Tag Archives: Seizure

Angleman Syndrome and Adulthood

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Angelman syndrome is a genetic disorder that affects the nervous system. Characteristics include developmental delays, intellectual disabilities, epilepsy, short attention span, hyperactivity, hand flapping and speech impairments. It is a rare disorder that affects 1 in 12,000 to 20,000 a year. there are less than 200,000 cases a year and affects all ethnicities and sexes equally.

Typically, there is a severe developmental delay and learning disability as well as near absence of speech and ataxia including ataxia including jerky movements of the arms and legs. People with Angelman syndrome generally have a happy demeanor and an inappropriate laughter.

Life expectancy appears to be normal. As people with Angelman syndrome age, scoliosis tends to worsen and for many people born with epilepsy, there are cases where seizures stop, however, they tend to return as the person gets older.

 

Due to cognitive and medical needs, a person with Angelman syndrome will always require support. Most people with Angelman syndrome attend a day habilitation setting. Support should include

  • staff trained on identifying seizures and providing assistance
  • programs that support the person’s short attention span
  • evaluation for a communication device or a means to communicate
  • support for self-injurious behavior

The following are resources and articles on Angelman syndrome and adulthood

Angelman Syndrome and Obesity (Angelman Syndrome) A 2017 study reporting on a third of Angelman syndrome patients were overweight or obese.

Angelman Syndrome in Adults– (Angelman Syndrome News). An article that describes signs and symptoms of individuals with Angelman Syndrome as they approach adulthood

Anxiety in Angelman Syndrome (Angelman Syndrome News). A topic on anxiety which is commonly observed in individuals with Angelman syndrome including information on triggers and ways to manage anxiety.

AS in Adulthood (AngelmanUKorg). A website describing the transition to adulthood and issues affecting people with Angelman syndrome.

Dental Problems in Angelman Syndrome (Angelman Syndrome News). Information on how people with Angelman Syndrome may be affected and possible treatment plans.

Transitioning to Adulthood Checklist. Created by the Angelman Syndrome Foundation, provides a 4-page checklist resource for students preparing to transition out of school.

Cerebral Palsy and Secondary Issues

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Cerebral palsy affects people differently including; learning disabilities, intellectual disabilities, behavioral challenges, Attention Deficit Hyperactivity Disorder (ADHD), and Executive Function Disorder. People with cerebral palsy often have medical issues as well including, epilepsy, hydrocephalus, swallowing difficulties, vision problems, aspiration and constipation.

Seizures
  • a seizure is a sudden, out of control event that can cause involuntary movement.
  • It occurs when there are bursts of abnormal electrical activity in the brain which interferes with normal brain functioning.
  • The brain constantly sends unusual electrical messages very close together.
  • Inform the participants that seizures are the most common of associated problems.
  • Experts believe that seizures are the result of scarred tissue in the brain.
  • About 50% of people with cerebral palsy suffer from seizures.
  • Epilepsy is more common in the child with spastic quadriplegic
  • Complex partial seizures are the most common in people with cerebral palsy
  • in most cases, it is unknown the cause of seizures.

Cases which the cause is known include

  • Serious brain injury
  • Lack of oxygen
  • Bleeding in the brain
  • Infection in the brain including meningitis and encephalitis
  • Inflammation of the brain
  • Co-occurring conditions that involve an intellectual or developmental disabilities

Treating Seizures

  • Try to keep calm and make sure the person having the seizure is comfortable and safe from harm.
  • A seizure can last from a second or several minutes

Do Not:

  • Do not hold the person down or try to stop his or her movements.
  • Do not put anything in the person’s mouth. This can injure teeth or the jaw. A person having a seizure cannot swallow his or her tongue.
  • Do not try to give mouth-to-mouth breaths (CPR). People usually start breathing again on their own after a seizure.
  • Do not offer the person water or food until he or she is fully alert.

After the seizure:

  • After the seizure ends, the person will probably be groggy and tired. He or she also may have a headache and be confused or embarrassed. Try to help the person find a place to rest.
Vision Issues
  • The term blindness refers to complete impairment of vision
  • Visual impairment refers to diminished vison or low vision but not total blindness.
  • A person’s right side may include problems with muscles that control the right eye making it difficult for the affected eye to move from side to side.
  • People may appear to be crossed eyes.
  • Explain that people with cerebral palsy may have a condition called strabismus, where eyes do not line up and focus properly because of differences between the left and right eye muscles
  • May only impact one eye which happens to those whose cerebral palsy affect one side of the body.
Learning Disability
  • People with cerebral palsy may have a short attention span, motor difficulties, perceptual difficulties and language difficulties
  • This can impact literacy, numeracy and other important skills.
  • Learning disabilities may also affect fine and gross motor coordination
  • They may tire easily since they have to put more effort into concentrating on their movements and sequence of actions.
ADHD
  • A disorder of the executive function of the brain that allows a person to focus and organize
  • It is a developmental disability that occurs in approximately 3 to 5% of children
  • 19% of children with cerebral palsy will be diagnosed with ADHD
Hydrocephalus 
  • Enlargement of the fluid filled spaces in and around the brain known as ventricles
  • To correct the damaging effects, the fluid build-up is performed involving a shunt
Behavior Challenges
  • Children and adults with cerebral palsy may be more prone to having problems controlling their impulses as well as having difficulty with focus and attention
Hearing Loss
  • Birth injuries can cause partial or complete loss of vision and or hearing in many individuals
  • Possible result of physical damage to the ear due to inflammation of built-up
  • Senorineural hearing loss- nerves that transmit sound information from the outside world to the brain are damaged
Tube Feeding
  • Gastrostomy is used with children with significant eating, drinking and swallowing difficulties to ensure they received enough food and drink.
  • It is surgically placed through the abdominal wall to feed formula, liquids and medication directly to the stomach.
Intellectual Disability
  • Approximately 2/3 of people with cerebral palsy have an intellectual disability
  • 1/3 have Mild
  • 1/3- Moderate
  • 1/3- Normal IQ
  • Children with spastic quadriplegia are more likely to have an intellectual disability

Early Signs of Rett Syndrome

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Rett syndrome is a neurodevelopmental disorder occurring mostly in females in which the child exhibits reduced muscle tone, autistic-like behaviors, stereotyped hand movements consisting of wringing and waving. It is a rare disorder that affects about 1 out of 10,000 children and is the most genetic causes of severe intellectual disability in females. Due to similarities to autism and cerebral palsy, it is often misdiagnosed.

Signs similar to autism include outburst, eye contact avoidance, lack of social reciprocity, sensory issues and loss of speech. Signs of Rett syndrome similar to cerebral palsy include short stature, difficulty with walking, gait movement difficulties and delayed ability to walk.

Typically, children born with Rett syndrome develop normally until the age of 6 and 18 months and reach typical development milestones including eye contact, rolling over and grasping objects. As children grow, motor development begins to slow such as walking and muscle movement. The first signs is usually the loss of muscle tone where the child’s arms and legs appear “floppy.”

Other early signs include:

  • Loss of communication abilities
  • Unusual eye movement
  • Breathing problems
  • Irritable and crying often
  • Intellectual disability
  • Seizures
  • Scoliosis
  • Sleep disturbance
  • Irregular heartbeat
More Resources on Rett Syndrome

Over 30 Online Resources on Rett Syndrome

 

Intellectual Disabilities And Epilepsy

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According to the Epilepsy Foundation, epilepsy is the fourth most common neurological disorder and affects people of all ages. In fact, 1 in 26 people have seizures and while people who are diagnosed with epilepsy may have no other problems, this is not the case for children and adults with an intellectual and developmental disability where the rates are much higher.

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It is estimated that 30% of children with epilepsy also have another type of disability. Some studies show that children with an intellectual disability and cerebral palsy, had a 35% chance of developing epilepsy, children with an intellectual disability alone had an 8% chance and children with a brain injury occurring after birth stood a 75% chance of developing epilepsy. Statistically, the risky of a child with a developmental disability experiencing an unprovoked seizure by age 5 is 4 times likely than the general population. It is estimated 1.8% of U.S. adults have epilepsy compared to 22% among people with intellectual disabilities.

People with intellectual disabilities tend to also have worse prognosis with adults having a higher rate of death including Sudden Unexplained Death In Epilepsy (SUDEP).

Epilepsy

Epilepsy is a chronic disorder with recurrent unprovoked seizures. According tot he National Institute of Neurological Disorders and Stroke (NINDS), epilepsys are a spectrum of brain disorders ranging from severe, life-threatening to ones that are more benign. The International League Against Epilepsy created a new definition of epilepsy. A person is considered to have epilepsy if they meet any of the following conditions:

  1. At least two provoked (or reflex) seizures occurring greater than 24 hours apart.
  2. One Unprovoked (or reflex) seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occurring over the next 10 years.
  3. Diagnosis of an epilepsy syndrome

A seizure is defined as an event and epilepsy is the disease involving recurrent unprovoked seizures.

Intellectual Disability

According to DSM-5, an intellectual disability is a disorder with onset during the developmental period that includes both intellectual and adaptive functioning deficits in conceptual social, and practical domains including deficits in intellectual functions such as reasoning, problem-solving, planning, abstract thinking, judgment, academic learning and learning from experiences.  The severity levels range from mild to profound.

Although there is a high relationship between epilepsy and an intellectual disability, little research has been conducted on safe prescribing practices of an antiepileptic drugs for people with intellectual disabilities. What is known about epilepsy and an intellectual disability is that more severe disabilities are typically caused by damage to the brain

Why is it important to discuss?

Given the large number of people with an intellectual disability and epilepsy, treatment may be more complicated due to multiple impairments including people with a diagnosis of autism, intellectual disability and epilepsy. More research needs to occur which will help to reduce morbidity rates and help to develop accurate guidelines.