CLIPPERS Without Steroids?

One key thing that characterises CLIPPERS is that it responds to steroids in the first instance. So characteristic is this that "Responds to Steroids" is the "RS" in CLIPPERS. Everyone who is treated for CLIPPERS, at some point or another, receives steroid treatment, which for many patients means high-doses of intra-venous Prednisolone.

 

A recent case study (but only a single case) hints that other treatment paths may be possible. I must stress that a single case in a condition that is hard to diagnose like CLIPPERS can't be definitive, but this is interesting none-the-less.

 

This patient had diagnoses first of Multiple Sclerosis and subsequently of neurosarcardosis (both conditions that were considered in my case also). Treatment was with hydroxychloroquine which is an anti-inflammatory used in the treatment of neurosarcoidosis. (Interestingly, the FAQ here, suggests that Prednisolone is the drug of choice for neurosarcoidosis, showing once again that there are many overlapping conditions which can be treated somewhat similarly.) The patient had good resolution of symptoms however, after their symptoms, tests and records were re-examined, the diagnosis was changed to CLIPPERS; the patient has remained well on maintenance doses of hydroxychloroquine since. Also interestingly, Dr Pittock at the Mayo Clinic, had previously tried hydroxychloroquine in one CLIPPERS patient but did not have as good results as in this case study.

 

The authors suggest hydroxychloroquine be considered as an alternative treatment for CLIPPERS because it is well-tolerated by patients, although it is not without side-effects. It will be interesting to see if this study affects treatment decisions in any future patients and whether the good results gained here can be reproduced.

 

Read other articles in this series at Living With CLIPPERS.

Living With CLIPPERS by Bill Crum is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.

CLIPPERS 2015

A "Happy" meal I had over Christmas

A belated Happy New Year to everyone out there. January 2015 marks the 3-year "anniversary" of when my CLIPPERS symptoms retreated far enough for me to function more or less normally. At the time I was still on tapering Prednisolone and attempting to ramp up Azathioprine while dealing with a few wobbles in various blood tests along the way. I remember book-keeping the changing doses of various drugs needed a certain amount of organisation. There were worries about long-term prospects in 2012 (there still are!) but I have been lucky to be stable on Azathioprine for some time now. 

Although Azathioprine is a common CLIPPERS treatment in conjunction with steroids, I don't recall any cases being reported who have remained stable for this long after the complete removal of steroids. It would be interesting to know if anyone out there is being treated similarly. Am I simply lucky, an anomaly or living on borrowed time? I still occasionally try walking "heel-to-toe", standing on one leg with/without eyes shut etc to try and pick up any early signs of recurring problems. It will be interesting if, as part of their CLIPPERS study, the Mayo team have any comments on my case compared with others.

Finally, a reminder that for those interested in events which raise the profile of rare conditions, the annual Rare Disease Day is coming in February (28th to be precise).

Read other articles in this series at Living With CLIPPERS.

Living With CLIPPERS by Bill Crum is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.

Another Year

I take no credit for this - my wife's creation.

Just a quick note to wish everyone a peaceful and happy end to 2014.  My own Christmas break started off with an invoice from my local health centre. They had received a request for my medical records from the Mayo Clinic as I had signed up for the Mayo research study. It hadn't occurred to me there would be a charge for this - after all it is making a contribution to medical research. Anyway, although £24 won't bankrupt me, it wasn't quite the Christmas card I was expecting when I opened the letter!

I will be back in 2015 to pass on anything I can find out about CLIPPERS. If anyone has any news to share please contact me or post in the forum.

Best Wishes

-Bill

Probable CLIPPERS since 2011.

P.S. After writing this post, I received very nice emails from Jessica Sagen and Dr Tobin at the Mayo, telling me that costs incurred providing records for their research will be covered from their research budget. So thanks very much to them for that.

Read other articles in this series at Living With CLIPPERS.

Living With CLIPPERS by Bill Crum is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.

Nothing to see here (probably)

A birthday card I saw
  recently - seriously!

It's all been a bit quiet on the CLIPPERS front lately, so apologies for the slightly sporadic updates at the moment. Another one of these complicated single-case studies has appeared though, so I'll give you a quick run-down. The essential feature of this 62-year old woman's story is that she was diagnosed with CLIPPERS after an episode of shingles which was concentrated on her left ear, eye and face. There is quite a lot of detail about different treatments and symptoms but essentially, when CLIPPERS symptoms first appeared, a left-sided brain lesion was seen on MRI which later developed into a more classical CLIPPERS pattern of lesions towards the back of the brain. This is an interesting case, but as is quit common at the moment, one that is so unusual it is hard to draw too many firm conclusions. I picked up on a couple of things in the paper though.

Firstly the authors speculate that (my paraphrasing) shingles and CLIPPERS in this case are related due to the "time-course" and "laterality". So the time-course means that one follows the other; but just because  the bus arrives after I stand at the bus-stop doesn't mean that I caused the bus to arrive by standing there - correlation versus causation. Secondly, "laterality" means "same-side"; so here, the shingles manifested on the same side as the subsequent brain-lesions. However, for the sake of argument consider that the shingles were completely unrelated to CLIPPERS and were a random occurence (the so-called "null-hypothesis"). Then there is a 50% chance they would manifest on the same side as the initial CLIPPERS lesion even if they had nothing to do with CLIPPERS. Of course, if we see more cases like this then the arguments for time-course and laterality strengthen.

To be fair the authors do go on to say that they "do not believe that the CLIPPERS syndrome is directly caused by the ... viral infection" and flag well the parts of their discussion which are speculation. I hope these cases keep being reported as my impression is that the diversity of CLIPPERS cases appearing over the last couple of years suggests that it could be part of a much larger puzzle concerning a variety of more common brain conditions.

It will be interesting to see if any more cases involving shingles appear. I had facial shingles in 2014 (also on the left-side) after developing CLIPPERS in 2011. In my case, shingles was almost certainly a consequence of immune-suppression from CLIPPERS treatment and being generally run-down. I am pleased to report, that in my case at least, shingles has not caused any resurgence in CLIPPERS symptoms.

Read other articles in this series at Living With CLIPPERS.

Living With CLIPPERS by Bill Crum is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.