Epilepsy
More than 80% of children with Angelman syndrome have epilepsy with onset
typically in the first three years of life. They frequently have mixed seizure disorders
ranging from as mild as occasional absence seizures to hundreds of drop attacks per
day. Seizures can be life threatening and correct medication is vital in order to control
them. Seizures can interfere with learning and can also be linked to certain unusual
behaviours.
Most (not all) children with Angelman Syndrome have abnormal EEGs
(electroencephalograms) and many are prone to significant seizure activity that is often
tricky to control in childhood. They usually demonstrate an abnormal EEG with a
characteristic pattern of large amplitude slow-spike waves. (Image here shows
Characteristic Angelman EEG, with Triphasic delta wave activity arrowed.)
More info on Angelman syndrome and Epilepsy:
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From the Epilepsy Foundation - read here
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Seizure treatment in Angelman syndrome (Download the pdf here ) - a recent case series from the Angelman Syndrome Clinic at Massachusetts General Hospital Elias A. Shaaya, Olivia R. Grocott, Olivia Laing, Ronald L. Thibert
New YouTube videos:
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2017 ASF Family Conference: Seizures in Angelman Syndrome - by Ronald Thibert, DO, MsPH
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What are the alternative treatments for Epilepsy? - AngelmanUK
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Scientists in Singapore discover mechanism causing epilepsy in Angelman syndrome (Jan 16, 2020)
What can you do?
If your child has seizures, it is helpful for the child, the parent, other carers/aides, and the medical
professionals involved, if you have a consistent plan of action.
Seizure Response Plan/Epilepsy Management Plan
Why is it important to have a Seizure Response Plan?.
A seizure response plan is a written document that provides general medical
information about the person with epilepsy (including personal identifying
information), emergency contacts, details about his or her specific seizures,
medicines, and information on what to do if a seizure happens. Your child's
GP or neurologist can help you fill this out. There are several samples of these
plans on the internet.
Download a template plan here, and/or here.
If your child has epilepsy, this seizure response plan should be on file with
teachers and the school nurse’s office (or wherever your child spends time,
such as an aftercare program, camp, or with a babysitter. Having a plan readily
available will help minimize the impact of seizures for the person with epilepsy,
and help others to manage seizures appropriately.
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The family plays a central role in your child's seizure management.
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Your success in managing your child's seizures/epilepsy will depend
on being prepared to tackle whatever comes your way – from understanding
epilepsy and maintaining seizure control, to responding to seizures and
managing safety.
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A Seizure Response Plan can help you organize your child's seizure
information and have it available when and where you need it. It can help
you know what to do to prevent an emergency, or tell others what to do in
emergency situations. You can also adapt these plans to different situations
in your child's life.
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By helping you be prepared, seizures (or the fear of seizures) won’t prevent
you from letting your child participate and enjoy their life to the fullest.
Support groups on Facebook
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Angelman Syndrome Seizure Information group – for sharing information
around seizures, AEDs, CBD oil, diet management, etc.
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Group for dealing with tremors: Shaking Angels
What are tremors?
Myoclonus describes a symptom and not a diagnosis of a disease. It refers to
sudden, involuntary jerking of a muscle or group of muscles. Read more here:
Myoclonus. Epileptic myoclonus occurs as part of an underlying epileptic disorder.
Subtypes of epilepsy-induced myoclonus include cortical reflex myoclonus,
progressive myoclonus epilepsy, and reticular reflex myoclonus. Each subtype
refers to a form of epilepsy that affects different brain regions. These tremors are
not the same as seizures and can be extremely difficult to treat.
Dysautonomia can also present as tremors. It is an umbrella medical term utilized
for a group of complex conditions that are caused by a dysfunction of the
autonomic nervous. Read more on this lesser known condition, here.
Informative Articles
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Analysis of EEG patterns and genotypes in patients with Angelman syndrome
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List of anti epileptic drugs - Anti-epileptic drugs (AEDs) are the main form of
treatment for people with epilepsy.
For more support and in-depth information on Epilepsy, see Epilepsy New Zealand
Vitamin Supplements
Vitamin D: Some anti-epileptic drugs (AEDs) can reduce bone density, making bones weaker and more likely to break. To help prevent this, doctors may suggest a bone density scan, and prescribe a vitamin D supplement. Vitamin D helps the body to absorb calcium and build healthy bones. Sunlight is the main source of vitamin D, and most people can store enough to last through the days when there is little sunlight. Vitamin D is also in eggs, oily fish, fish liver oils and foods with added vitamin D, such as some cereals, fat spreads and dairy products.
Vitamin B6 : Side effects of taking Keppra include changes in mood or behavior, clumsiness or unsteadiness, or unusual tiredness or weakness. More serious symptoms can include depression and hallucinations. A study reported in "Clinical Epilepsy" in October 2005 found that the addition of vitamin B6 may prevent or reduce some of the psychiatric symptoms associated with Keppra.
CBD oil - cannabidiol
Does medical cannabis help seizures? Evidence from laboratory studies, anecdotal reports, and small clinical studies from a number of years ago suggest that cannabidiol, a non-psychoactive compound of cannabis, could potentially be helpful in controlling seizures. More testing needs to be done to ascertain safety levels. CBD does interact with some other seizure medicines, so this must also be taken into consideration. CBD should not be taken casually as an extra supplement.
Watch new Youtube video:
Should we use Cannabis to treat Epilepsy? - AngelmanUK.
Read more on Medical marijuana and epilepsy (Epilepsy Foundation).
1. Log Book
Keep a detailed log to record unusual behaviors or possible seizure activity. A sample template for a seizure log/journal can be found here.
This can be a helpful tool to identify triggers and seizure types, should further investigations be needed.
2. Take video clips
Video clips of your child that record unusual behaviors or possible seizure activity is useful information for a specialist when trying to identify what type of seizures your child is having.
3. Seizure Types
Different seizure types require different treatments and medication. Get to know Seizure Types, here. Share questions and concerns with other parents on FB here: Angelman Syndrome Seizure Information
4. Download and print:
Have these on display/on file for your Seizure Response Plan for carers, support workers, teachers, aides, etc. More info here at International Epilepsy Day
RESEARCH on Epilepsy in AS
Due to the high rate of seizures among people who have AS, there is ongoing research on Epilepsy and Angelman Syndrome. Some research articles are listed here:
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largest study to date examining epilepsy and its treatments in AS.
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Can genetic therapy help kids with Angelman syndrome overcome seizures? (2018)
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The effects of herbal medicine on epilepsy (2017) - Wei Liu, Tongtong Ge, Zhenxiang Pan, Yashu Leng, Jiayin Lv and Bingjin Li
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'Dietary Treatment Proven Successful in Reducing Seizures in Angelman Syndrome by up to 90 Percent ' - Dr. Ron Thibert on the LGIT diet (2018)
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Scientists in Singapore discover mechanism causing epilepsy in Angelman syndrome (Youtube)