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Lennox-Gastaut Syndrome (LGS)

What is LGS?

The term “lennox-gastaut syndrome” refers to a group of disorders characterized by abnormalities in the gastrointestinal tract. These include:

Intestinal obstruction

Constipation

Stool retention/discharge (emesis) from the intestines, which may result in abdominal pain or discomfort.

Abnormalities in the secretion of certain hormones (insulin-like growth factor 1 [IGF1]) from the pancreas. This results in an accumulation of urine, toxic metabolites, and abdominal swelling.

The symptoms of lennox-gastaut syndrome generally appear within the first three years of life, but can also become apparent during adolescence. LGS can affect all ages, but is most common among children between the ages of 2 and 6. LGS affects boys twice as often as it does girls.

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Children with lennox-gastaut syndrome experience a range of symptoms, including:

Intense seizures that are difficult to control with anticonvulsant drugs.

Learning disabilities that can affect memory, attention, and problem-solving.

A severe form of autism called regression. This includes a subtype called Autistic Enterocolitis (A).

Growth problems, including a small head circumference (microcephaly), low body weight, and short stature.

Problems with muscle control and movement. This can result in clumsiness or problems with walking, running, or standing still.

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Motor and vocal tics (such as blinking or twitching).

Swallowing difficulties, which can cause food or saliva to collect in the mouth (known as a “frequent cough”).

Several other genetic syndromes (such as Smith-Magenis syndrome and Williams syndrome) have been linked to problems with the growth of the heart and brain.

Many of the symptoms of lennox-gastaut syndrome resemble those of another condition known as Dravet syndrome. This has led some to believe that lennox-gastaut syndrome is a severe form of Dravet syndrome, although it is thought that Dravet and lennox-gastaut represent separate disorders that may have separate causes.

One of the most important factors in diagnosing lennox-gastaut syndrome is ruling out other conditions that may produce similar symptoms. This may require a number of tests (or a combination of tests) including:

Electroencephalogram (EEG). This test measures the electrical activity in your brain.

Magnetic resonance imaging (MRI). This test uses a magnetic field and radio waves to produce images of your brain.

Positron emission tomography (PET) scan. This test shows images of your brain that are produced by small doses of radioactive water.

Lennox-Gastaut syndrome is a severe, lifelong form of epilepsy that can be caused by a number of different factors. It is important to determine the cause of lennox-gastaut syndrome in order to develop a treatment plan that will help prevent or reduce the frequency and intensity of seizures. The causes of lennox-gastaut syndrome are divided into two broad categories: genetic and acquired.

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Genetic factors: There is a strong inherited (genetic) component to lennox-gastaut syndrome. In most cases, it is caused by a mutation in one of at least 16 different genes. Each of these genes provides instructions for making a specific protein that is involved in the transmission of nerve signals within the brain. When these genes have a mutation, the corresponding protein becomes defective. This, in turn, weakens or completely disrupts the normal functioning of nerve cells in the brain.

Most cases of lennox-gastaut syndrome are caused by a new spontaneous mutation. This means that the defective gene appears for the first time in a child following the conception of one of his parents. There is no history of the defective gene in the family.

In other cases, lennox-gastaut syndrome is inherited from a parent. In these cases, the defective gene was present in the family line (that is, it was present in either a parent or grandparent), but it did not cause symptoms in prior generations.

Acquired factors: In fewer cases of lennox-gastaut syndrome, the condition is caused by an acquired factor, such as brain damage or other injuries to the brain. The most common acquired causes of lennox-gastaut syndrome are:

Being born with a very low birth weight.

Severe head injury.

Severe gum disease (periodontitis) affecting the brain.

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Complications from common viruses (such as the chickenpox virus or herpes simplex virus).

Complications from other medical conditions: Some infants with lennox-gastaut syndrome have a small narrowing (stenosis) of a channel carrying spinal fluid (the spinal canal). This spinal stenosis can be caused by a number of different conditions that can affect the spine or brain at a young age, including:

Meningitis (an infection in the covering of the brain and spinal cord).

An tumor (a growth of abnormal tissue) on the brain.

A tumor (often a congenital, or birth, defect) on the spinal cord.

A traumatic head injury.

The cause of lennox-gastaut syndrome in other patients is unknown. In these cases, the condition is called idiopathic.

Learn more about lennox-gastaut syndrome at MedlinePlus Medical Encyclopedia

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Aspergers Syndrome

Asperger syndrome is a type of autism, but not everyone with Asperger syndrome is “on the spectrum.” The condition was first identified by Austrian pediatrician Hans Asperger in 1944, but was not given a name until 1981. Asperger believed that the children he was treating had “little professors,” due to their ability to talk about their favorite subjects in great detail. He also noticed that they had significant difficulties in social situations, and he referred to the children as “little professors” because of their social ineptitude.

Asperger’s work was later overshadowed by the work of fellow Austrian, psychiatrist Leo Kanner, who first described autism in a 1943 paper.

Asperger’s syndrome is now recognized as a distinct developmental disorder in the autistic spectrum.

Most people with Asperger’s syndrome are of average or above average intelligence, but there is usually a significant gap between a person’s ability in a language (such as reading and writing) and his ability in natural languages (the ability to converse with others).

Asperger’s syndrome is defined by a set of symptoms called the “triad of impairments.” The symptoms of Asperger’s syndrome are:

Lack of empathy, failure to develop friendships, and awkwardness in social situations.

Restricted, repetitive patterns of behavior, interests, or activities.

Significant difficulty in initiating or maintaining conversation.

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Unusual language development: delayed language skills or isolated special interests in conversation.

Other symptoms of Asperger’s syndrome may include a preoccupation with a particular subject, high level of intelligence, and clumsy movements.

Although Asperger’s syndrome can be diagnosed in children as young as two or three, the signs of the condition are different at different ages. As a result, some children may be diagnosed later than others.

The cause of Asperger’s syndrome is unknown. Researchers believe that genes play a major role in causing the various forms of autism. They also believe that there may be a link between bowel disease and the syndrome.

The signs of Asperger’s syndrome usually appear within the first few years of a child’s life. Infants with Asperger’s syndrome may:

Not smile back when their parents smile at them.

Not turn their heads towards a person speaking to them.

Not reach out to people or make eye contact with people.

Not babble (say “da-da” or “ga-ga”) by the time they are 12 months old.

Not play “peek-a-boo” by 18 months.

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Not say single words by 16 months and not speak at all by two years old.

Not have a tantrum by the time they are three.

Of course, all children are different, and these symptoms do not apply to all children with Asperger’s syndrome.

Asperger’s syndrome is a lifelong condition. It cannot be cured, but it can be treated to help reduce its effects.

A child with Asperger’s syndrome needs support at home, school, and in the community. He will also need to access special education programs to help him reach his fullest potential in life.

Many people with Asperger’s syndrome go on to live independent lives. They are often very bright and may go on to university or college.

Recent research suggests that early intervention (such as speech and language therapy) can make a difference in the long-term prognosis.

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Asperger’s syndrome is not a mental illness — it is a neurological condition. A person with Asperger’s syndrome may suffer from various emotional problems as a result of the condition. For example, a child may feel very lonely at times, because he finds it difficult to make friends. A teenager or adult with the condition may become severely depressed.

Medical professionals and parents should always be on the look-out for signs of emotional problems in a child or adult with Asperger’s syndrome. If a person with the condition starts to behave in an unusual way (for example, becoming very quiet or very angry for no reason), he should seek assistance from a psychiatrist or other medical professional.

There is a range of support and therapy options for people with Asperger’s syndrome and their families. Most importantly, there is a strong sense of community with other families facing the same issues.

This range of support includes special education programs in schools, adult daycare centers, and recreational programs run by non-profits.

A doctor can run some simple tests to see if a child is developing normally. If a child seems to have trouble with words and emotions, the doctor may refer the child to a psychiatrist or pediatrician who is trained to identify the symptoms of autism and other developmental disorders.

A diagnosis of Asperger’s syndrome can only be made by a professional, such as a psychiatrist or pediatrician. Parents should not attempt to self-diagnose their child.

There is no medical treatment for Asperger’s syndrome, but symptoms can be improved with therapies and medications. Children and adults with the condition should have routine eye tests, hearing tests, dental checkups, regular health checks, and treatments for any illness they may contract.

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Most children with Asperger’s syndrome benefit from special education programs in school. These can include:

Special classes to help a child keep up with their education.

One-on-one support in a classroom.

Access to special equipment (such as computers) to help learning.

Support in learning social skills. A child with Asperger’s syndrome may find it easier to understand physics than the rules of a game. He or she will need help to learn the rules of social games, such as how to negotiate, share, play nicely with others, and understand non-verbal signals.

Parents and teachers of a child with Asperger’s syndrome should always seek therapy and support for the child as well as themselves.

A teenager or adult with Asperger’s syndrome may benefit from:

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Training in life skills, such as cooking, cleaning, and personal management.

Job training or placement. An individual with Asperger’s syndrome may have difficulties in a crowded office, but may excel in a position which involves few social demands, such as working from home.

Anger management courses. Many people with Asperger’s syndrome have limited ways of coping with stressful situations. They may express their feelings in a blunt, insensitive way which may be perceived as aggressive.

A conscious effort to control the tone of voice and other non-verbal communication. A person with Asperger’s syndrome may speak concise, formal English, lacking in intonation and emotional content.

Speech therapy to improve the quality of their voice and intonation.

A service animal (such as a dog) to provide comfort and companionship.

Social skills courses or counseling.

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Self-help support groups.

Some people with Asperger’s syndrome can live and work independently. Often, it is the person’s area of interest which provides motivation to develop and maintain the social and life skills necessary to function in the workplace. For example, a keen astronomer may develop exceptional skill in telescope maintenance, and apply for a job at the local planetarium.

Others may need more extensive support to manage daily life. These people may need to live in a supportive living situation where they have help with personal care and other needs. They may even qualify for government assistance such as Supplemental Security Income (SSI) or Medicaid.

Complications

Asperger’s syndrome is not a life-threatening condition, but the symptoms can cause severe complications in life if left untreated or unsupported.

Children with the condition are at increased risk for:

Being bullied by their peers.

Rejecting social interaction.

Aggressive behavior.

School failure.

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Children with Asperger’s syndrome are also at risk of more minor problems such as:

Speech delay.

Vision or hearing impairment.

Loss of self-esteem.

Adolescents with Asperger’s syndrome are at increased risk for:

Anxiety.

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Depression.

Alcohol or substance abuse.

Cigarette smoking.

Suicidal thoughts or attempts.

Adults with Asperger’s syndrome are at increased risk for:

Sources & references used in this article:

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