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.

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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".

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