Progressive encephalomyelitis with rigidity and myoclonus (PERM): Help

@ellenanne33 This must be so difficult for you and your family. Is your husband currently in hospital or home?
Is he followed in a specialty clinic or by special physicians? They would have access to the most up-to-date information.

Can you tell me what PERM stands for? I’m unfamiliar with the term so it’s difficult to give you better information.
This is one site I have found for help. Have you contacted the ones like this?
https://www.911healthwatch.org/where-to-get-help/
Will you stay with us? I’ll see if one of the other mentors know more

My husband is home .receives his ivig treatments at home every 4 weeks ..has his rituxamab treatments set for oct 2nd round at columbia presbyterian in nyc .yes we have an autimmune specialist from columbia w have been seeing .Perm is progressive encephyelomyetis with rigidiy monoclonous ..there really isnt any literature on this disease which is so frustrating .on same spectrun as stiff persons but more rare and complex ..just want to make sure we are doing everything right as far as his treatments and if we should be doing more or going to a doctor if we can find one that specializes in this disease. Very frustrating that the FDNY is not recognizing autoimmune diseases qhen he was a healthy 60 at the time of the start of his symptoms and actually thought he was improving any help i sure would appreciate .sincerely Ellen ODonnell

Hi @ellenanne33, I would like to add my welcome to Connect along with Becky @becsbuddy. I also was not familiar with the condition but did find the following information.

"Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare and severe neurological syndrome characterized by muscular rigidity and spasms as well as brain stem and autonomic dysfunction. It can be associated with anti-GAD, GlyR, and DPPX antibodies."
-- Progressive Encephalomyelitis with Rigidity and Myoclonus Associated With Anti-GlyR Antibodies and Hodgkin’s Lymphoma: A Case Report: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5554358/

I also found this article on treatment that you may not have seen.
-- Progressive Encephalomyelitis with Rigidity and Myoclonus Treated with Rituximab: Case Report and Review of the Literature: https://repository.library.brown.edu/studio/item/bdr:tg25ev5w/

I think there are a lot of conditions that are extremely difficult to determine what the cause is and even more so when it's rare like in your husband's case. Here's a couple of articles that discuss the health effects that are a result of 9/11
-- Health Effects of 9/11 Still Plague Responders and Survivors: https://www.scientificamerican.com/article/health-effects-of-9-11-still-plague-responders-and-survivors/
-- Implications of the World Trade Center Attack for the Public Health and Health Care Infrastructures: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1447752/

Have you tried talking with any of your local county or state representatives to see if they have any contacts that might be helpful?

Thank you all !! Just very frustrating ...yes i have reached out to representatives and they told me to touch base with niosh.gov which i did .it just doesnt make sense that they can be so certain that this disease has nothing to do w 9/11 when they dont knoq much about this disease ....i find it hard to believe when they see autoimmune diseases on the rise ...just feel helpless !!

Somewhat related to the 3rd week waning effect of the gamma globolin

Suggestion for the waning effect of the gamma globulin IV infusion. I am self administering Hizentra gamma globulin infusions weekly for a different autoimmune disease subcutaneously with a hand held pump. The infusion takes a couple of hours, is easy, and maintains a constant blood level of IgG.
This subcu self administering was MD recommended to maintain levels from week to week.

;

@ellenanne33 - I’m so sorry to hear what a difficult illness your husband has to deal with.
I lived in NY 9/11 and remember everything from that time.
I am with you believing that your husband’s condition is due to his exposure.
Since his condition is so rare, I doubt that anyone is going to confirm the connection.
As we know, many illnesses may take years to appear.
I agree with you that you want to know that everything possible is done for him.
Columbia Presbyterian is an excellent institution- you are lucky that way.
However , everyone has the right to a second opinion. You may ask his current physician to recommend where you can get a second opinion.
There is always Mayo Clinic or Cleveland Clinic.
Please thank your husband for all the work he did at the site- I can’t even imagine what it must have been like.

I appreciate that ...and thank you for taking the time to read ..God Bless !!

He is home .He receives his ivig treatments at home now every 3 weeks ,was every 4 weeks..Perm is on same spectrum as stiff persons but rarer .Progressive encephelomyetis qith rigiduty monoclonous ..he had plasmapharesis and ivig treatment in hospital after brain surgery then had 2 rounds of rituxamab and has his 2nd round coming up in oct ..any help please

Amy husband larry is currently in hospital 6 weeks now in critical condition w what they are saying is perm .apparently ivig ,rituxan and plasmapharesis is not working anymore they now want to to try cytoxin .does anyone know the pros and cons of this medication will it help him or prolong his horrible existence .no quality of life i am seeing if anyone has any info if this has helped ? They are saying now that he has a rarer form.of perm effecting his brainstem ..help please so i can make a descion if we should try new meds

Progressive enecpelomyetis w rigidty monoclonous also believe affecting his brain stem. Currently in columbia presbyterian nyc .This is the hospital we have been using since 2021