CLIPPERS or SLIPPERS?

https://commons.wikimedia.org/wiki/File:Illu_tentorium.jpg

I was recently contacted by someone with a close relative who had been diagnosed with possible "SLIPPERS" syndrome. I initially suspected a typo but then realised that this was a CLIPPERS variant which had passed me by. Initially proposed by Dr Armand in 2015 this condition differs from CLIPPERS in which part of the brain is predominantly affected. In CLIPPERS the enhancing lesions seen in MRI are concentrated below the Tentorium (see image above). In SLIPPERS (Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids) the lesions are concentrated above the Tentorium. The two patients in the original case-study both responded to treatment similarly to CLIPPERS but had presented with seizures and headaches respectively which I think are less common in CLIPPERS. Another patient was subsequently reported by Dr Horng in 2017 who also responded to similar treatment. It's unclear to me whether this is really a distinct syndrome or just a variant of CLIPPERS but at least the treatment response is very similar so the difference for treatment purposes is not crucial (it would seem - I'm not  a doctor).

In other news my medication (Azathioprine) is mysteriously unobtainable in my locale currently for reasons unknown to me. Is it a manufacturing problem, supply problem, hoarding problem? I don't know but it's been suggested I ask my doctor for an alternative - may be easier said than done as, having been stable for quite a few years, changing meds at this stage is not on my agenda. There was a shortage a few years ago which resolved after a few weeks so hopefully the same thing will happen again.

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.

2019 Update

I'm acutely aware that the interval between posts on this blog is steadily lengthening. The reason for this is simple - CLIPPERS simply isn't affecting my day-to-day life much. My current schedule is: take medication twice a day, renew prescription every two months, get blood test every 3 months, see consultant every 12 months ... and that's about it. In other regards I live normally apart from being a bit careful going down stairs. Of course it may not always be like this. I may eventually have some malign consequence of being on these drugs for so long. This could in itself be serious or force me to switch medication. I could have some unrelated health problem which has the same effect. And then, all bets are off.

In parallel, as far as I can tell, researchers are still working on CLIPPERS, presenting new exotic case-studies and gradually gaining some more understanding of what it does. One recent letter succinctly sums up the current state of affairs for diagnosis of new patients and things haven't changed much: "its lack of specific lab findings, poorly understood pathogenesis, and variable symptoms, making it a clinical and radiological diagnosis of exclusion." If you are diagnosed with CLIPPERS you have probably had the following conditions excluded first "neurosarcoidosis, central nervous system Behçet's disease, vasculitis, central nervous system lymphoma, lymphomatoid granulomatosis, Bickerstaff brainstem encephalitis, chronic perivascular infectious process, glioma, central nervous system demyelinating disease, and Langerhans cell histiocytosis"

On the other hand, CLIPPERS is now sufficiently recognised that a recent paper examined the diagnostic costs involved for two patients who had differing numbers of tests but the same outcome in terms of treatment. The cost was approximately $176,000 versus $12,000. Given the diagnostic problems discussed above this seems to me quite a dangerous publication. The authors do comment on reducing invasive testing being good for patients - I agree - and following criterion reccommended by Dr Tobin, but the emphasis is on cost comparison even in the title. Skimping on diagnostic work-up when the consequences of mis-diagnosis are so huge and there is no definitive test for CLIPPERS  is foolhardy and I hope cost doesn't become an issue for proper diagnosis.

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.

Autumn already

A red pineapple plant.

Doesn't time fly? It's been six months since my last proper update and since then a very hot summer (by UK standards, 30C+) has been and gone and Brexit looms nearer. It's been hard to add interesting content to this blog recently. There are still new research papers on CLIPPERS appearing and I add them to the CLIPPERS Research Papers page when I get a chance. However, the majority seem to be quite niche and/or too specialised for me to understand to any extent. In addition I don't have any personal medical developments to report - just a vague feeling I've been taking Azathioprine too long (nearly 7 years) but not ready to try coming off it yet. We need more data!

Perhaps the most important new research is in cases of CLIPPERS in children. Two recent papers discuss this issue. The first from Mario Sa et al in the UK present three case studies of children diagnosed with CLIPPERS all of which had a poor outcome. They suggest that CLIPPERS may be a more aggressive and harder to treat condition in children. However in the second paper, Dr Tobin suggests that the three cases don't meet current diagnostic criteria for CLIPPERS (although they have a lot of shared characteristics). I have commented before on how this diagnostic uncertainty in CLIPPERS comes up again and again and, although the situation is slowly improving,  it would be a real advance to strengthen the diagnostic tests.


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.

Some updates

Another critter

A few snippets. First of all, apologies to any forum users who got put off by all the junk that had built up there in the last month or two. I have now cleared this out and will endeavour to keep a better eye on things.

One of the main international sites for rare diseases at the Genetic and Rare Diseases Information Centre has recently updated their page on CLIPPERS. Of potential interest for UK readers, they now list two UK organisations (Encephalitis Society and Brain & Spine Foundation) who may be able to offer support for CLIPPERS.

I'm seeing my neurologist next week for the first time in a while. As usual I'm wondering whether he will suggest any change in treatment. With that in mind, another recent case-report caught my eye as there were similarities to my own experience* and an emphasis on the need for continuing immuno-suppression.  The patient had facial paraesthesia (check), ataxia (check) and diplopia (check) and was treated with intravenous methyl prednisolone for 5 days (check) followed by a steroid taper starting at 60mg/day (check). However other non-steroid medication was not used in addition, and after steroid treatment ceased she relapsed. In the second round of treatment, Azathioprine was introduced during the steroid taper and maintained subsequently; there has been no further relapse for at least two years. So I think I'll be sticking with the Azathioprine.

*Update: Dr Tobin remarks that this case isn't completely typical of CLIPPERS.

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.