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.

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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.

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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.


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CLIPPERS diagnosis criteria revisited

Autumn mushrooms

The Mayo team have been busy as another interesting paper has appeared which really emphasises the continuing problems of diagnosis in CLIPPERS.

In the paper they perform a detailed comparison of 35 patients who had symptoms suggestive of CLIPPERS. (This blog gets a credit in the text as a patient advocacy site which helped connect some of the subjects with the Mayo). After re-assessment including detailed consideration of imaging and response to steroids, CLIPPERS was confirmed in 23 of the patients. One of the most striking results is that when the differences between the CLIPPERS and non-CLIPPERS groups were analysed, there were no significant differences in terms of symptoms commonly associated with CLIPPERS (e.g. gait ataxia, diplopia, dysarthria etc) or in terms of pre-existing cancers or smoking status. 

There is a lot of detail on the cases here which leads the authors to suggest a distinction between CLIPPERS cases: "probable" CLIPPERS for patients who fit all criteria but didn't have brain tissue biopsy and "definite" CLIPPERS for patients who fit all criteria but also had brain tissue biopsy with supportive findings. Unlike the paper from Dr Taieb's group I talked about in June, there is no focus here on the relapsing nature of the disease.

A revised set of diagnostic criteria is presented which includes some statements on neuropathology (i.e. tissue analysis). Interestingly both the clinical presentation and neuropathological criteria include the requirement "no better explanation" which emphasises that CLIPPERS is still regarded as somewhat of a "last resort" diagnosis.

On a slightly more optimistic note, I am now over 6 years since being discharged from hospital with a bag full of drugs, double vision and problems walking straight. The diagnosis was "probable" CLIPPERS and the outlook was distinctly uncertain. The outlook is still not exactly clear but everything else is pretty good.

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CPPERS, PPERS, LIPPERS or CLIPPERS?

There's a storm coming ...

A very interesting paper has recently appeared by Dr Taieb and colleagues about treatment strategies in CLIPPERS. I will return to this theme in a subsequent post but wanted to focus on something more basic, namely whether CLIPPERS is a single condition. In this recent works which reviews the majority of previously published cases, Dr Taieb proposes some division of CLIPPERS into sub-types. My reading of this is that it is a sub-division of convenience based on symptoms, investigations and response to treatment rather than any new insight into underlying biology. Nevertheless, it has been apparent for some time that there is immense variability under the CLIPPERS "umbrella".

Dr Taieb lists 5 key features of CLIPPERS which I paraphrase more simply here: (i) characteristic signs and symptoms, (ii) characteristic pattern of lesions seen in MRI, (iii) prompt response to steroid treatment, (iv) no competing diagnosis, (v) characteristic appearance of brain biopsy. So as a reminder, CLIPPERS stands for "Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids". With this in mind, Dr Taieb suggests that sufferers with a single attack and the first 4 or 5 key features are "PPERS" or "LIPPERS" respectively and sufferers with more than one attack and the first 4 or 5 key features are "CPPERS" or "CLIPPERS" respectively. 

I think the key interpretation of this system is that it is designed to reflect the available evidence about each case and make comparing cases easier. However it is influenced by the range of tests done (i.e. not everyone has brain biopsy) and the success of treatment. For instance, to date I have had a single attack and I declined a brain biopsy so I have the first 4 features and am "PPERS", but this can't distinguish between someone who has successful treatment and someone who simply has a disease that doesn't relapse.

In the diagnosis of Multiple Sclerosis, one of the criteria is that damage must have occurred at two different times - it is an inherently relapsing disease. However, presumably this is only true while effective treatments which could be given after a single episode are not available. I think the same is true of CLIPPERS and that when treatment strategies improve, the role of the "relapse" in diagnosis may dwindle.

(NOT A DOCTOR)

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CLIPPERS or IPPERS?

Not a family-friendly beach!

In a recent letter, Dr Taieb remarks that "most reported cases of CLIPPERS are in fact only ... PPERS due to the short follow-up and the absence of biopsy". So he is pointing out that in the absence of biopsy the "Chronic Lymphocytic" part of CLIPPERS is unproven. (He also takes out the "I" for Inflammation but I would argue that MRI findings in presumptive CLIPPERS patients suggest some form of inflammation.) I am happy to call myself an IPPER instead of a CLIPPER (as I am one of those unbiopsied cases). I'm not sure what the alternative is - pePPERS? 

This hides a serious debate about diagnosis of CLIPPERS - the CLIPPERS population is a mix of folk with different kinds of diagnostic tests and different levels of diagnostic confidence. We have all at least had most other conditions excluded. Dr Taieb also suggests that a relapsing-remitting pattern should form part of the diagnosis for CLIPPERS (like in Multiple Sclerosis). However, that would mean that I don't have CLIPPERS as I have, so far, been relapse-free on medication since my first onset.

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Diagnosing CLIPPERS Using MRI is Tricky

Spring is in the air, as evidenced by this recent visitor who has successfully found a way through the garden fence and is getting bigger.

Contrast-enhanced MRI of my brain in 2011.
The bright spots in the middle indicate CLIPPERS-related damage.

One of the hallmarks of CLIPPERS is the characteristic pattern of enhancing lesions many of you will have seen in MRI. It is tempting to regard the presence of a pattern like this as sufficient evidence to diagnose CLIPPERS, particularly when there is no clear alternative. Unfortunately, life is rarely that simple and there may be other reasons why patterns of lesions like this can develop.

Dr Taieb and colleagues have helpfully written a guide to diagnosis in these cases. Their paper, Punctate and curvilinear gadolinium enhancing lesions in the brain: a practical approach, examines 39 cases of their own where similar patterns of brain lesions occur, together with other reported cases, and details the many different problems which could be the cause. (Unfortunately, this paper isn't freely available to read from Springer - you can try asking Dr Taieb for a pre-print.)

This (above) is perhaps the most interesting part, a diagnostic flow-chart for when lesions are present. Interestingly Dr Taieb suggests brain-biopsy only as a last resort. Fitting CLIPPERS into a standard diagnostic process is clearly important. This paper may not be the last word on the subject but is a step in the right direction.

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CLIPPERS Cases Update

Trees by Bonfire Light

Dr Tobin and colleagues from the Mayo Clinic in the US and from Ghent in Belgium recently presented a paper at the ECTRIMS (European Committee for Treatment and Research in Multiple Sclerosis) conference. The paper is called "Defining a clinical, radiological and pathological signature of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids)" and that it was presented at a meeting primarily associated with Multiple Sclerosis emphasises that these brain disorders shouldn't be considered in isolation.  The work concerns efforts for a better working definition of CLIPPERS to aid with diagnosis and involves finding the key things which CLIPPERS patients have in common. In this still relatively small group of 34 subjects, eleven were excluded because of various findings which conflicted with what is known about CLIPPERS. This shows the difficulties of the whole diagnostic process for CLIPPERS.

One thing I found interesting, is that of the 23 subjects remaining, gait ataxia was the most common symptom (21/23) with diplopia (double vision), although the second most common symptom, lagging behind (13/23). In my case, diplopia was the first symptom with ataxia problems following, first with balance problems and later on with coordination and speech problems.

Another interesting thing is that all 11 patients in the study who stopped steroid treatment suffered symptom recurrence; it is not stated whether these patients were on other immuno-suppressant medication or not. I have managed to stay off steroids while taking Azathioprine but it is not clear whether I am just lucky or whether there is something which distinguishes my disease from others. I should avoid the temptation to read too much into this paper though, as conference presentations are usually early work in very short format which are followed up later by more substantial journal publications. Clearly though, this shows there are on-going collaborative efforts between researchers in the US and in Europe to move towards a better understanding of how CLIPPERS presents in patients.

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A Brazillian CLIPPERS case

The Peak District in September

To date, the majority of reported CLIPPERS cases have been from the United States, Europe and Australia. As awareness of the condition spreads, cases are being reported from other parts of the world. Recently the first Brazillian case report has been published where a 49 year-old first presented with CLIPPERS-like symptoms in 2001; of course CLIPPERS wasn't first published in a scientific journal until 2010. 

Interestingly, straight away this patient received pulse-steroid treatment which is now fairly standard when CLIPPERS is suspected; his symptoms improved. Unfortunately, Multiple Sclerosis was suspected and so other drug treatments which were not effective were subsequently used. In 2014, after significant deterioration, treatment for CLIPPERS was begun (Prednisolone and Azathioprine). There was then marked improvement of the lesion appearance in MRI but much less clinical (i.e. patient) improvement.

Although this is only a single case, it adds to the evidence that early diagnosis and treatment is important in CLIPPERS, as there may be progressive damage over time which cannot be completely reversed if treatment is delayed. I began steroid  treatment 3 months after I had my first symptoms which I think was quite fast. In my case my first symptom was diplopia and I was lucky to be spotted by someone in an eye clinic (once I'd been back a couple of times) who was worried about my worsening symptoms and referred me quickly to a neuro-opthalmology clinic. From there, I was lucky to be seen by a neurologist who was also sufficiently worried - especially when I failed all the balance tests - to get me admitted quickly to a neurology ward for in-patient investigation. It still took a month in hospital (in 2011) before they realised CLIPPERS was the likely culprit and began treatment. I think given that CLIPPERS was not widely recognised in 2011, this was still very fast and I am profoundly grateful to the people who pushed me rapidly through the healthcare system.

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CLIPPERS in the news

The Detroit News (not a publication I was previously familiar with) has recently run an article about CLIPPERS based on the experience of Roger, a military veteran in Michigan. You can read the article here:

http://www.detroitnews.com/story/life/wellness/2015/05/25/worlds-st-case-disorder-michigan/27944751/

(I can't guarantee this link will stay working forever as it is on an external site).

It's an interesting story and reminded me of my own experience when after several weeks of uncertainty one of my doctors produced this article about this "thing called CLIPPERS" which seemed to describe quite accurately what I had. I did wonder about the title: "World's 51st case of disorder is in Michigan". I haven't counted lately, but I suspect the number of published cases may be around (or probably exceed) 51 as they say in the article, but I also suspect the number of diagnosed cases worldwide runs into the hundreds. Whatever, it is still a very rare condition and stories like this which help raise the profile can only be a good thing. I hope Roger continues with his recovery from CLIPPERS.

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Historical CLIPPERS cases

Holiday snap from Southern Italy

I recently read an interesting short communication entitled "CLIPPERS among patients diagnosed with non-specific CNS neuroinflammatory diseases" by Dr Kerrn-Jespersen and colleagues from hospitals in Denmark. As regular readers of this blog will know, the term "CLIPPERS" was first used in 2010 in the now well-known paper by Dr Pittock and colleagues. They observed a consistent pattern of symptoms and treatment responses in a group of patients over several years which led them to the conclusion that a single previously unreported condition was responsible. 

CLIPPERS is very rare, which is presumably why it was not identified earlier. However, it is reasonable to suspect that there were other cases out there "in the wild" before 2010; these cases were presumably either diagnosed as something else or diagnosed as some generic inflammatory condition. In the Danish paper, the authors searched their hospital records between 1999 and 2013 for cases with descriptions reminiscent of CLIPPERS. After some investigation they found 3 patients (= 12.5% of their initial list of suspects) who justified being reclassified with a CLIPPERS diagnosis. This number may seem small, but it is from a limited number of European centres over a limited time-period and suggests there could be significant further cases out there.

 

Perhaps the most important practical outcome of the study for us patients is that the follow-up of their 3 cases confirmed that early and sustained treatment was important to minimise longer-term problems.

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More Medical Ponderings on CLIPPERS

Another paper from Dr Taieb appeared recently, again pondering on the relationship of CLIPPERS to other central nervous system disorders. In this short letter, "A central nervous system B-cell lymphoma arising two years after initial diagnosis of CLIPPERS", he considers a new case where an initial diagnosis of CLIPPERS was made which was based on tests including brain biopsy. Eighteen months later the patient relapsed whilst still taking Prednisolone and a diagnosis of presumed central nervous system B-cell lymphoma was made.

 

These cases are always worrying for those of us being treated for CLIPPERS. I guess the diagnosis had to change as the patient was no longer "responsive to steroids" which is required for CLIPPERS. But as ever, the question is, was this a simple case of mis-diagnosis under difficult conditions (not least as Lymphoma can also respond to steroids initially) or was it suggestive of CLIPPERS progressing to something else? Dr Taieb considers both these scenarios as possible and doesn't offer an opinion about which scenario he thinks most likely. It is also worth mentioning that Dr Taieb says that this case is one patient out of twelve studied in the 2012 French CLIPPERS cohort, the rest of whom (presumably) retained their original CLIPPERS diagnosis.

In other news, my shingles rash didn't get any worse and has largely cleared up. I have no idea if seven days of Acyclovir five times a day helped or not, but the rash stayed fairly localised. From what I have read, this was pretty mild for shingles as any discomfort stayed at the level of an annoying ache.

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CLIPPERS and Grade 1 Lymphomatoid Granulomatosis

In May, I reported on a curious case of CLIPPERS which involved skin lesions. There have now been two follow-up letters discussing this case. In the first, Dr Taieb (who has also published CLIPPERS-related papers) suggests that CLIPPERS could be a manifestation of a grade 1 Lymphomatoid Granulomatosis which is thought to be a pre-lymphoma condition. The key comment (from my reading) is that grade 1 Lymphomatoid Granulomatosis and CLIPPERS may be indistinguishable in terms of diagnosis and treatment response. In addition,  grade 1 Lymphomatoid Granulomatosis "does not necessarily progress to grades II or III" which would presumably explain why there are so many "stable" cases of CLIPPERS out there.

 

In response to this, the original author, Dr Kossard suggests this association is premature. My interpretation of the letters is that it is hard to be sure, even from studying tissue samples, about any possible relationship between CLIPPERS and Lymphomatoid Granulomatosis. It does seem, though, that the net is very gradually tightening around CLIPPERS in terms of figuring out exactly what it is and where it sits in relation to other rare conditions.

 

 

In other not-so-exciting news, I came out in a painful rash on my face which I initially thought was caused by  insect bites but got gradually larger. I was quite surprised to be told by my doctor I had shingles. Of course, being immune-suppressed, means that despite this rash being fairly small on the scale of shingles, no chances are being taken. So now I am taking anti-viral medication five-times a day as well as anti-viral ointment for my eye (as the rash is closer than it looks on the picture).

 

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Yet Another Curious Case of CLIPPERS

 A very interesting variant on CLIPPERS which may offer more research clues has recently appeared. In the paper, "Neurotropic T-cell lymphocytosis : a cutaneous expression of CLIPPERS",  a patient is described who satisfies the current diagnostic criteria for CLIPPERS. However, in addition this person also suffered from sub-cutaneous ("beneath skin")  lesions for some years before, and after, their CLIPPERS diagnosis. The authors points out the similarities in the composition of the skin lesions compared with what is observed in CLIPPERS brain lesions. They make the point that sampling  tissue outside the brain to test for CLIPPERS would be very helpful for diagnosis. However, in this case a brain biopsy for direct comparison was not available so at least one key part of the puzzle is currently missing. 

This is certainly the only case like this that I have read about; the observations are intriguing. I think that time will tell whether this turns out to be a key insight or an interesting aside.

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Strike 2

The second of the recent mini-flurry of CLIPPERS papers returns to the topic of CLIPPERS being mistaken for Primary Central Nervous System Lymphoma (PCNSL), with a short comment and response on the recent case-study by De Graaf et al where a patient fulfilling the diagnostic criteria for CLIPPERS went on to develop PCNSL. There is some discussion which I don't understand (as I am not a doctor) about the time-course of tests and patient response in this case. The advice from both sets of authors is that patients newly diagnosed with possible CLIPPERS should be closely followed, especially for the first 12 months, in case there is another cause. This comes back to the lack of a definitive diagnostic test for CLIPPERS, and the fact that ultimately the diagnosis is by exclusion of other conditions which could also have similar symptoms and MRI appearance. I was told in 2011 there was an outside chance I could have PCNSL rather than CLIPPERS - the big problem is that initially PCNSL responds to steroids like CLIPPERS but then returns and is very hard to treat. I spent a nervous few months on steroids before getting to the point where I thought that if it was PCNSL, that it should probably have recurred by now.

In fact, the most controversial part of the new comment is the title: "Should CLIPPERS Be Considered a Prelymphoma State or a New Inflammatory Disease?" From my understanding (doctor disclaimer again) it is pretty clear that whatever the problems presented by CLIPPERS, it is still preferable to PCNSL. While there have been one or two cases reported of PCNSL being mis-diagnosed as CLIPPERS, I'm not aware of much evidence for CLIPPERS being a precursor to something else - but it is quite early days. As there is some variation in CLIPPERS symptoms and response to treatment, it may be that it isn't a single well-defined "condition" but one which differs from person-to-person. In the absence of a definitive test for CLIPPERS this remains a possibility, particularly as there is increasing variability in the published cases regarded as CLIPPERS. 

Still lots to do then.

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I'll Have Mayo With That ...

... Light Mayo Obviously

I came across an interesting article on the website of the Mayo Clinic recently. Doctors from the Mayo were the first to characterise CLIPPERS and of course define the CLIPPERS acronym. The article - Multiple Sclerosis Mimickers - discusses conditions that can be mistakenly diagnosed as Multiple Sclerosis in the clinic. CLIPPERS is number two on the list, after Neuromyelitis Optica and before lymphoma. I'm sure I'm not the only one with first-hand experience of the diagnostic challenge that CLIPPERS presents. Really, the article is about recognising the contribution that Mayo researchers have made to our understanding of these diseases and conditions. Particularly interesting is how cases of what is now thought to be CLIPPERS were investigated by Dr Keegan very early in his career and then later in conjunction with Dr Pittock.

If anyone reading has picked up any interesting CLIPPERS-related snippets from the Mayo let me know and I'll post them here.

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Still Talking About CLIPPERS

Dr Keegan gave a talk about CLIPPERS at the National Institute of Health (USA) just over a week ago. From the events calendar:

"For his lecture at NIH, he will discuss Clippers Syndrome and offers the following objectives:
-Identify and treat a chronic inflammatory CNS disease
-Review differential diagnosis of brainstem predominant inflammatory disease
-Add a challenging acronym to neurological differential diagnosis"

This all sounds very interesting. We definitely want to identify and treat CLIPPERS and I'm assuming the third point is to promote CLIPPERS as a possible diagnosis which should be considered more widely. Raising awareness of CLIPPERS within the neurology community  can only be a good thing as presumably, the more cases are found, the more likely research will be done to find definitive causes and treatments.

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Complicating CLIPPERS

Case Study

Another interesting Case Study appeared last week in the journal Neurology. Unfortunately this paper isn't Open Access but the author, Dr Ortega, was kind enough to send me a copy. The paper describes a 61 year old woman who had a history of Multiple Sclerosis but then went on to develop CLIPPERS. The authors speculate that CLIPPERS was triggered following withdrawal of other MS medication.This is an interesting case but hopefully quite rare. Even so any more CLIPPERS clues are welcome and interactions with other disorders or medications are definitely worth hearing about..

CLIPPERS on the web

I stumbled across my brain on the web the other day - not something that happens every day. It was on the web site of  a private medical practice in the US specialising in dizziness and balance problems. My brain (scan) is on their page about auto-immune brain-disorders. Dr Hain says he's never seen a scan like it in 30 years of practice. I guess I'll take that as a compliment ...

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A Not So Curious Case of CLIPPERS?

This month another CLIPPERS case study appeared, a report about a 56 year old man who I'll call "Sheldon" for ease of discussion. Although these single subject studies are limited in the sense that small numbers of cases don't allow general conclusions about CLIPPERS to be drawn, they are still of interest given the relatively small number of published reports to date. To me the interest in the recent report from Biotti et al is that this case is unremarkable and seems to fit fairly well with the bulk of the other reports - and with my own experience.

Once More Unto the Breach, Dear Friends

Six months ago I first got symptoms of what I now know is probably CLIPPERS. This week it's time for two days of reassessment, fortunately as an out-patient this time. This also coincides with the last few days of my reducing steroid course which so far has done it's job and kept CLIPPERS at bay.  So with the benefit of hindsight, time for a brief recap of the last few months.