I Think My Husband Has CLIPPERS

[The Wife]

Hello Bill and Everyone,

My husband Jim is 44 and over Thanksgiving weekend, experienced his second neurological event in the last five years.  In November 2008, he presented with double vision, some numbness mostly on left side in deltoid, fingers, toes, and little less in the right fingers and toes.  He also had some balance issues which we thought were related to the double vision.  MRI showed lesions on the pons.  After the megadose of steroids from Hosptial #1, he started a recovery at home and came crashing down within 10 days or so.  We then went to Hospital #2, where he received megadose again, followed by a long taper at home.  Within six months or so, the MRI showed no lesions, so they ruled ADEM by default.

Thanksgiving weekend 2013 started with a headache that could not be alleviated by anything, followed by slurred speech, some trouble swallowing, trouble walking, and return of the numbness (he had some residual numbness leftover from 2008).  He basically looked like he had a stroke when he presented at Hospital #2.  Many more tests revealed something with the appendix and although probably not related, but who knows, he underwent open surgery to remove his appendix and reconstruct part of his colon that was severely inflamed.  Apparently he was probably suffering from acute appendicitis for who knows how long.  Anyway, they did not start megadose, putting the surgery as priority since his neuro symptoms had started to improve, another MRI showed less lesions, and they would have interfered with the surgery and healing.  Two days post-op, he reported feeling disconnected and weird.  Although they sent someone from neuro to check in on him and they said he was fine, that was not the case.  He was discharged from Hospital #2 mid December and after six days of hell, we were at Hospital #3 looking for help, as by now his surgical incision had opened a bit and it had then been three weeks and his neuro symptoms were never addressed.  He was admitted and not a moment too soon.  By the next morning, his vision split again, like five years ago, and the facial numbness showed up.    

Hospital #3 got all records and images and did their own MRI.  The lesions had increased again and were out beyond the pons.  They started him on megadose Christmas Eve day, our Christmas miracle.  He was discharged with the taper December 30th.  We have had a revolving door of visiting nurse, OT, and PT.  We were looking forward to getting him back to outpatient, neuro based OT and PT and meeting the next neurologist referral this Monday 1/13.  But as I sit here at 2:30 am, I am planning and organizing once again to bring him later today to Hospital #4, as his vision has worsened again in the last two days.  The neuro from Hospital #3 kindly called us last night and thinks it is a good idea to go directly to the Hospital #4 where she gave the referral.  The hardest thing is to watch my husband be so sad and scared with all of this.  But I reminded him and I that five years ago, he had two megadoses and a taper within days of the symptoms starting, so we need to be realistic about how "one and done" was probably not going to work.

They have pretty much ruled out several things at this point, which is why I started looking around for unusual things.  I don't even remember how I stumbled across CLIPPERS as a diagnosis or this blog, but I am glad I did.  Thank you if you have read all the way through this and whatever thoughts you have, I will take.

Jennifer      

[BillCrum]

Hi Jennifer,

Thanks for sharing your story on the forum.

It's very hard to give advice as CLIPPERS is very variable and there isn't a simple test for it. It's worth mentioning CLIPPERS to your doctors if you haven't already - it is still not all that well known  because it is so unusual.

In the US, the people at the Mayo are the experts (Pittock and Keegan) so don't know if you can get your doctors to talk to them to see what they think?

I know that as well as MRI doctors do all sorts of blood and CSF (spinal fluid) tests to rule out other disorders. Some patients also have tests on a tiny sample of brain tissue so they can see what is really happening down a microscope.

In terms of relapses, my understanding is that most (all?) CLIPPERS patients have to stay on some form of medication (either steroids or an alternative "steroid-sparing" agent) to prevent relapses, but again there seems to be a lot of variability in recovery and relapse rate.

I hope this helps, even if only a little.

Best Wishes

-Bill