Seizure Monitors for Adrenoleukodystrophy

Seizure Monitors for Adrenoleukodystrophy
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Seizures are a common symptom of cerebral adrenoleukodystrophy, especially as the disease progresses. Using seizure monitors can help you manage, and possibly better treat your child’s condition.

What is ALD?

Adrenoleukodystrophy (ALD) is a rare genetic disorder characterized by the progressive degeneration of nerve cells, which carry information to and from the brain. ALD also can affect the adrenal glands, causing them to produce lower levels of hormones than they should.

When the disease affects the brain, as it most often does in its childhood form, doctors refer to it as cerebral ALD.

What are seizures?

Seizures are sudden electrical disturbances in the brain that disrupt its ability to function. They can have an effect on mood, behavior, and consciousness.

Doctors classify seizures based on where in the brain they begin and how much of the brain they affect.

Most seizures last between 30 seconds and two minutes. Those lasting longer than five minutes are a medical emergency.

Seizures in ALD

ALD is caused by mutations in the ABCD1 gene that prevent the ALDP protein from being made correctly. ALDP is a transporter protein that is necessary for the breakdown of saturated very-long-chain fatty acids, known as VLCFAs. When the protein doesn’t work as it should, VLCFAs accumulate inside cells, increasing brain inflammation and damaging nerve cells. This causes disease symptoms, which sometimes include seizures.

What are seizure monitors?

A seizure monitor, sometimes called an alarm, notifies others when a seizure occurs so that assistance can be provided. It can be particularly helpful for children who have seizures during the night.

While a monitor cannot accurately detect all seizures, it can provide caregivers some peace of mind.

Types of seizure monitors

There are different kinds of monitors. Consult with your child’s primary physician when choosing one, and learn as much as you can about them.

Some monitors or alert devices involve wearable technologysmart electronic devices that are worn like a watch. These devices respond to repeated shaking movements, which may indicate the wearer is having a seizure. In the event it detects abnormal activity, the monitor usually connects with a smartphone to send alerts.

Mattress devices are usually placed under a mattress and can detect vibrations. An alarm will sound if the device detects seizure-like movements.

Camera devices record audio and video information from a remote infrared video camera. They send the information to a smartphone and an application records and analyzes the video for seizure-like activity. When it detects an unusual event, an alarm sounds, followed by live sound and video.

Motion detection devices monitor body movements, such as intense shaking in the arm muscles, to detect activity that could be a seizure. Some devices also may record sounds and send alerts to caregivers.

Questions to ask

Some devices can not detect all types of seizures, especially those that don’t involve big, repeated movements. They also may not be affordable for some.

Some questions to ask about individual products include:

  • Which seizure type or types does the monitor pick up?
  • Is it suitable for children?
  • How many or what percentage of seizures is the monitor likely to pick up?
  • How often does the monitor give out a false alarm?
  • Will the monitor get in the way of my child’s day-to-day living or sleeping?
  • What are the potential issues I may experience with the monitor?

Last updated: Sept. 2, 2020

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Adrenoleukodystrophy News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

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Özge has a MSc. in Molecular Genetics from the University of Leicester and a PhD in Developmental Biology from Queen Mary University of London. She worked as a Post-doctoral Research Associate at the University of Leicester for six years in the field of Behavioural Neurology before moving into science communication. She worked as the Research Communication Officer at a London based charity for almost two years.
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