on herbal therapy also covers herb-drug interactions, while the chapter entitled, Comprehensive Neurobehavioral Approach, highlights the 12 essential steps for patients seeking control of their epilepsy. Numerous approaches to promoting endocrine regulation are also discussed, as this appears to play a part in seizure frequency and severity in some women.

Unfortunately, the information is sometimes presented at a rudimentary level and therefore the text should not be confused with a protocol-specific resource. Nonetheless, Complementary and Alternative Therapies for Epilepsy is a helpful and necessary text toward the relief of epileptic suffering. It will be of great benefit to practitioners trying to review epilepsy treatment options and acquire appreciation for a fitting patient referral. Overall, the text does a great service to medicine by acting as a bridge for skeptical physicians and even hospital and insurance administrators, while also presenting an overview of modalities not often discussed given society's focus on the immediate results of pharmacological drugs and surgery. Successful epilepsy treatments are a great persuader.
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Why A High Fat Diet Is Actually Good For You

The History of “Bad Fat”

For many years, it was common knowledge that fatty foods were the main reason to blame for weight gain. High fat diets were scorned by doctors because they cause weight gain, and they were also a main contributor to the prevalence of heart disease. Scientific studies that were carried out in the late 1940′s sought to answer the factors that contribute to coronary heart disease. In the end, they found that those who had elevated levels of cholesterol were more likely to be diagnosed with heart disease and ultimately die from it. In the coming decades, the American Heart Association began promoting a diet that they claimed would help to minimize the heart disease that was plaguing the country. This diet was low in fats, and as such, suggested that people try to reduce the amount of butter and other animal products that are high-fat. They said it would be beneficial to eat a diet that was made up of low-fat foods such as chicken and margarine.
The findings of these studies resulted in a number of fast-food establishments changing their fry oil to vegetable oil, which contains much less saturated fat than the palm oil and animal fats that were used previously. Unsurprisingly, the studies that prompted these actions had more public policy effects in the coming years. The classical “Food Pyramid” is really what drove this point home. Fatty foods were placed near the top and were supposed to be consumed sparingly. Carbohydrates, on the other hand, were chosen to be located near the base as their consumption was encouraged to be the healthier option. Scientific studies conducted over the last couple of decades have turned this idea on its head. It turns out that a diet that’s high in fats and low in carbohydrates is a great way to shed fat and keep it off. Even more intriguing is the idea that a high fat diet may actually protect the heart from diseases.

High A High Fat Diet Works

High fat diets work by depriving the dieter of carbohydrates, which in turn pushes the body into a state known as ketosis. This means that instead of burning glucose for its energy (which comes from carbohydrates), the body will instead turn fat into a usable supply of fuel. Several studies have shown that a high fat diet is a great promoter of weight loss and may even be effective in reducing the insulin needs of diabetics. When an adequate amount of both protein and fat are provided, those who choose a high fat diet will experience no muscle loss – something that cannot be said about other diets which limit overall caloric intake.
Many people find avoiding carbohydrates to be the hardest part of sticking with a high fat diet. Soda and other sugary beverages are a popular treat, same as is ice cream, candy and other dessert items. Unfortunately, the carbs don’t stop during dessert time. Bread, rice, potatoes and other starchy vegetables are also major sources of carbs. If you can manage to stick to it, a high fat diet that is low in carbs will pay off in the end.
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A Primer On Ketogenic Diet

If you are on a ketogenic diet, you will need to have a ketogenic diet menu to serve as your guide. You will also be happy to know that this particular diet is not complex and the kinds of food involved are not as varied as you might expect with a less restrictive diet. However, the menu isn’t something that you have to be thrilled about. It’s a type of restricted diet.
A ketogenic diet is a high-fat, adequate -protein, low-carbohydrate diet that is primarily used to treat difficult-to-control epilepsy in children. This resembles some aspects of starvation by forcing the body to burn fat rather than the carbohydrates. If you follow the menu, you will need to cut out most sources of carbohydrates such as bread, pasta, soda, sugar, ice cream, milk, pizza and even some types of fruits. The only carbs that you are allowed to eat are vegetables and some berries because they have lower carb content than the other fruits.
If you are suffering from obesity, type II diabetes or epilepsy, then a ketogenic diet menu should be very important to you. This type of diet is so effective that you may never have to take medication again. Even those who have serious metabolic disorder would rather eat a restricted menu than suffer an excruciating pain. Following this diet will take a lot of willpower, more so if you are suffering from junk food addition. It will also take time to be able to successfully follow through with this diet.
ketogenic diet menu consists of high-fat foods like:
  • meats
  • fatty fish
  • nuts
  • cream
  • cheese
  • butter
  • coconut oil
  • coconut milk

Vegetables are recommended as well. They include all varieties that are low in carbs. On the other hand, you might want to cut down on the potatoes.
Here’s an example of a simple ketogenic diet menu:
  • For breakfast: 15g coconut oil, frozen vegetables. Scrambled altogether on a pan
  • For lunch: Coconut milk smoothie with half a scoop protein powder, almonds and blueberries
  • For snack: A handful of two almonds
  • For dinner: Ground beef with 15% fat; fried in butter, vegetables on the side

 An example of a ketogenic diet menuA ketogenic diet should have a calorie ratio of 4:1 meaning, fat should equal 80% and carbs plus protein should equal 20%. It should raise ketone body levels to the point of testing positive with urine test strips. However, it is not really recommended that you count the calories or try so hard to reach a urinary ketone level. Rather, you should aim for a carbohydrate level under 50-80 grams a day. If you are really sick and on medication, then the menu should be made with the advice of your doctorn. It is also important that you consult a qualified nutritionist.
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Cerebral palsy

Richard Robinson
Cerebral palsy (CP), or static encephalopathy, is the name for a collection of movement disorders caused by brain damage that occurs before, during, or shortly after birth. A person with CP is often also affected by other conditions caused by brain damage.

The affected muscles of a person with CP may become rigid or excessively loose, or the person may lose control of muscles, or have problems with balance and coordination. A combination of these is also possible. The person may be primarily affected in the legs (paraplegia or diplegia), or in the arm and leg of one side of the body (hemiplegia), or all four limbs may be involved (quadriplegia).

A person with CP may also be affected by a number of other problems, including seizure disorder, visual deficits, hearing problems, mental retardation, learning disabilities, and attention-deficit/hyperactivity disorder. None of these is necessarily part of CP, however, and a person with CP may have no other impairments except for the movement disorder.

CP affects approximately 500,000 children and adults in the United States, and is diagnosed in more than 6,000 newborns and young children each year. CP is not an inherited disorder, and as of yet there is no way to predict with certainty which children will develop it. It is not a disease, and is not communicable. CP is a nonprogressive disorder, which means that symptoms neither worsen nor improve over time. However manifestation of the symptoms may become more severe over time; for example, rigidity of muscles can lead to contractures and deformities that require a variety of interventions.

Causes & symptoms
Cerebral palsy is caused by damage to the motor control centers of the brain. When the nerve cells (neurons) in these regions die, the appropriate signals can no longer be sent to the muscles under their control. The resulting poor control of these muscles causes the symptoms of CP.

This brain damage may be caused by lack of oxygen (asphyxia), infection, trauma, malnutrition, drugs or other chemicals, or hemorrhage. In most cases it is impossible to determine the actual cause, although prematurity is recognized as a significant risk factor. Although it was once thought that difficult or prolonged delivery was responsible for many cases of CP, most researchers now believe that the great majority of cases result from brain damage occurring before birth. The same injury that damages the motor areas can harm other areas as well, leading to other problems commonly associated with CP.

If brain cells do not get enough oxygen because of poor circulation, they may die. Defects in circulation in the developing brain may cause CP in some cases. Asphyxia during birth is also possible, and about half of newborns known to have suffered asphyxia during birth (perinatal asphyxia) develop CP. However, asphyxia during birth is usually considered a symptom of an underlying neurological problem in a newborn, rather than its cause, and the resulting CP may be another sign of that problem. Asphyxia after birth can be caused by choking, poisoning (such as from carbon monoxide or barbiturates), or near-drowning.

The fetal brain may be damaged by an infection contracted by the mother. Infections correlated with CP include rubella (German measles), toxoplasmosis (often contracted from cat feces or undercooked meat), cytomegalovirus (a herpes virus), and HIV (the virus that causes AIDS). Encephalitis and meningitis, infections of the brain and its coverings, can also cause CP when contracted by infants.

Physical trauma to the pregnant mother or infant may cause brain damage. Blows to the infant's head, as from a motor vehicle accident, violent shaking, or other physical abuse can damage the infant's brain. Maternal malnutrition may cause brain damage, as can the use of drugs, including cocaine or alcohol. Although these factors may cause CP, they may be more likely to cause mental retardation or other impairments.

Incompatibility between the Rh blood types of mother and child was once a major cause of athetoid CP, one type of movement impairment seen in cerebral palsy. In some cases, this incompatibility can cause the mother's defense (immune) system to attack and destroy the child's blood cells during the pregnancy, a condition called erythroblastosis fetalis. High levels in the child's circulation of a blood cell breakdown product called bilirubin can result, leading to yellowish pigmentation of the skin caused by bile (jaundice) and causing brain damage. This condition is now rare because of testing procedures that identify potential Rh incompatibility, and treatment procedures that prevent the mother's immune system from attacking the child's blood cells. Jaundice that does occur can be treated with special lights that help the breakdown of bilirubin. Blood transfusions for the child are also possible in extreme cases. Despite the virtual elimination of this cause of CP in the last few decades, CP rates have not declined, largely because of the increase of survival of premature babies.

Prematurity is one of the most significant risk factors for CP. About 7% of babies weighing less than 3 lbs at birth develop CP, and the risk increases dramatically as weight falls. Prematurity may increase the risk of CP because of the increased likelihood of hemorrhaging in the brain associated with low birth weight. Brain hemorrhage is most common in babies born weighing less that 4 lbs, and the risk increases as weight decreases. The hemorrhage may destroy brain tissue, either through asphyxia or release of toxic breakdown products.

The symptoms of CP are usually not noticeable at birth; as children develop through the first 18 months of life, though, they progress through a predictable set of developmental milestones. Children with CP will develop these skills more slowly because of their motor impairments, and delay in reaching milestones is usually the first symptom of CP. The more severe the CP, the earlier the diagnosis is usually made.

Selected developmental milestones, and the ages at which a child will normally acquire them, are given below. There is some cause for concern if the child does not acquire the skill by the age shown in parentheses.

Sits well unsupported--6 months (8-10 months)
Babbles--6 months (8 months)
Crawls--9 months (12 months)
Finger feeds, holds bottle--9 months (12 months)
Walks alone--12 months (15-18 months)
Uses one or two words other than dada/mama--12 months (15 months)
Walks up and down steps--24 months (24-36 months)
Turns pages in books; removes shoes and socks--24 months (30 months).
Children do not consistently favor one hand over the other before 18 months, and doing so may be a sign that the child has difficulty using the other hand. This same preference for one side of the body may show up as an asymmetric crawling effort, or continuing to use only one leg for the work of stair climbing after age three.

It must be remembered that children normally progress at somewhat different rates, and slow beginning accomplishment is often followed by normal development. There are also other causes for delay in reaching some milestones, including problems with vision or hearing. Because CP is a nonprogressive disease, loss of previously acquired milestones indicates that CP is not the cause of the problem.

The impairments of CP become recognizable in early childhood. The type of motor impairment and its location are used as the basis for classification. There are five generally recognized types of impairment:

Spastic--Muscles are rigid, posture may be abnormal, and fine motor control is impaired
Athetoid--Marked by slow, writhing, involuntary movements
Hypotonic--Muscles are floppy, without tone
Ataxic--Balance and coordination are impaired
The location of the impairment usually falls into one of three broad categories:

Hemiplegia--One arm and one leg on the same side of the body involved
Diplegia--Both legs; arms may be partially involved
Quadriplegia--All four extremities involved.
Therefore, a person with CP may be said to have spastic diplegia, or ataxic hemiplegia, for instance. CP is also termed mild, moderate, or severe, although these are subjective categories with no firm boundaries.

Loss of muscle control, especially of the spastic type, can cause serious orthopedic problems, including scoliosis (spine curvature), hip dislocation, or contractures. A contracture is a shortening of a muscle, caused by an imbalance of opposing force from a neighboring muscle. Contractures begin as prolonged contractions, but can become fixed or irreversible without regular range of motion exercises. A fixed contracture occurs when the contracted muscle adapts by reducing its overall length. Fixed contractures may cause postural abnormalities in the affected limbs, including clenched fists, tightly pressed or crossed thighs, or equinus. In equinus, the most common postural deformity, the foot is extended by the strong pull of the rear calf muscles, causing the toes to point. The foot is commonly pulled inward as well, a condition called equinovarus. Contractures of all kinds may be painful, and may interfere with normal activities of daily living, including hygiene and mobility.

As noted, the brain damage that causes CP may also cause a large number of other disorders. These may include:

Mental retardation
Learning disabilities
Attention-deficit/hyperactivity disorder
Seizure disorder
Visual impairment, especially strabismus ("cross-eye")
Hearing loss
Speech impairment.
These problems may have an even greater impact on the child's life than the physical impairment of CP, although not all children with CP are affected by other problems. About one-third of children with CP have moderate-to-severe mental retardation, one-third have mild mental retardation, and one-third have normal to above average intelligence.

The tracking of developmental progress is the most important test the physician has in determining whether a child has cerebral palsy. Most children with CP can be confidently diagnosed by 18 months. However, diagnosing CP is not always easy, since variations in child development may account for delays in achieving milestones, and since even children who are obviously delayed may continue to progress through the various developmental stages, and attain a normal range of skills later on. Serious or prolonged childhood illness may cause delays that are made up later on.

Evidence of other risk factors may aid the diagnosis. The Apgar score, evaluated immediately after birth, measures the newborn's heart rate, cry, color, muscle tone, and motor reactions. Apgar scores of less than 3 out a possible 10 are associated with a highly increased risk for CP. Presence of abnormal muscle tone or movements may signal CP, as may the persistence of infantile reflexes. A child with seizures or congenital organ malformation has an increased likelihood of CP. Ultrasound examination, a diagnostic technique that creates a two-dimensional image of internal body structures, may help to identify brain abnormalities, such as enlarged ventricles (chambers containing fluid) or periventricular leukomalacia (an abnormality of the area surrounding the ventricles), which may be associated with CP.

X rays, MRIs, and CT scans are often used to look for scarring, cysts, expansion of the cerebral ventricles (hydrocephalus), or other brain abnormalities that may indicate the cause of the symptoms. Blood tests and genetic tests may be used to rule out other possible causes, including muscular dystrophy (a disease characterized by the progressive wasting of muscles), mitochondrial (cellular) disease, other inherited disorders, or infection.

Cerebral palsy cannot be cured, but many of the disabilities it causes can be managed through planning and timely care. Treatment for a child with CP depends on the severity, nature, and location of the impairment, as well as the associated problems the child has. Optimal care of a child with mild CP may involve regular interaction with only a physical therapist and occupational therapist, whereas care for a more severely affected child may include a speech-language therapist, special education teacher, adaptive sports therapist, nutritionist, orthopedic surgeon, and neurosurgeon. Since CP is not a progressive disorder, its symptoms will not worsen with time. Nonetheless, the way in which those symptoms affect the growing child will change over time, and may require new strategies for treatment, adaptation, and compensation.

Parents of a child newly diagnosed with CP are not likely to have the necessary expertise to coordinate the full range of care their child will need. Although knowledgeable and caring medical professionals are indispensable for developing a care plan, a potentially more important source of information and advice is other parents who have dealt with the same set of difficulties. Support groups of parents of physically or mentally impaired children can be significant sources of both practical advice and emotional support. Many cities have support groups that can be located through the United Cerebral Palsy Association or a local hospital or social service agency. Children with CP are also eligible for special education services. The diagnosing doctor should refer parents to the local school district for these services. Even children aged 0-3 years are eligible through "early intervention."

The influence of CP on development
Much of a child's normal intellectual, physical, and social development occurs through play and exploration of the environment. The ability to reach for and grasp objects, to move about, to explore the properties of toys, and to communicate with others are all central activities in the child's growth. CP may restrict a child's ability to engage in these activities, and therefore prevent the acquisition of motor skills, knowledge of the world, and social competence. The family can do much to overcome these restrictions by adapting the child's environment to meet his or her needs and providing challenges within the child's abilities to accomplish. The advice and direction of an occupational therapist can be critical to promoting normal development of the child with CP.

Posture and mobility
Spasticity, muscle coordination, ataxia, and scoliosis are all significant impairments that affect the posture and mobility of a person with cerebral palsy. The physical therapist works with the family to maximize the child's ability to move affected limbs, to develop normal motor patterns, and to maintain posture. Adaptive equipment may be needed, including wheelchairs, walkers, shoe inserts, crutches, or braces. The need for adaptive equipment may change as the person develops, or as new treatments are introduced. The parents or other caregivers are taught safe transfer techniques to aid the person who cannot move independently from wheelchair to bed, for example.

Spasticity causes muscles to shorten, joints to tighten, and postures to change. Spasticity can affect the ability to walk, use a wheelchair, and sit unaided; and it can prevent independent feeding, dressing, hygiene, or other activities of daily living. Contracture and dislocations are common consequences of spasticity.

Mild spasticity may be treated by regular stretching of the affected muscles through their full range of motion. This usually is done at least daily. Moderate spasticity may require bracing to keep the limb out of the abnormal position, or serial casting to return it to its normal position. Ankle-foot braces (orthoses) made of lightweight plastic are often used to increase a child's stability and to promote proper joint alignment. Before fixed contracture develops, botulinum toxin (a highly active neurotoxin; Botox) injections can help loosen the affected muscles. Alcohol or phenol injection into the nerve controlling the muscle is also done; these injections are less costly than botulinum toxin, but they may cause more serious side effects, including pain, loss of sensation, and excess weakness. Fixed contractures are usually treated with either serial casting or tenotomy surgery. In this surgery, the tendons of the affected muscle are cut. The limb is then cast in a more normal position while the tendon regrows. Tenotomy is commonly done to prevent hip dislocation and to correct equinus. Another type of surgery available is dorsal rhizotomy. In this procedure, selected nerve roots in the spinal cord are cut to prevent them from stimulating the spastic muscles.

Spasticity may also be treated with muscle relaxing drugs, including diazepam (Valium), dantrolene (Dantrium), and baclofen (Lioresal). A baclofen pump can also be implanted to deliver the drug directly to the spinal cord, its site of action, allowing more effective spasticity reduction with fewer side effects.

A variety of experimental surgeries have been tried for people with cerebral palsy, most often to control spasticity. Most of these have not proved effective for most people. A new procedure implants an electrode into the brain to control the tremors found in some forms of CP, but as of 1998 the procedure was too new for its success to be fully evaluated.

Ataxia and coordination
Ataxia, or lack of balance control, is another factor affecting mobility. Physical therapy is an important tool to help the child with CP maximize balance. Coordination can be worsened if one member of a muscle pair is overly strong; bracing or surgical transfer of the muscle to a less overpowering position may help.

Scoliosis, or spine curvature, can develop when the muscles that hold the spine in place become either weak or spastic. In either case, an imbalance of forces pulls the vertebrae (bones making up the spinal column) out of alignment. This can cause pain, as well as interfere with normal posture and internal organ function. Scoliosis may be treated with a trunk brace. If this proves unsuccessful, spinal fusion surgery may be needed to join the vertebrae together, which keeps the spine straight.

Seizures occur in 30-50% of children with CP. Seizures may be restricted to one limb (focal) or generalized. Grand mal seizures are the most common type of generalized seizure for people with CP. Seizures may be treated with drugs, most commonly carbamazepine (Tegretol) or ethosuximide (Zarontin). A combination of a ketogenic diet and fasting may also be used to control seizures. Although the need for antiseizure medication is temporary in some children, it may be required throughout life for others.

Strabismus occurs in nearly half of all people with spastic CP. Strabismus may be treated with patching and corrective lenses. When these do not work, it may be treated with either surgery on the eye muscles causing the problem or by injection of botulinum toxin.

The person with CP may not be able to feed easily, because of poor coordination of the tongue and mouth muscles, or inability to hold and move utensils independently. The person may not take in adequate nutrition for full growth and development, worsening the results of the disorder. Careful attention to nutritional needs can prevent these problems. Nutritional supplements may be needed. Poor swallowing coordination may lead to aspiration, or inhaling of food or saliva. A speech-language therapist may be able to teach the person more effective movement patterns to avoid aspiration. In severe cases, a gastrostomy tube may be required to provide adequate nutrition directly into the digestive system while preventing aspiration. Nutrition may need to be monitored throughout childhood and adolescence, since meeting the increased food demands of a growing body may become difficult.

Other common medical problems
Drooling, dental caries (cavities), and gum disease are more common in people with CP than in the general population, partly because of lowered coordination and increased muscle tightness in the mouth and jaw. Each of these can be prevented to some degree, either through behavioral changes alone or in combination with drug therapy. Constipation is more common as well, and may be treated through dietary changes, or with enemas or suppositories when necessary.

Poor coordination of the tongue and mouth muscles can also affect speech. The inability to be understood can influence the child's intellectual development, especially if parents don't take the extra time needed to understand their child's attempts at speech. Children may benefit from picture boards or other communication devices that allow them to point to make their desires known. For school-age children or older persons with CP, there are a large number of augmentative communication devices, including shorthand typing programs and computer-assisted speech devices. A speech-language therapist can offer valuable advice on the types of equipment available.

The best choice of school for the child with CP depends on the presence and degree of mental impairment and physical impairment, as well as the facilities available in the area. "Inclusion," or mainstreaming the child in a regular public school classroom, may work well for the child with mild physical impairment. Separate classrooms or special schools may be needed for more severely involved children. Schooling for disabled students is governed by the Individuals with Disabilities Education Act (IDEA) at the federal level and state special education rules at the local level. An educational specialist either within the school system or from the community social services agency may be able to help the family navigate the various bureaucratic pathways that will ensure the best schooling available.

The process of developing an educational plan for a child with CP begins with an assessment of the child's needs. The assessment is carried out under state guidelines by a team of medical professionals. After the assessment, the school district works with the parents and others involved in the child's education and treatment to develop an Individualized Educational Plan (IEP). The IEP states the child's specific needs for special instruction and indicates what services will be provided. The special services may be as simple as allowing extra time to travel between classes or as extensive as individualized instruction, adapted classroom equipment, and special testing procedures. More information about assessments and IEPs is available through the National Information Center for Children and Youth with Disabilities. The United Cerebral Palsy Assocation is another resource for advocacy, information, and legal rights.

Behavioral and mental health services
The child with CP may have behavioral problems or emotional issues that affect psychological development and social interactions. These may require special intervention or treatment, including behavior modification programs or individual and family counseling. Attention-deficit/hyperactivity disorder is common in children with CP, and may require behavioral, educational, and medical intervention.

Alternative treatment
A number of people with cerebral palsy, both children and adults, have found systematic relief and enhanced quality of life from a combination of alternative and complementary treatments, including homeopathy, massage therapy, vitamin treatments, herbal medicine, and acupuncture.

Cerebral palsy can affect every stage of maturation, from childhood through adolescence to adulthood. At each stage, the person with CP and his or her caregivers must strive to achieve and maintain the fullest range of experiences and education consistent with the person's abilities. The advice and intervention of professionals remains crucial for many people with CP.

Although CP is not a terminal disorder, it can affect a person's lifespan by increasing the risk of infection, especially lung infections. Poor nutrition can contribute to the likelihood of infection. People with mild cerebral palsy may have near-normal lifespans. The lifespan of those with more severe forms, especially spastic quadriplegia, is often considerably shortened. However, over 90% of infants with CP survive into adulthood.

The cause of most cases of CP is unknown, but it has become clear in recent years that birth difficulties are not to blame in most cases. Rather, developmental problems before birth, usually unknown and generally undiagnosable, are responsible for most cases. Although the incidence of CP caused by Rh factor incompatibility has declined markedly, the incidence of CP as a consequence of prematurity has increased, because of the increasing success of medical intervention in keeping premature babies alive.

The risk of CP can be decreased through good maternal nutrition, avoidance of drugs or alcohol during pregnancy, and prevention or prompt treatment of infections. Recent preliminary research suggests that magnesium sulfate may reduce the risk of CP in mothers taking it for the medical treatment for preeclampsia and preterm labor.

Key Terms
Ataxic refers to a condition called ataxia, in which balance and coordination are impaired.
Athetoid refs to a condition called athetonia, which is marked by slow, writhing, involuntary muscle movements.
Attention-deficit/hyperactivity disorder
A behavioral disorder marked by inattentiveness, hyperactivity, and impulsivity.
Augmentative communication devices
Computers, picture boards, and other devices that increase the ability to communicate, either with or without speech.
A shortening of a muscle caused by an imbalance of force between opposing muscles.
Paralysis of corresponding parts on both sides of the body.
Dorsal rhizotomy
Surgical procedure that cuts nerve roots to reduce spasticity in affected muscles.
Dystonic refers to a condition called dystonia, in which fine motor control is confused.
A condition in which the foot is commonly pulled inward.
The most common postural deformity.
Paralysis of one side of the body.
Hypotonic refers to a condition called hypotonia, in which fine motor control is floppy, without tone.
Individualized Educational Plan; a plan that guides the delivery of services to a child with special education needs.
Ketogenic diet
A specialized diet designed to increase the blood levels of breakdown products known as ketone bodies. For unknown reasons, this aids in seizure control.
Perinatal asphyxia
Asphyxia that occurs during birth.
Paralysis of all four limbs.
Serial casting
A series of casts designed to gradually move a limb into a more functional position_as opposed to doing it all at once with one cast, as would be done in setting a broken bone.
Spastic refers to a condition in which the muscles are rigid, posture may be abnormal, and fine motor control is impaired.
Surgical procedure that cuts the tendon of a contractured muscle to allow lengthening.
Further Reading
For Your Information
Kramer, Laura. Uncommon Voyage: Parenting a Special Needs Child in the World of Alternative Medicine. Faber & Faber, 1996.
Miller, Freema, and Steven J. Bachrach. Cerebral Palsy: A Complete Guide for Caregiving. Johns Hopkins University Press, 1995.
Vickers, Andrew. Health Options: Complementary Therapies for Cerebral Palsy and Related Conditions. Element Publications, 1994.
Exceptional Parent Magazine 555 Kinderkamack Road, Oradell, NJ 07649-1517; 800-EPARENT, 201-634-6550.
Kuban, KCK, and A. Leviton. "Cerebral Palsy." New England Journal of Medicine 1994: 188-95.
National Information Center for Children and Youth with Disabilities. PO Box 1492, Washington DC 20013-1492. (800) 695-0285.
United Cerebral Palsy Association. 1660 L Street, NW Washington, DC 20036-5602. (800) USA-5-UCP, (202) 776-0406, (202) 973-7197 (TTY). (202) 776-0414 (fax).
Electronic forum for cerebral palsy
Gale Encyclopedia of Medicine. Gale Research, 1999.
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Atkins diet for epilepsy

Alan R. Gaby

Six patients (aged 7-52 years) with focal or multifocal epilepsy who had failed to respond to therapy with 2 to 18 anticonvulsant medications (median, 6.5) were started on the Atkins diet. Five patients maintained moderate-to-large ketosis for periods of 6 weeks to 24 months. Three patients had a significant reduction in seizure activity during that time and were able to reduce their anticonvulsant medications. Complete elimination of seizures occurred in a 7-year-old female and a 10-year-old male. An 18-year-old female had a 90% reduction in seizures. A 12-year-old female had a 20% reduction in seizures. A 42-year-old male and a 52-year-old male had no improvement. These results suggest that the Atkins diet may be beneficial for patients with treatment-resistant epilepsy, particularly younger patients.

Comment: Like the ketogenic diet, which has been found to be of great benefit for some people with epilepsy, the Atkins diet can induce a ketotic state. Compared with the very restrictive ketogenic diet, however, the Atkins diet has fewer protein and calorie restrictions and has been used with apparent safety by millions of people for weight reduction. There is some concern that long-term use of the Atkins diet can promote the development of osteoporosis, kidney stones and other problems. Presumably, some of these potential adverse effects can be prevented by appropriate supplementation with vitamins and minerals, as recommended by Dr. Atkins in his writings.

Kossoff EH, et al. Efficacy of the Atkins diet as therapy for intractable epilepsy. Neurology 2003;61:1789-1791.
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Advances in the Treatment of Epilepsy

Selim R. Benbadis

Significant advances have been made in the diagnosis and treatment of epilepsy over the past decade. With the advent of electroencephalographic video monitoring, physicians are now able to reliably differentiate epilepsy from other conditions that can mimic it, such as pseudoseizures. In addition, neuroimaging has changed the way treatment for difficult epilepsy is approached. As a result, the classification systems that have been in use since the early 1980s are currently being revised. A broader range of treatment options for epilepsy is now available. Many new antiepileptic drugs have become available in recent years, including felbamate, gabapentin, lamotrigine, topiramate, tiagabine, levetiracetam, oxcarbazepine and zonisamide. These medications offer options for patients with epilepsy whose seizures cannot be controlled using the classic agents. Several classic antiepileptic drugs have been modified and reformulated. The ketogenic diet has resurfaced as a treatment option in certain types of epilepsy. The vagus nerve stimulator, approved in 1997, represents a completely new treatment modality for patients with seizures not controlled by medications. Epilepsy surgery is now a well-documented and effective treatment for some patients with intractable epilepsy. (Am Fam Physician 2001;64:91-8,105-6.)

An estimated 1 to 2 percent of the U.S. population has epilepsy. In the past decade, more options have become available for patients with seizures. This article describes recent advances in the diagnosis and management of epilepsy.

New Concepts in Classification


An understanding of the conceptual distinction between "seizure" and "epileptic syndrome" is essential. The word "seizure" refers to an abnormal behavior (with symptoms or signs) that results from abnormal discharges of cortical neurons. It is an observable phenomenon that is finite in time. By contrast, the term "epilepsy" refers to a chronic condition characterized by recurrent seizures. A syndrome is a cluster of symptoms and signs that occur together but, unlike a disease, a syndrome does not have a single known etiology or pathology. Thus, epilepsy (or epileptic syndrome), in contrast to a seizure, cannot be diagnosed simply by direct observation or video review because the diagnosis requires other information, such as age of onset, etiology, family history, seizure frequency, imaging studies, precipitating factors, electroencephalography (EEG) and natural history.

Most types of epilepsy are characterized by more than one type of seizure. Patients with focal (or partial) epilepsy may have simple partial, complex partial and secondarily generalized tonic-clonic seizures (e.g., partial seizures with secondary generalization). Patients with generalized epilepsy may have one or more of the following seizure types: absence, myoclonic, tonic, clonic, tonic-clonic and atonic. Thus, no seizure type is specific for a single type of epilepsy. Seizures are symptoms, and patients should be treated for a type of epilepsy, not for a type of seizure.(1) Table 1 shows the main seizure types and their characteristics. Table 2 shows the main types of epilepsy.

   Seizure Types and Characteristics
   Seizure type            Characteristics
    Grand mal              Unconsciousness, convulsions, muscle rigidity
    Absence                Brief loss of consciousness
    Myoclonic              Sporadic (isolated) jerking movements
    Clonic                 Repetitive, rhythmic jerking movements
    Tonic                  Muscle stiffness, rigidity
    Atonic                 Loss of muscle tone
    Simple (awareness
     is retained)
     Motor symptoms        Jerking, muscle rigidity, spasms, head-turning
     Sensory symptoms      Unusual sensations affecting vision, hearing,
                            smell, taste or touch
    Autonomic symptoms     Stomach sensation
    Psychologic symptoms   Memory or emotional disturbances
                            (e.g., deja vu, fear)
    Complex (impairment    Automatisms such as lip smacking, chewing,
     of awareness)          fidgeting, walking and other repetitive,
                            stereotyped movements
    Partial seizure that   Begins as partial (simple or complex) and
     becomes generalized    evolves into grand mal seizure
   TABLE 2
   Comparison of the Main Types of Epilepsy
   Generalized epilepsy--seizure types: absence, myoclonic, tonic, clonic,
   tonic-clonic and atonic
     Idiopathic (genetic causes)
       Childhood absence epilepsy, juvenile myoclonic epilepsy, epilepsy with
         grand-mal seizures on awakening, others
     Symptomatic (cause known) or cryptogenic (cause unknown)
       West syndrome, Lennox-Gastaut syndrome, others
   Partial epilepsy--seizure types: simple partial, complex partial and
   secondarily generalized tonic-clonic seizures
     Idiopathic (genetic causes)
       Benign epilepsy of childhood with centrotemporal spikes ("Rolandic"
         epilepsy), others
     Symptomatic (cause known) or cryptogenic (cause unknown)
       Temporal lobe epilepsy, frontal lobe epilepsy, others

The international classification of epileptic seizures (ICES)2 that was published in 1981 is widely used by physicians as a diagnostic tool. Despite some advantages, the ICES has limitations, the most important of which is that it is based on clinical and EEG data.(2,3) An alternative seizure classification, the semiological classification, which is a purely symptom-based seizure classification, was recently proposed.(4) This simple classification consists of four major categories that correspond to symptoms affecting a different domain of behavior: sensorial (auras), consciousness, autonomic and motor. Because seizures can include symptoms in more than one domain of behavior, they are classified according to the predominant symptoms (without placing emphasis on particular symptoms as the ICES does). This classification can be made with the use of video recording but, because it is based exclusively on clinical data, it can be based on history alone.

The International League Against Epilepsy has recently acknowledged the shortcomings of the ICES system and is developing a new classification system.5 The League's system will establish four levels of classification: (1) a descriptive seizure classification largely based on the semiologic classification described above; (2) a pathophysiologic seizure classification; (3) an epileptic syndrome disease classification comparable to the existing epilepsy classification(1,6); and (4) a classification based on functional disability.


In addition to changes in the classification of seizures, the definition of status epilepticus is evolving. While the classic definition required 30 minutes of convulsions, experts now agree that treatment protocol for status epilepticus should be initiated after five minutes of convulsive seizures.(7)

Diagnosis: Advances in EEG


Despite tremendous recent advances in neuroimaging, the EEG retains an important role in the diagnosis of epilepsy because seizures are a disorder of electrical function rather than of structure. Routine EEG may be useful in supporting a clinical diagnosis of epilepsy by showing epileptiform discharges (e.g., spikes or sharp waves) because it is highly specific. However, a routine EEG typically consisting of a 20- to 30-minute sample of brain activity is not highly sensitive. Fewer than 50 percent of routine EEGs are abnormal in patients who are known to have epilepsy. While this yield increases with repeated EEGs, many patients with epilepsy continue to have normal EEGs.


Prolonged EEG-video monitoring is critical in providing information about electrographic seizures and seizure semiology (video).(8) The prolonged nature of the recording allows a more thorough analysis of the EEG, thus increasing the likelihood of capturing epileptiform discharges. This analysis is also aided by the use of automated spike detection. More importantly, video monitoring allows recording of the actual events for which medical attention is sought.

For monitoring purposes, medications are carefully reduced to allow a seizure to occur within a reasonable time. Seizures can be detected by the patient or a family member and signaled by pressing an alarm, or they can be detected by an automated EEG seizure-detection mechanism. Careful correlation between clinical semiology (video) and ictal EEG allows for a definitive diagnosis of epilepsy or nonepileptic events. About 15 to 20 percent of patients referred for resistant seizures do not actually have epilepsy but instead have psychogenic seizures.

A definitive diagnosis of epilepsy can only be made using EEG-video monitoring.(9,10) The correlation between clinical semiology and ictal EEG also allows epilepsy to be categorized as partial or generalized and, in most cases, for the zone of seizure onset to be located.8 These data are critical in deciding on the correct treatment options, including drug choice and surgical candidacy.


Ambulatory EEG, analogous to the Holter monitor for cardiac arrhythmias, allows the recording of electrographic seizures but does not permit correlation between EEG and seizure semiology. Ambulatory EEG can be a useful extension of routine EEG,(11) especially in the differential diagnosis of seizures and nonepileptic events. It does not, however, replace comprehensive EEG-video monitoring.


Invasive EEG is necessary only when surgery is being considered and a regular (scalp) EEG evaluation fails to identify the zone of seizure onset with sufficient confidence, or when the zone of seizure onset must be defined with high precision in relation to nearby cortex. Various techniques, each having advantages and limitations, are available, including subdural, epidural, foramen ovale and intracerebral (depth) electrodes.(12)


Many new drugs for the treatment of epilepsy have become available in the past eight years.(13,14) The new drugs whose labeling has been approved in the United States are (in the order of their release since 1993): felbamate (Felbatol), gabapentin (Neurontin), lamotrigine (Lamictal), topiramate (Topamax), tiagabine (Gabitril), levetiracetam (Keppra) and zonisamide (Zonegran). Each of these drugs was initially approved as adjunct treatment to a classic drug for refractory partial epilepsy. However, indications for these drugs are gradually broadening. For example, lamotrigine was recently labeled for monotherapy(15) and topiramate for treatment of primary generalized seizures.(16)

All of these drugs provide added seizure control and, in clinical trials on patients with highly refractory epilepsy, resulted in at least a 50 percent seizure reduction in 30 to 50 percent of patients. In clinical use in less selected populations, the efficacy of these drugs is even greater. They are also increasingly being used in settings other than epilepsy treatment, such as pain management and treatment of psychiatric disorders. Comparisons among these new drugs are difficult to make. In general, they are similar to each other in terms of efficacy. Therefore, the choice of a particular agent is often based on other factors, including side effect profile.

All anti-epilepsy drugs are central nervous system depressants and are associated with sedation, dizziness, ataxia, cognitive and visual disturbances, and gastrointestinal symptoms. These side effects are predictable, benign and dose- or rate-dependent. In most instances, the new antiepileptic drugs are better tolerated than the older drugs. However, significant differences exist among the drugs with regard to side effects, potential toxicity and pharmacokinetics.

Felbamate has a broad spectrum of activity in both partial and generalized seizures, but rare reports of fatal aplastic anemia and hepatic failure limit its use to patients for whom no other treatment alternative exists.

Gabapentin is characterized by excellent tolerability. It is not protein bound, has no appreciable hepatic metabolism and is excreted by the kidneys. Thus, gabapentin is appropriate for use in patients who require relatively quick titration, who have multiple drug intolerances or who are taking multiple drugs with the potential for interaction, including the elderly.

Lamotrigine has a broad spectrum of activity against multiple seizure types. Sedation is notably rare in monotherapy, and it even has an "alerting" response in some patients. One idiosyncratic side effect of lamotrigine, which is similar to effects of older antiepileptic drugs, is a rash. Infrequently (in less than 1 percent of adults), the rash can be serious and may progress to Stevens-Johnson syndrome, which can be life-threatening. Rashes are more common in children when lamotrigine is taken in association with valproate sodium (Depakote) and with rapid titration.

Topiramate also has a broad spectrum of activity. Weight loss has been noted, which can be a desirable lateral side effect. The development of nephrolithiasis, which is rare, and paresthesias, which is common, likely reflects carbonic anhydrase inhibition.

Tiagabine has no significant systemic or serious idiosyncratic adverse side effects, but it does have a relatively narrow spectrum of activity and must be titrated slowly. One limitation of lamotrigine, topiramate and tiagabine is that they need to be initiated at a low dosage and slowly increased in dosage over several weeks.

Levetiracetam is unique among the new antiepileptic drugs because it is effective starting with the initial dose. It also has a mechanism of action that appears to be different from that of other antiepileptic drugs and, like gabapentin, its tolerability and pharmacokinetics are very attractive. Levetiracetam is not metabolized by the liver (more than 60 percent is renally excreted unchanged), and less than 10 percent is protein bound. As a result, drug interactions are minimal.

Zonisamide has been used in Japan for 11 years and benefits from a large patient exposure, which supports its safety. Table 3(14) summarizes the principal characteristics of these new drugs.


Comparison of the Important Characteristics of New Antiepileptic Agents

                 Unique              Idiosyncratic
Agent            side effects        reactions

Felbamate        Headache,           Aplastic
 (Felbatol),      insomnia            anemia,
 1993                                 hepatitis

Gabapentin       Weight gain

Lamotrigine                          Rash

Topiramate       Nephrolithiasis,
 (Topamax),       paresthesias,
 1996             weight loss


Oxcarbazepine    Hyponatremia


Zonisamide       Nephrolithiasis,
 (Zonegran),      weight loss

Agent            Pharmacokinetics

Felbamate        Protein binding: 25 percent
 (Felbatol),     Metabolism: liver
 1993            Liver enzyme: inhibitor

Gabapentin       Protein binding: 0 percent
 (Neurontin),    Metabolism: kidney
 1993            Liver enzyme: none

Lamotrigine      Protein binding: 55 percent
 (Lamictal),     Metabolism: liver
 1994            Liver enzyme: inducer (mild)

Topiramate       Protein binding: 15 percent
 (Topamax),      Metabolism: kidney
 1996            Liver enzyme: inducer (mild)

Tiagabine        Protein binding: 95 percent
 (Gabitril),     Metabolism: liver
 1997            Liver enzyme: inducer (mild)

Oxcarbazepine    Protein binding: 40 percent
 (Trileptal),    Metabolism: liver and kidney
 1999            Liver enzyme: inducer (mild)

Levetiracetam    Protein binding: 0 percent
 (Keppra),       Metabolism: kidney
 1999            Liver enzyme: none

Zonisamide       Protein binding: 50 percent
 (Zonegran),     Metabolism: liver
 2000            Liver enzyme: inducer (mild)

                 Starting dosage/    Titration/
Agent            average dosage      administration

Felbamate        600 to 1,200        Slow (every week)/
 (Felbatol),      mg/2,400 to         two to three
 1993             3,600 mg            times daily

Gabapentin       300 mg/1,800        Fast (increase
 (Neurontin),     to 3,600 mg         every day)/three
 1993                                 times daily

Lamotrigine      25 to 50 mg/        Slow (every one
 (Lamictal),      300 to 500 mg       to two weeks)/
 1994                                 twice daily

Topiramate       25 to 50 mg/        Slow (every one
 (Topamax),       200 to 400 mg       to two weeks)/
 1996                                 twice daily

Tiagabine        4 mg/32 to 64 mg    Slow (every four
 (Gabitril),                          weeks)/two to
 1997                                 four times daily

Oxcarbazepine    300 to 600 mg/      Slow (every week)/
 (Trileptal),     600 to 2,400        twice daily
 1999             mg

Levetiracetam    1,000 mg/1,000      Effective at starting
 (Keppra),        to 3,000 mg         dose/twice daily

Zonisamide       100 to 200 mg/      Slow (every one to
 (Zonegran),      400 to 600 mg       two weeks)/every
 2000                                 day to twice daily

Agent            FDA indications

Felbamate        Focal and generalized
 (Felbatol),      epilepsy; adjunct
 1993             and monotherapy

Gabapentin       Focal epilepsy; adjunct

Lamotrigine      Focal epilepsy; adjunct
 (Lamictal),      and monotherapy

Topiramate       Focal and generalized
 (Topamax),       epilepsy; adjunct

Tiagabine        Focal epilepsy; adjunct

Oxcarbazepine    Focal epilepsy; adjunct
 (Trileptal),     and monotherapy

Levetiracetam    Focal epilepsy; adjunct

Zonisamide       Focal epilepsy; adjunct

NOTE: New antiepileptic drugs are, overall, comparable in efficacy
and have nonspecific dose-related side effects (e.g., fatigue,
dizziness). The choice of a given medication depends largely on
other factors, some of which are shown here.

FDA = U.S. Food and Drug Administration.

Information from Tatum WO 4th, Galvez R, Benbadis S, Carrazana E.
New antiepileptic drugs: into the new millennium. Arch Fam Med (In press).

In addition to the newer drugs, several chemical reformulations have been made to the old antiepileptic drugs that have resulted in more favorable usability. Fosphenytoin (Cerebyx) is a reformulated version of phenytoin for use in the treatment of status epilepticus. Long-acting carbamazepine (Tegretol XR) allows for a twice-daily dosing that was not possible with the earlier version of carbamazepine. Oxcarbazepine (Trileptal) is a better-tolerated reformulation of carbamazepine. Intravenous valproate sodium (Depacon) can be useful for replacement in an acute setting or for rapid loading. Finally, rectal diazepam (Diastat) can be self-administered as a "fire extinguisher" by patients who have seizure clusters to abort impending status epilepticus.

In addition to fosphenytoin, other drugs are increasingly being used in the treatment of status epilepticus. In particular, midazolam (Versed) and propofol (Diprivan) may soon become standard therapy because of their very short half-life, which allows rapid titration based on the EEG.

Ketogenic Diet

The ketogenic diet was first advocated in 1921 after it was noted that ketosis and acidosis induced by a high fat-low carbohydrate diet had anticonvulsant effects similar to the effects of starvation. The treatment was rarely used once drugs became available to treat epilepsy. However, there has been a recent resurgence of interest in this treatment modality.

The diet is initiated with starvation until ketones are present in the urine. This therapy should be initiated in a hospital because of the risk of development of hypoglycemia. The diet consists of very large amounts of fat, 1 g per kg per day of protein and minimal amounts of carbohydrates. A typical fat-to-carbohydrate ratio is 4:1 or 3:1. A recent popular modification to the diet is the medium-chain triglyceride variant.

The diet is indicated for use primarily in young children with intractable symptomatic generalized epilepsy of the Lennox-Gastaut type, which is typically associated with diffuse brain abnormalities and some degree of mental retardation. Overall, 30 to 50 percent of children respond favorably. Those who respond show dramatic improvement, with at least a 50 percent reduction in seizure frequency within two to three weeks. The diet is typically maintained for two years. Some evidence suggests that it also may be effective in adults.(17)

While the ketogenic diet does not have the sedative and cognitive effects of antiepileptic drugs, there are some potential concerns regarding its effects on growth in children and on serum cholesterol levels in adults.

Vagus Nerve Stimulation

Vagus nerve stimulation (VNS) is an entirely new treatment modality that has been extensively studied. VNS has an advantage over other electrical stimulations that have been investigated (e.g., cerebellar, thalamic) in that it does not require craniotomy. The mechanism of action is unclear, but it is likely mediated by the widespread afferent connections of the vagal nerve (which terminates in the nucleus of the solitary tract).

The NeuroCybernetic Prosthesis (Cyberonics, Inc., Houston) was labeled by the U.S. Food and Drug Administration in 1997 for adjunct treatment of partial epilepsy. It consists of two components: an electrode attached to the left vagus nerve through an incision in the neck and a generator, similar to a pacemaker, that is surgically implanted in the chest wall. Efficacy is comparable to adjunctive antiepileptic drugs (a mean seizure frequency reduction of 25 to 35 percent and a seizure frequency reduction of at least 50 percent in 40 percent of patients). Furthermore, efficacy may increase over time.(18)

Unlike medications, VNS has no significant neurocognitive or systemic toxicity. The only common side effect is hoarseness of the voice or a mild cough on stimulation. Experience with this new treatment modality is gradually increasing.(19) Like the newer antiepileptic drugs, VNS is being investigated for use in conditions other than epilepsy, and trials for its use in the treatment of depression are ongoing.

Epilepsy Surgery

A National Institute of Health consensus conference on epilepsy surgery estimated that seizures are intractable in approximately 20 percent of patients with epilepsy. Consequently, it is estimated that the number of surgeries performed is well below the number of possible surgeries,(20,21) despite the fact that surgery is now a well-accepted modality for the treatment of medically intractable epilepsy.

Medical intractability is a relative concept rather than an absolute one. The number of antiepileptic drugs that should be tried before a patient is deemed medically intractable is a matter of judgment. However, it is now well documented that when the first drug fails an adequate trial, the chances that another drug will succeed are less than 20 percent. If a second trial fails, the chances of future success with a medication are less than 10 percent.(22) In addition, because of all the new and forthcoming antiepileptic drugs, it is clear that trials of all antiepileptic drugs cannot be required before surgery is considered. Surgery should not be a treatment of last resort that is considered only after exhaustive and futile trials of every available antiepileptic drug. In practice, a usual medical trial may include two to four major drugs, with some used as monotherapy and at maximal tolerated dosages.

When considering surgery as a treatment for epilepsy, a patient's seizures must be frequent enough or severe enough to interfere significantly with quality of life. The risk-benefit analysis for surgery must be individualized, and the benefits must clearly outweigh the potential complications of the procedure. This analysis can be ascertained only through a comprehensive presurgical evaluation.(23,24) This evaluation is multidisciplinary and includes EEG-video monitoring,(8) structural imaging with special magnetic resonance imaging (MRI) using a dedicated epilepsy protocol,(25) and functional neuro-imaging.(26,27) Recent advances in imaging have significantly reduced the need for invasive EEG.

In general, postoperative seizure control is most successful in patients with temporal lobe epilepsy and has a greater than 90 percent rate of excellent outcome when MRI and EEG data are concordant.(28) Surgery for extratemporal epilepsy is often successful when associated with an identifiable structural lesion on imaging, but surgery in patients with nonlesional extratemporal epilepsy is less likely to result in elimination of seizures.(28)

Final Comment

Many new options exist for the treatment of epilepsy. Prolonged EEG-video monitoring is the first step in making an accurate diagnosis. If the first few drug trials fail (keeping in mind that "intractability declares itself early"), other treatment options should be investigated promptly.

In general, any patient who continues to have seizures despite treatment should be referred for further evaluation. If a patient's seizures occur once a week or more often, prolonged EEG-video monitoring may be a valuable method of determining if the patient has epilepsy, if the seizures are partial or generalized and from which cortical region(s) the partial seizures arise. This information will allow the physician to consider all the available treatment options to provide the patient with the best chance of gaining control of seizures. The respective places of various treatment modalities for intractable epilepsy are still evolving.(29)

The authors indicate that they do not have any conflicts of interest. Sources of funding: none reported.


(1.) Benbadis SR, Luders HO. Epileptic syndromes: an underutilized concept [Editorial]. Epilepsia 1996; 37:1029-34.

(2.) Proposal for revised clinical and electroencephalographic classification of epileptic seizures. From the Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia 1981;22:489-501.

(3.) Luders HO, Burgess R, Noachtar S. Expanding the international classification of seizures to provide localization information. Neurology 1993;43: 1650-5.

(4.) Luders H, Acharya J, Baumgartner C, Benbadis S, Bleasel A, Burgess R, et al. Semiological seizure. Epilepsia 1998;39:1006-13.

(5.) Engel J. Classifications of the International League Against Epilepsy: time for reappraisal. Epilepsia 1998;39:1014-7.

(6.) Proposal for revised classification of epilepsies and epileptic syndromes. Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia 1989;30:389-99.

(7.) Lowenstein DH, Bleck T, Macdonald RL. It's time to revise the definition of status epilepticus. Epilepsia 1999;40(1):120-2.

(8.) Benbadis SR. What can EEG-video monitoring do for you and your patients? J Fla Med Assoc 1997; 84:320-2.

(9.) Emedicine Free online medical textbooks for physicians, veterinarians, medical students, physician assistants, nurse practitioners, nurses and the public. Retrieved October 24, 2000, from: http://www.

(10.) Lesser RP. Psychogenic seizures. Neurology 1996; 46:1499-507.

(11.) Liporace J, Tatum W 4th, Morris GL 3d, French J. Clinical utility of sleep-deprived versus computer-assisted ambulatory 16-channel EEG in epilepsy patients: a multi-center study. Epilepsy Res 1998; 32:357-62.

(12.) Benbadis SR, Wyllie E, Bingaman W. Intracranial EEG and localization studies. In: Wyllie E, ed. The treatment of epilepsy: principles and practice. 3d ed. Philadelphia: Lippincott Williams & Wilkins, 2001.

(13.) Bourgeois BF. New antiepileptic drugs. Arch Neurol 1998;55:1181-3.

(14.) Tatum WO 4th, Galvez R, Benbadis S, Carrazana E. New antiepileptic drugs: into the new millennium. Arch Fam Med (In press).

(15.) Gilliam F, Vazquez B, Sackellarea JC, Chang GY, Messenheimer J, Nyberg J, et al. An active-control trial of lamotrigine monotherapy for partial seizures. Neurology 1998;51:1018-25.

(16.) Biton V, Montouris GD, Ritter F, Riviello JJ, Reife R, Lim P, et al. A randomized, placebo-controlled study of topiramate in primary generalized tonic-clonic seizures. Topiramate YTC Study Group. Neurology 1999;52:1330-7.

(17.) Sirven J, Whedon B, Caplan D, Liporace J, Glosser D, O'Dwyer J, et al. The ketogenic diet for intractable epilepsy in adults: preliminary results. Epilepsia 1999;40:1721-6.

(18.) Morris GL 3d, Mueller WM. Long-term treatment with vagus nerve stimulation in patients with refractory epilepsy. The Vagus Nerve Stimulation Study Group E01-E05 Neurology 1999;53:1731-5 [Published erratum appears in Neurology 2000 Apr 25;54:1712].

(19.) Tatum WO 4th, Moore DB, Stecker MM, Baltuch GH, French JA, Ferreira JA, et al. Ventricular asystole during vagus nerve stimulation for epilepsy in humans. Neurology 1999;52:1267-9.

(20.) Engel J Jr, ed. Surgical treatment of the epilepsies. Palm Desert International Conference on the Surgical Treatment of the Epilepsies, 1992. Indian Wells, Calif. 2d ed. New York: Raven, 1993.

(21.) Engel J. Surgery for seizures. N Engl J Med 1996; 334:647-52.

(22.) Kwan P, Brodie MJ. Early identification of refractory epilepsy. N Engl J Med 2000;342:314-9.

(23.) Albright AL, Pollack IF, Adelson PD, eds. Principles and practice of pediatric neurosurgery. New York: Thieme, 1999.

(24.) Benbadis SR. Evaluation for surgical treatment of partial epilepsy: an overview. Wis Med J 1995;94:500-4.

(25.) Bergin PS, Fish DR, Shorvon SD, Oatridge A, deSouza NM, Bydder GM. Magnetic resonance imaging in partial epilepsy: additional abnormalities shown with the fluid attenuated inversion recovery (FLAIR) pulse sequence. J Neurol Neurosurg Psychiatry 1995;58:439-43.

(26.) Tatum WO 4th, Sperling MR, O'Connor MJ, Jacobstein JG. Interictal single-photon emission computed tomography in partial epilepsy. Accuracy in localization and prediction of outcome. J Neuroimaging 1995;5:142-4.

(27.) Benbadis SR, So NK, Antar MA, Barnett GH, Morris HH. The value of PET scan (and MRI and Wada test) in patients with bitemporal epileptiform abnormalities. Arch Neurol 1995;52:1062-8.

(28.) Benbadis SR, Chelune GJ, Stanford L, Vale F. Outcome and complications of epilepsy surgery. In: Wyllie E, ed. The treatment of epilepsy: principles and practice. 3d ed. Philadelphia: Lippincott Williams & Wilkins, 2001

(29.) Benbadis SR, Tatum WO IV, Vale FL. When drugs don't work: an algorithmic approach to intractable epilepsy. Neurology 2000;55:1780-4.

SELIM R. BENBADIS, M.D., is associate professor of neurology in the departments of neurology and neurosurgery at the University of South Florida College of Medicine, Tampa, Fla. He is also director of the Comprehensive Epilepsy Program and the Clinical Neurophysiology Laboratory at Tampa General Health Care, Tampa. Dr. Benbadis received his medical degree from the University of Nice, Nice, France, and completed a neurology residency and a fellowship in epilepsy and sleep disorders at the Cleveland Clinic Foundation, Cleveland, Ohio.

WILLIAM O. TATUM IV, D.O., is clinical associate professor of neurology at the University of South Florida College of Medicine. After graduating from the College of Osteopathic Medicine and Surgery, Des Moines, Ia., he completed a residency in neurology at Loyola University Medical Center, Chicago, and a fellowship in epilepsy at Graduate Hospital, which is associated with the University of Pennsylvania School of Medicine, Philadelphia.

Address correspondence to Selim R. Benbadis, M.D., University of South Florida College of Medicine, 4 Columbia Dr., Ste. 730, Tampa, FL 33606 (e-mail: sbenbadi@ Reprints are not available from the authors.
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