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.

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.

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.

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.

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.

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

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.

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

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.

 

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.

People With CLIPPERS

The elusive Cyril.

You may have noticed a new page has appeared below the title-bar - People With CLIPPERS.  I've now heard privately from several people who have been diagnosed with CLIPPERS and thought it would be great if we can use the power of the InterWeb to record and map some of these cases in a suitably anonymised fashion. So if you have been diagnosed with CLIPPERS please take a look and see if you want to contribute a brief summary of your CLIPPERS experience. There's absolutely no obligation and no nefarious, underhand motivation on my part except for thinking it would be fantastic to get more cases listed here than have appeared in any of the scientific papers published to date.

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.