CLIPPERS Christmas 2013

To finish off 2013, two CLIPPERS cases are reported in a single paper which focuses on movement disorders. In the first case, the problem was limb tremor and mild dystonia. In the second case, the problem was hand and facial jerks, which manifested two years after CLIPPERS was diagnosed. The authors suggest that movement disorders, although not "classic" CLIPPERS symptoms, may be present because of the lesion location in particular cases. They point out that, in such cases, specialised treatment for the specific movment disorder may be warranted. (Incidentally, I'm not sure if this paper is freely available, but if anyone is interested and has problems accessing it, they can click through to the author information on the journal web-site and try emailing them for a "pre-print".)

Interestingly, I developed some intermittent stiffness and jerking movements in my legs after I began steroid treatment in 2011, but these gradually went away. They were definitely coincident with the start of treatment, but I've no idea why they should have appeared. My (no doubt completely wrong) theory was that as the brain inflammation improved slowly in response to steroids there was a period where some affected bits of brain were only partly functional?

Anyway, many thanks as ever to friends old and new who follow this site, and I wish you a happy and healthy 2014.

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CLIPPERS linked with MS again.

Another case study has appeared, this time originating  from Japan. This concerns a single subject (33 year old male) who displays enough symptoms to be considered a CLIPPERS sufferer. I'm sure I'm not the only one who reads these things and mentally ticks off the list of symptoms we have in common. This is one of those cases which I don't relate to particularly as I had a different set of symptoms (e.g. I didn't have scanning speech and retained my ability to walk - with difficulty). However CLIPPERS is hard to ring-fence because of the diffuse nature of the disease and I know the symptoms can vary quite a lot both in their type and their intensity. The pattern of lesions in this patient also looks more extensive than I had - compare my scan here with the patient brain in figure 1a - which may explain some of the differences.

The authors are reporting this case for the record without speculating too much. They suggest that CLIPPERS could be a syndrome rather than a disease - this is regularly discussed in the various papers. They also suggest in passing, that the similarities with Multiple Sclerosis mean that CLIPPERS could have something fundamentally in common with it. Here, "could" is the key word, as lots of things "could" be possible but there doesn't seem to be enough evidence (disclaimer: I am not a doctor) to suggest a firm linkage yet.

Read other articles in this series at Living With CLIPPERS.

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Aside: You say CLIPPERS ...

... I say CHIPPERS.

I'm sure that, before you found this site, you had to wade through lots of Google links for the Los Angeles Clippers and various pieces of personal grooming equipment. So I wondered if a more distinctive acronym could be squeezed out of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids. Fortunately, there are many web-sites out there to help with forming acronyms. I used Acronym Creator which worked pretty well.

As I used the same words which make up CLIPPERS as input, some of the results are quite contrived, or don't use all of the terms properly. Still, some of the better ones (which I have adapted slightly) were:

CHIPPERS     Chronic lympHocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids

CRIPES       ChRonic lymphocytic Inflammation with Pontine perivascular Enhancement responsive to Steroids 

CaLIPERS     Chronic Lymphocytic Inflammation with Pontine perivascular Enhancement Responsive to Steroids

CHIMERaS     CHronic lymphocytic  InflaMmation with Pontine perivascular Enhancement Responsive to Steroids

fLaPPERS     chronic  Lymphocytic inflammation with Pontine Perivascular Enhancement Responsive to Steroids

CaROLINES     ChROnic Lymphocytic INflammation with pontine perivascular Enhancement responsive to Steroids

CyCLOPS     ChroniC LymphOcytic inflammation with Pontine perivascular enhancement responsiv to Steroids

CavaLIERS     Chronic Lymphocytic Inflammation with pontine perivascular Enhancement Responsive to Steroids

Perhaps inevitably, none of these trip off the tongue quite as well as CLIPPERS. So we are probably stuck with it, at least until someone renames it Pittock-Keegan Syndrome ....

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Text Book Case?

Autumnal Shrooms

Another CLIPPERS case study has appeared and the interesting thing about it (to my untrained eye) is that there seems to be nothing interesting about it. In fact, if there was a text-book about CLIPPERS, I would go as far as to say it is a text-book case.

In brief, the subject is a 51 year old man who suffered progressive ataxia, diplopia and weakness before being scanned and revealing the characteristic pattern of CLIPPERS-like lesions in the MR images. There is a lot more detail in the article, but essentially treatment was high-dose (1g/day)  IV Prenisolone for 5 days followed by oral Prednisolone tapering down from 60mg/day and  Azathioprine  introduced in parallel to the tapering. This is basically the treatment I received, except I had a slower taper and a few stops and starts with the Azathioprine (because of blips in my blood-work).

This case is reassuring to see in the sense that many of the more recent case studies have either stretched the definition of CLIPPERS or have been complicated by CLIPPERS being diagnosed after, or alongside, other conditions. It is often difficult to get studies published when they are not sufficiently different to what has come before but, as the authors of this study say, "as a recently defined syndrome, any reported case is .... increasing the awareness of this disorder".

Read other articles in this series at Living With CLIPPERS.

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New On-Line CLIPPERS Resource

 

Recently I was contacted by Dave, who has a young daughter who has recently been diagnosed with CLIPPERS. He is keen to contribute to the CLIPPERS community and has set up a new web-site to relate his experience together with other information about CLIPPERS.  The website is: http://www.clippersdisease.org.uk/. Please take a look if you have a minute - from my perspective it's great to be able to go and read someone else's blog (!) It's also important to have more than one voice broadcasting about CLIPPERS to raise some awareness. 

Coincidentally, I just added a new case to People With CLIPPERS where the symptoms also occurred at a very young age. From the evidence to date it seems that CLIPPERS can strike at virtually any age, although it is fortunately less common (or at least less commonly diagnosed) in the young.

Read other articles in this series at Living With CLIPPERS.

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A World of CLIPPERS

This map, taken from Google Analytics, shows where everyone who has visited this blog comes from. Perhaps as expected, the most visitors so far have come from the USA (3877) and the UK (2680). But there have also been visitors from many other countries, including Canada (559), Australia (392) and Germany (214). From the map you can see there have been visitors from South America, Europe, Africa, Russia, China and Japan amongst others.

So if we assume that most people visiting are associated with someone who has (suspected) CLIPPERS, then it seems to be a truely global condition. So thanks to all who continue to visit the blog. I'll keep writing while there are still things to say, so let me know if you hear anything interesting about CLIPPERS.

Read other articles in this series at Living With CLIPPERS.

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CLIPPERS: State of the Nation

Greeek Cat on Patrol

The third, and most important, paper that appeared over the summer is to be published in the journal Clinical and Experimental Immunology. This paper, entitled CLIPPERS: Review of an increasingly recognized entity within the spectrum of inflammatory CNS disorders, is an up-to-date and substantial review of CLIPPERS in terms of definition, patients and treatments. I'll just pick a few things from the paper to talk about here. 

One is that the "pathogenetic concepts in CLIPPERS are principally still based on assumptions and speculations" which means that the cause and mechanism of CLIPPERS is still unknown.The authors emphasise that finding a unique cause is necessary for CLIPPERS to be confirmed to be a unique condition (as opposed to a strange variant of some other condition).

On the evidence so far, the authors say that the average age when CLIPPERS appears in is between 43 and 53 (this is only the average and obviously there are people much older and much younger who have CLIPPERS). They say there is some evidence that men are slightly more likely to be affected - or at least reported. These figures are based on more cases than were available when I speculated on who gets CLIPPERS in January 2012. Interestingly they also claim that the "clinical course ... seems to be relapsing-remitting". Although I progressed fairly slowly (over a few months) before treatment, I was definitely getting worse and didn't get as far as any remission before treatment. I'd be interested to hear whether anyone out there had remissions independent of treatment.

Also discussed are the various treatment strategies, focussing on the use of corticosteroids with or without additional immunosuppressants. Interestingly the authors note that the success of the steroid sparing agents (like Methotrexate, Azathioprine, Cyclophosphamide and Rituximab) when used in the absence of steroids has not been proven. It will take more longer-term patient monitoring and reporting to figure this out. I've now been on Azathioprine-only for over a year and OK so far ....

Finally, they note 56 cases reported in the literature. Presumably more cases than that are out there in the wild (including me!). It's hard to estimate the true number of cases - is it twice this number or ten times it or more?

This paper as a whole is a useful and detailed summary of where CLIPPERS is at in 2013. It also confirms there is still a way to go in terms of understanding, diagnosing and reliably treating the disease.

Read other articles in this series at Living With CLIPPERS.

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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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Darn it.

 Well, no sooner had I claimed there was no CLIPPERS news, then a mini-flurry of new reports has appeared. So the first (and probably least interesting from the patient perspective) is as follows:

3.0T MR investigation of CLIPPERS: 

Role of susceptibility weighted and perfusion weighted imaging. 

Pesaresi I, Sabato M, Desideri I, Puglioli M, Moretti P, Cosottini M., Unit of Neuroradiology, AOUP, Santa Chiara Hospital, Pisa, Italy.

This is a paper which investigates the appearance of CLIPPERS lesions on different kinds of MR image. Those of you who have had an MRI scan will probably remember the radiographer saying things like "First scan now for 2 minutes" or "Stay still, this one takes about 6 minutes". Each time, the scanner is being adjusted to collect different kinds of image which highlight different properties of brain tissue. Now, the images people most often report in CLIPPERS papers are the contrast-enhanced ones where an image is collected before and after the injection of a drug which changes the appearance of any lesions (because of damage to the Blood-Brain-Barrier). You can see images of my CLIPPER brain in this blog entry from 2011. 

I'm getting to the point now. It would be better if CLIPPERS lesions could be reliably identified using images which don't require injections. Also other kinds of MR images might show up new information about CLIPPERS in the brain. So these researchers in Italy looked at two different kinds of MR image on a 3T scanner (the number of T is the magnetic field strength and is usually 1.5T or 3T in hospitals). Although this sounds like a methods paper, it is really another case study as they only look at results from one patient - we're still a rare breed. However they do discuss what could be going on in the tissue to make the lesions visible in the two kinds of image which I'm sure will be of interest to the medics. More updates soon.

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NEWS FLASH: CLIPPER climbs Snowdon

Half-way up, before the cloud came down.

Two years ago this week I was discharged from hospital with unsteady (but improving) gait, coordination problems, double vision and a bag full of Prednisolone. I also had some new symptoms of occasional jerking and rigid limbs, and stalling of speech. Today I walked up Snowdon, the highest mountain in Wales, in what turned out to be fairly atrocious conditions. It still feels like a birthday though.

Everything could still turn 180 degrees in the future, but things are stable for now. I want to thank everyone who reads the blog for making it worthwhile and in particular those who have contacted me personally over the last couple of years. I know it's been a bit quiet recently, but unfortunately there just hasn't been much CLIPPERS news to report. If you know differently, let me know, or feel free to post something in the forum. 

Best Wishes,

 -Bill

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Rising interest in CLIPPERS

One way of measuring how interested the scientific community is in CLIPPERS is to look at the number of CLIPPERS papers which have been published in medical journals. To get a paper describing a study published, it has to be deemed appropriate for the readership of the journal (i.e. you don't usually see papers about liver disease in neurology journals), to show evidence of novelty or a contribution to knowledge, and to demonstrate that the work has been carried out to commonly acknowledged scientific standards. 

A related measure for each paper, which many people get obsessed by, is how many times that paper was referred to by other papers - the number of citations. A paper which generates a lot of interest will generally be referred to (cited) by many other papers in the future. So although it is early days, what can we deduce about CLIPPERS by looking at the number of papers published each year, and the number of citations of these papers.

Number of papers published per year

The first graph shows the number of papers published about CLIPPERS in each of the last four years. The numbers are relatively small, but they are rising. A list of most of these papers can be found here.

Number of CLIPPERS citations per year

The second graph shows the number of times the papers have been referred to (cited). Of course, often the later CLIPPERS papers are citing the earlier ones, but occasionally other papers in neurology refer to CLIPPERS too. The single most cited paper is the original one from Prof. Pittock at the Mayo Clinic. Coincidentally, I managed to speak with Prof. Pittock recently and hope to report some outcomes from that conversation quite soon. Watch this space.

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Sides

Sometimes it's easy to get into a routine with treatment, especially when it is pill-popping. Until something "interesting" (read "bad") happens I am not seeing the docs very often at all. To save me getting too complacent though, there are always treatment-related side-effects to worry about. While taking Azathioprine these have been very mild, at least the overt ones. In fact, the only thing I can report is occasional bouts of a metallic taste, and that seems quite common over a range of drugs. It is the covert side-effects which are potentially the more worrying which is why regular blood tests are the order of the day. 

I recently had one of those worrying phone messages from the medical centre which have zero information content and much larger worrying content "Please call the centre as soon as possible.". This was 2 days after I had been in for blood tests. So something obviously amiss, but what? Well to cut a long story short, one of my liver scores came back as "borderline" which triggered a "make an appointment to see your doctor" call. However this was done without reference to my history so when I phoned up and it got referred back to my regular doctor, he said it was OK. So in the land of CLIPPERS, "borderline" equals "fine" on this occasion ...

This, and some recent email correspondence with other CLIPPERS sufferers, made me think about side-effects a bit more, as I think they are often under-reported. So I started a new Forum topic if anyone is interested in relating their experience on medication.

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Steroids - Short-term, medium-term or long-term?

It is now fairly well established in the research literature that for many (most?) people diagnosed with CLIPPERS, high-dose intra-venous corticosteroids (usually Prednisolone) followed by lower-dose orally administered treatment improves the condition. What to do after this initial treatment phase and how to manage the condition in the medium and long term is more debatable. What seems clear, is that some kind of continuing treatment is required. The most common reported variants involve tapering the steroid dose to a low level and then either :

1. maintaining the patient at that dose 
2. maintaing the patient at that dose together with another immuno-suppressant
3. tapering the steroid down to nothing and maintaining on another immuno-suppressant

A new case study discusses these issues in the context of a 63 year old man diagnosed with CLIPPERS. This patient was treated with option 2 above (80mg/day Prednisolone) and then Methotrexate (2.5mg/weekly). However he had complications on Methotrexate and was moved to Azathioprine (100mg/day) and Prednisolone (now 20mg/day). On this treatment, the patient had a good response and no CLIPPERS relapses for 18 months (and onward).

The interesting thing about this paper, is the authors consider the treatment in the context of somewhat related disorders like cerebral vasculitis. There, option 2 is maintained for 2-5 years to prevent relapse, and they suggest a similar treatment strategy may be necessary in CLIPPERS. Now, this week is my 2-year anniversary of first CLIPPERS symptoms and 1-year anniversary of coming off Prednisolone entirely (option 3 above). The authors of this paper also say that successful steroid discontinuation in CLIPPERS has not been reported. My 1-year off-steroid period is too short to be called successful, as others have had relapses after longer periods without Prednisolone. So caution is required, but so far, so good.

Read other articles in this series at Living With CLIPPERS.

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CLIPPERS?

Regular readers will know that the individual CLIPPERS case studies coming out now seem to be about CLIPPERS in conjunction with other conditions. I think pure individual cases of CLIPPERS are not adding much to previous research, but studies with large numbers of subjects are much harder to organise, especially with the still relatively small number of cases.

So this new paper is about a case of Lymphoma following CLIPPERS. This  seems to be better argued (at least in my definitely inexpert opinion) than this previous one relating CLIPPERS to previous influenza. This new connection is slightly worrying, as the authors discuss possible mechanisms (that I don't pretend to understand) whereby this case of CLIPPERS was an early indicator of something worse. 

As ever, it is impossible to draw strong conclusions from a study of one patient and, given that this link has not been previously reported, it is certainly not common in the studies which have been seen to date. When I was being diagnosed, I was told some form of Lymphoma was a possible alternative diagnosis and crucially, that it would also respond to steroid treatment but only in the short term. In this paper, the steroid treatment seemed to become less effective quite quickly and MRI subsequently showed a very un-CLIPPERS like lesion, even though the early appearance was CLIPPERS-like. I held my breath for about the first 3 months I was on steroids but when my improvement was sustained, it became less likely to be Lymphoma. So is this case CLIPPERS becoming Lymphoma or early Lymphoma mimicking CLIPPERS? I tend to think it's the latter as diffuse Lymphoma is reportedly very rare and very hard to diagnose. But I am not a doctor so what do I know!?

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The Tip of the Iceberg?

General Wolfe, originally from my neck of the woods.

 A paper reviewing the state of CLIPPERS was presented at the 2013 meeting of the American Academy of Neurology. It was called "CLIPPERS: Rare Disease or Tip of the Iceberg? A Case Report and Literature Review".

The abstract linked to above is actually much less revealing than is implied by the title. The main part of the paper seems to be another CLIPPERS case study (at least as much as I can deduce from the abstract). The patient in this case was a 70 year old woman who as usual did not find an alternative diagnosis and did respond to high-dose Prednisolone. Frustratingly, there is a lack of follow-up detail here as, apparently the patient had no further steroids and was OK at 8 weeks, but from the abstract we don't know if she remained well longer term.

The promised critical review of the literature concluded that "CLIPPERS syndrome may be more common than realized" which has been the conclusion of many of the more extensive published papers. So I conclude we will have to wait a while for a more definitive assessment of the prevalence of CLIPPERS.

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News Updates

Some quick snippets this week.

• I had some informal feedback about my recent MRI. Apparently no sign of any new lesions and only a small bit of enhancing tissue (i.e. a bright spot) which has been there in all the scans so far. So no change.

• I got excited when I found out my case is being discussed at a big MRI conference in the US next week. It turns out it will probably just be one or two pictures in a Question and Answer educational session for radiologists and imaging scientists rather than a full conference paper but hey-ho!

• A new US/UK report on the impact of rare diseases on carers and sufferers was released last week; you can read it on-line here. In many ways the results aren't too surprising. For instance "Nearly all (96%/98% in the UK/USA) physicians stated more office vists are required to diagnose a rare disease patient". Still, I think that actually recording these things provides a baseline for policy-makers to look at and hopefully make better provision for those affected by rare diseases. At the moment CLIPPERS is still too rare to make it into the list of diseases in the report - it should be inserted between "Chromosome 9p deletion" and "Chronic lymphocytic leukemia" in the UK listing, but we're still too rare for that.

Read other articles in this series at Living With CLIPPERS.

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This week was that time again, another trip to the good old NHNN for a brain-scan. This was a pre-emptive monitoring scan to see if there is anything untoward going on, as I haven't had one since last summer. I've no reason to think there is anything to see but, given the gap, it would be better to be able to intervene (with steroids etc) before overt symptoms appear. I've had enough of these now to know the routine pretty well. 1. Blood-test (beforehand), 2. Safety form, 3. Remove coins, keys, watch, belt, bank-cards, etc, 4. Scan 1, 5. Stick tube in arm for contrast, 6. Scan 2, 7. Wait for report. So I'm at 7 for now and it usually takes a week or two.

 

Interestingly, in the middle of the night after I had the scan, I woke up with a thumping headache and vomited three times over the next couple of hours (I should stress that no alcohol was involved ...). I did a bit of checking on the internet to see if the injected contrast ("Gadolinium") can have these effects. Nausea/vomiting are rarely reported but usually occur within minutes of the injection; reactions like itching, hives and rashes are more common. So I think it was probably coincidence; my doctor thought it sounded like a conventional migraine.

 

PS A new request for help on the forum this week from a CLIPPERS patient in the US. Does anyone out there have a similar experience?

 

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Another Case

In a recent abstract from a conference program, published in the Journal of Investigative Medicine (vol. 61, no.2, Feb. 2013), a new case of CLIPPERS in the USA is described. This case is slightly more "classic" CLIPPERS than some of the more recent reported cases. Essentially a 52 year old woman suffered from left-sided weakness and gait ataxis which progressed to diplopia, disarthria and right-sided facial numbness. The doctors were initially stuck between possible diagnoses of CNS lymphoma, neurosarcoidosis and inflammatory disease (including CLIPPERS). These were also considered for me along the way and are all difficult to diagnose. This patient had a good response to treatment with corticosteroids and Azathioprine, although the authors noted that other non-CLIPPERS pathology also seemed to be present.

The authors suggest that patients with CLIPPERS-like signs can be treated for CLIPPERS without brain-biopsy. This point still seems controversial - there are a growing number of patients (including me) who have been treated without brain-biopsy to confirm the "Lymphocytic Inflammation" part of CLIPPERS. However, even with successful treatment, there remains diagnostic uncertainty without physical examination of brain tissue. Currently the diagnostic and treatment decisions have to be made carefully on a case by case basis.

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First Clinic of 2013 ...

The good old NHNN.

Well after a rather uneventful time on the CLIPPERS front I had my first clinic appointment for a while a couple of weeks ago. Essentially a meet and greet with my consultant, I did a few of the usual tricks. First was "walking in a straight-line heel-to-toe" (being able to do this was my personal benchmark of progress when my balance was screwed). Second was "touch your nose, now touch my finger". Third was "follow my finger without moving  your  head". I'm sure these are familliar to anyone with CLIPPERS or probably most neurological problems. I think he seemed reasonably happy and I didn't have any changes to report. I feel myself "shuffling" a bit sometimes but I can't measure it and it doesn't show up if I do any mobility tests. Might just be impending middle age. 

Anyway, I was a bit concerned he might decide to try and wean me off Azathioprine - my view is it's too early for anything like that. So I said "I'd like to stay on Azathioprine for at least another year." and he said something along the lines of "It will probably be a lot longer than that.". If it keeps me well, I'm not complaining. My consultant also reminded me that as I didn't have the brain biopsy, there is a missing piece of information in my diagnosis since the "Chronic Lymphocytic" bit of CLIPPERS hasn't been proven ... so technically it could still be something else (now that would be ironic ...) . But as I fit the CLIPPERS pattern very well on all the other tests and respond to the treatment it is still "presumed CLIPPERS". I requested another MRI as the last one I had was about 9 months ago, just to see what is going on, if anything. I'll let you know when I have that in a few weeks.

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New CLIPPERS Forum

The eagle-eyed amongst you may have spotted a new page in the bar above. I've added a forum to the blog (courtesy of Nabble) so anyone can post questions, requests for advice, CLIPPERS wisdom or other related anecdotes. This is a bit of an experiment - by the slowly increasing number of CLIPPERS-related emails I get, I think there is a need for somewhere that broader discussion can happen. On the other hand, if the forum is empty in 12 months I'll know that's not the case!

 

I'll have to change the setup if it fills up with junk too quickly, but so far that hasn't been a problem. Apparently, I am in some kind of grace period where the forum is run without advertisements. When that expires, in three weeks or so, I'll review how intrusive the advertising is and see how to proceed. Any adverts that do appear should only be on the forum page and enable Nabble to maintain their free forum service.

 

I put an initial post in the forum to welcome everyone. At some point I will add some proper guidelines but for now I think it best to let this thing evolve (or not as the case may be). Having said that, one or two things should be remembered if you intend posting to the forum:

 

1. remember this is public - anybody can read this
2. think twice (or thrice) before posting email addresses or contact details
3. this forum can't be a substitute for expert medical opinion

 

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Rare Disease Day

In the absence of a picture of a rare disease, here is a picture of a rare fruit.
(A Dragon Fruit)

I recently found out about Rare Disease Day which is next Thursday (28th February). This was because I came across Rare Disease UK ("The National Alliance for people with rare diseases & all who support them"). Apparently, in Europe the definition of a rare disease is one that affects less than 1 in 2000 people, and in the USA it is one which affects less than 200,000 Americans at any one time. So CLIPPERS is officially rare by either of these definitions, and on current numbers I suspect at the rarer end of the spectrum.

 

The point of Rare Disease Day and of Rare Disease UK (and similar organisations in other countries) is to raise awareness and particularly make the point that, although individual diseases and conditions may be rare, collectively it is not unusual to have experience of a rare disease. Indeed the statistic they quote is that 1 in 17 people will develop a rare disease at some point in their life. Rare Disease UK's longer term mission is to "campaign for the development and implementation of an effective strategy for rare diseases in the UK" which can be no bad thing.

 

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

One That Slipped Under the Radar

Or something like that.

I held off talking about this abstract in the hope I might find some more material.

0918: Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS): No Evidence For Antibodies To Neuronal Surface Antigens

P Maddison, P Gozzard, T Jaspan. Nottingham University Hospitals NHS Trust, UK

This was a paper presented at the Association of British Neurologists Annual Meeting 2011. I was hoping in the mean-time a full-length follow-up paper might appear but no joy. I don't pretend to understand the detail of this abstract but what makes it interesting is it is one of the only bits of work I have seen which focuses specifically on possible mechanisms from analysis of tissue samples rather than patient studies. The key phrase is (I think) "we looked for evidence of antibodies to neuronal surface antigens that could be pathogenic, or act as biomarkers of CLIPPERS". Deconstructing this as best I can, the authors believe that CLIPPERS has an auto-immune component which presumably means that antibodies are involved somewhere or other. According to Wikipedia, antigens are things which "evoke the production of ... antibodies" and pathogens are things which cause disease. So in this work, experiments were performed to see if specific kinds of antibodies could be found which either were the cause of CLIPPERS and/or were characteristic of it so that their presence in tissue could be used as a test for CLIPPERS i.e. be a biomarker for CLIPPERS.

Unfortunately they didn't find anything but finish by saying: "It is possible that CLIPPERS could be mediated by other organ-specific antibodies to perivascular antigenic targets in the central nervous system, or alternatively a predominantly cell-mediated immune process.". So the jury is still out on this one.

In other news, my first clinic visit for a while is next week. Will report back as and when.

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.

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

A Cold Start to 2013

A slightly delayed Happy New Year to everyone. It's been a quiet Christmas break on all CLIPPERS fronts. Nothing seems to have changed either personally (still taking the tablets) or research-wise (nothing new that I have found).  I passed my blood-monitoring test just before Christmas which was a minor relief as the levels of various things have fluctuated a bit previously. The next scheduled medical appointment I have is at the "Demyelinating Clinic" with my consultant in February, even though CLIPPERS is not thought to be a demyelinating disease (see e.g. Pittock et al, but of course I am not a doctor) - there's not enough of us in London to have a CLIPPERS clinic!

Currently we're pretty much snowed in and, like the garden birds,  waiting for the cold air to shift back East!

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.