Brain Inflammation

[SongStream]

Last week I completed the MIN-102 trial which concluded with an MRI. The next day I received a call from the neurologist saying there are changes from the last MRI (9/19) and what is brain inflammation. She felt it urgent enough to prompt a video visit with me and my neurologist.

I got the radiologist notes: "When compared with prior MRI dated 10/21/2019, there is interval increase in right periventricular, temporal stem, as well as right hemi pons FLAIR signal, with associated areas of patchy enhancement; and "new FLAIR signal abnormality involving the “genu of splenium of the corpus callosum” and associated patchy enhancement"; and "There is a background of further mild hazy FLAIR signal abnormality within the periventricular white matter", which I gather has an effect on cognition. Researching MRI of cerebral ALD, most notably is the "genu of splenium of the corpus callosum".

So, I conclude I have inflammatory brain demyelination that is indicative of cerebral ALD and luckily, I am presymptomatic. Naturally, my head is heavy with worry and alarm while waiting for the requested MRI to be reviewed by my neurologist who wants to see and examine me in person. (No video appointment.)

I will be 58 in 2 weeks and adult cerebral ALD can occur in your 20's through 50's.

I know that stem cell therapy and bone marrow transplant is an option. But I am weary.

[StevenSims]

Hi,

I’m sorry to hear about this! A few years ago I was told I have a lesion in the splenium of the corpus collosum! I have mri’s every 6 months to keep check as recommended by dr Fatemi at Kennedy Krieger! but it has remained stable! I have no enhancement showing though? Hopefully it’s a more powerful machine? I’m hoping yours will remain stable too! Catching it early is good though and they are getting good with BMT’s now!

All the best

Steve

[SongStream in reply to StevenSims]

It's a 3 Tesla and I had with and without contrast. I appreciate your support. We're our own tribe with this thing. Others don't get it and it leaves you feeling so alone.

[StevenSims]

Hi,

This is the doctors reply:

We reviewed your MRIs, we agree that there is a lesion in the splenium of the corpus callosum which is very typical for the cerebral form of ALD and also agree that a) it has not gotten worse over time and b) there is no gadolinium enhancement. This may indeed may very good news. In boys with ALD this would suggest that disease progression has stopped which we see in 5-10 percent of all cases. In adults we have less data to be that certain and we also don’t know if it may progress again next year, I would continue doing MRIs with contrast every 6 months for the next two years and then annually. The reason it may make sense is that there is a handful of case reports that bone marrow transplantation may help in adults with cerebral ALD if done early, however, it is a very risky procedure and I would not pursue it unless there is clear sign of progression.

Hope this helps,

S. Ali Fatemi, MD

Director, Division of Neurogenetics

Director, Moser Center for Leukodystrophies

Assistant Professor of Neurology & Pediatrics

Kennedy Krieger Institute

Johns Hopkins University

[SongStream in reply to StevenSims]

So glad to hear, Steve! Perhaps I may have overreacted. I've been mildly depressed with the wildfires, pandemic and the state of our nation. My MRI also states "involving the

genu" as well as the "splenium of the corpus callosum with associated patchy enhancement". So, the addition of genu is bothersome.

[fmorgan458 in reply to SongStream]

Over reaction means your brain is doing fine! It's not necessarily the slippery slope of childhood ALD. It has been slow progression until now, and may halt or continue the slow progression. My doctor said just live a few more years. Am now 62. Dr. Eichler at MSG is very experienced with this. Don't give in to fear. We are rooting for you!!!

[SongStream in reply to fmorgan458]

Very comforting to hear! First time in 58 years with this change so that is good.

[artortega78 in reply to StevenSims]

I am 5-10% of boys that have the disease arrested. I was being misdiagonsed with Autism and ADHD when I was younger. I have some lesions on my brain.

[artortega78]

What is your loes score of this year and last year? I am sorry hear about this.

[SongStream in reply to artortega78]

Thanks artortega. Don't know yet but I am requesting the MRIs and will ensure that the experts review them. I realize moreso how meaningful the loes scores are.

[Kevin814 in reply to SongStream]

thanks for sharing this info

[Kranal]

Hello SongStream

Do not worry. I am guessing you are from California, and it is amazing how detailed your MRI result has been, and how informative your neurologist is. My 30 year old son's cerebral adrenoleukodystrophy seems to have arrested, however, it was quite severe right from the start of diagnosis in 2015. NHS Glasgow and Clyde may have proceeded with bone marrow transplant, if his cognition had not been so badly affected. His Loes score was 13 in 2016, and it was too late for stem cell transplant. My 30 year old son's cALD has NOT progressed in the way we had thought it might have, when we read the scientific papers back in 2015, so you rest easy, and do not worry so much. Take care.

Best regards

Kranal

[SongStream in reply to Kranal]

Thank you so much!

[COwithAMN Administrator AMN EASIER Volunteer]

This is a worrying time for you Songstream and all of us here send our warm wishes and support. The possibility of AMN turning cerebral is something we all think about so we can all understand how you feel. But as the earlier postings say, this is not a black and white issue.

Your MRI result is certainly detailed. Trying to understand and digest all the findings is difficult, not least because we are not neurologists but also because the neuoroscience itself proceeds slowly. You seem to have good neurologists advising you but even they are on the cutting edge.

You have probably seen this already but this report from Eichler is worth a scan.

jamanetwork.com/journals/ja...

Stay cheerful and remember we are all there with you.

Chris

[SongStream in reply to COwithAMN]

This is great news! There is much we still don't know but it varies and not conclusive. Thanks for this!

[Hillary]

Hi SongStream, as a bereaved mum, lost my boy to ALD at age 9 and my brother aged 7. I have read lots of papers about this awful disease while trying to understand why my son died and I now have female symptoms. Nothing compares to what you are going through except the fear and loneliness. Know that you have all of us on this forum rooting for you and sending our good wishes, hoping it helps to know that you are far from alone in your fight.

[SongStream in reply to Hillary]

Thank you! I'm so grateful for our "tribe".

[Aaron98]

Wow, Steve, I don't even know what to say. I will try to give you a call today. I'm pulling for you and praying for you, buddy...

[SongStream]

Aaron, I really appreciate your support! Thank you for your call and encouragement!

[Aaron98 in reply to SongStream]

You've got it, Steve! Please keep us in the loop. Please let us know when you are able to send your MRI to the specialists and what they have to say.

[tieaknot]

I’m just seeing this now and wanted to send a virtual hug and lots of prayers for you. You’re not alone...