Adrenoleukodystrophy (ALD) Neurology & MRI Information

MRI Monitoring for CCALD

A neurologist is crucial in the care of your son. MRI of the brain is necessary in order to observe any changes to the brain, other than the blood test which confirms the VLCFA which confirms your child has an ALD Diagnosis, MRI will diagnose if a lesion is present and next steps in the treatment of preventing the progression of ALD. MRI is crucial in order to observe if ALD has been triggered. Before the onset of physical symptoms, a lesion can only be identified through MRI. It is extremely important to follow the approved guidelines for how often your son will have an MRI.

What is an MRI scan?

An MRI or Magnetic Resonance Imaging is a medical imaging technique used in radiology to form pictures of the brain. An MRI or Magnetic Resonance Imaging scanner is a machine that generates magnetic fields (the same magnetic field found on fridge magnets! But a lot stronger). The magnetic field “spins” water inside the body, and these spins generate the images of the brain. There is no radiation involved (unlike CAT or CT scans). 

What is a contrast agent, GAD or gadolinium, and what does it have to do with ALD?

A contrast agent is a fluid that is injected into the patient’s IV during the MRI scan. For a short time, it is taken up by different parts of the body, and allows for greater differences to be seen on MRI scans, including inflammation.

Role of Contrast Enhancement

If the contrast agent is also seen in a brain lesion (“contrast-enhancing”), it confirms the presence of an inflammatory Cerebral ALD. If the contrast agent is not seen in the lesion, it indicates that the lesion is inactive. This is very important in deciding to initiate life-saving therapies such as stem cell transplantation. Active, inflammatory Cerebral ALD requires urgent treatment.

Why does my son need multiple MRIs?

In ALD, if a lesion is seen on MRI, the patient is diagnosed with Cerebral ALD. Cerebral ALD occurs most often between the ages of 3 and 12 years old, this is why we need to actively monitor between these ages to make sure changes are not missed.

When and how often does your son need an MRI?

New guidelines have been published by a national work group of ALD experts. Surveillance Recommendations are summarized in the table here:

Table 1: MRI Surveillance Recommendations

AgeMRI FrequencyContrast Administration Recommended?*
1 – 1.5 years oldOnceNo
2 – 2.5 years oldOnceNo
3 – 12 years old
Every 6 months
Yes, unless real time reading is available.
12+ years oldYearlyLesion Positive?  Yes
Lesion Negative? No
*If, at any time a lesion appears, a repeat MRI with Contrast should be repeated 3 months later

Surveillance Guidelines summarized in text form here:

  • The 1st MRI Scan should be performed between 12-18 months old. This time period is variable, to allow the family some flexibility in setting up a schedule. Contrast is not needed at this time point as the likelihood of a brain lesion developing is lower in this age group.
  • The 2nd MRI Scan should be performed 12 months after the first scan. Contrast is not needed at this time point as the likelihood of a brain lesion developing is lower in this age group.
  • From the ages of 3 through 12 an MRI Scan should be performed every 6 months. Contrast should be administered (unless your child is at a center with real-time reading’ capability[1]) as the likelihood of developing a brain lesion is highest in this age group.
  • From the age of 12 and up an MRI Scan should be performed annually.  Contrast is not needed from this time point onwards unless there is evidence of a lesion on a previous MRI.
  • If a lesion is appears on MRI at any time, a repeat MRI should be performed with contrast within 3 months.
  • The MRI’s should ALWAYS be reviewed by an ALD specialist.

Find a list of specialists here.

Real-Time Reading

Some MRI centers have real-time reading capacities. A doctor with a specialization in radiology is present, will look at the scan while the patient is still in the machine, and will administer contrast only if a lesion is present. The purpose of real-time reading is to limit exposure to contrast and sedation time.

Prior to the MRI

  1. Check in with your endocrinologist.  Ask if your child will need stress dose steroids during the procedure.
  2. “MRI Clearance”: Many medical institutions require a physical exam by a pediatrician to “clear” the patient for an MRI. The physical exam is usually submitted to the office within 6 months of the MRI.  It is also helpful to have your pediatrician review your child’s routine metabolic labs (including kidney function: BUN and Creatinine) prior to the MRI.
  3. “NPO prior to MRI”: If your son requires sedation, expect that he will not be able to eat or drink prior to the MRI.  The amount of time is usually 2-6 hours depending on the anesthesiologist’s determination.

Tips from Parents of Boys with ALD:

What to do to make undergoing MRIs easier on you and your son

  1. Practice wearing a play medical face mask at home with your son prior to the MRI so it is not foreign when it comes time for sedation. Making it a game beforehand will allow your child to be much more comfortable when it comes time to wear the mask in the hospital.
  2. There are YouTube videos which replicates the noise of the MRI machine. Look under “MRI Sounds”. It would be useful to listen to this and make it a game with your child so they are not frightened by the noise.
  3. Practice lying still at home prior the MRI.  Again, making it a game and practicing beforehand will make it familiar, and much easier for the boys to stay still during the actual MRI.
  4. For younger children, it may be helpful to wake them up early on the day of the MRI and to keep them awake (no naps) such that they arrive sleepy to the scan.  It will be easier to sedate the patients if needed, or allow them to sleep through the MRI.
  5. If available at your local institution, utilize the “Child Life” staff.  These are staff members specifically hired and trained to make the medical experience for pediatric patients easier and more fun.
  6. Ask if your local institution can play music in the MRI scanner.  Some facilities allow the parents to talk to their children through a microphone as well. Lastly, some facilities have movie goggles. They can play a movie for your son during the scan.
  7. Tips for MRI day from an ALD Mom.

What to expect after the MRI

If your son required sedation, recovery time can range from 30 to 90 minutes.  They will recover (i.e. slowly awaken from sleep) in the MRI suite in a bed under monitoring by a nurse.  Each boy will wake up differently. While transitioning from sleep to fully awake, some patients can be disoriented or cry.  This is transient, is usually not associated with pain, and patients do not remember this transition period.

Quick FAQs

Why does my son need MRI scans?

To routinely detect for cerebral disease, which is potentially life-threatening.

Is there radiation associated with MRI?

There is no radiation from MRI scans.

Will my child have to be sedated in the scanner?

Until the child can lie in the scanner for 20 minutes without moving, he will have to be sedated.

Are there risks from repeated MRI sedations?

Recent published data suggests that repeated exposure to anesthesia (sedations) does not have any adverse effect on IQ later in life.

Are there risks associated with gadolinium (the contrast agent)?

In healthy people, there are no known adverse events associated to receiving gadolinium.  In patients with pre-existing kidney disease/Renal Failure, there is a known risk of developing nephrogenic systemic sclerosis.

(More information from the FDA here:

What happens if he has a lesion?

A repeat MRI should be repeated in 3 months with contrast.  

What happens if I miss or forget an MRI appointment or forget to do a scan?

You should schedule a scan as soon as possible, following the timeline in the MRI guidelines.

What is the most important thing I can do for a child with ALD as a family member?

  1. Make a personal schedule of your doctor appointments and MRI scan dates.
  2. Schedule appointments with your providers ahead of time. In some cities there may be a longer time (months) needed to schedule an appointment, try to do this in advance.
  3. Ask your neurologist to reach out to an ALD expert to discuss your son’s case and MRI scans.