Saturday, 19 July 2014

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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Monday, 7 July 2014

News Update


About 3 weeks ago, I had my first brain-scan of 2014, this time at the Institute of Neurology in London. I did ask my neurologist whether they were going to try anything different, as the IoN scanners are more research-oriented, but he told me they were just spreading the load of clinical imaging cases. It's funny, that having scans less often is in some ways more stressful because you wonder what might have been going on in the interim. Anyway, I have now had the unofficial feedback on the scans which was that:

"Everything is good. There was a tiny bit of signal change in the pons, as before and no enhancement. No change from last year."

So this is good news, especially as I have now been off Prednisolone for two years. Sounds like a bit of residual CLIPPERS-related damage which I will have to put up with, but I can't associate that with anything specific in day-to-day life.

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Saturday, 28 June 2014

An interesting case study

Swarming Bees in Kent
I recently came across an interesting CLIPPERS case report (Paroxysmal dysarthria and ataxia in chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) in which the patient had some experiences in common with mine. I have to be a bit careful as it is very tempting to pick out the bits I relate to while ignoring the bits I don't.
 
Anyway, this patient, a 56 year old man, was treated with 1g Prednisolone / day for three days followed by lower dose oral Prednisolone (30mg / day). The really interesting part is what happened immediately after the high-dose treatment. Quoting from the paper, "... 3 days after the first steroid pulse therapy, the patient presented with paroxysmal exacerbation of dysarthria and paroxysmal limb ataxia". Here, "paroxysmal" means "sudden outburst", dysarthria is speech disturbance and limb ataxia is problems with muscle control of limbs. They go on to say "These attacks lasted several seconds and recurred 20 or more times each day".
 
I have mentioned before, that I had what superficially seems a very similar experience when first treated with high-dose steroids. My problems came when initiating an action (e.g. getting up from the sofa, answering the telephone, crossing the road) and resulted in very restricted "stiff-limbed" motion and inability to talk for several seconds. I had to carefully plan things in advance so I didn't freeze at the wrong moment - especially when crossing the road! Unlike this patient, I had no additional treatment and my episodes gradually reduced in frequency and severity over a few weeks. I don't know if this strikes a chord with anyone else, but it is the first time I remember seeing this effect reported in a paper.
 
I'm currently waiting on a report of a recent "routine" brain-scan - will update when I get it.
 
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Sunday, 8 June 2014

CLIPPERS Research Update

A good day for sun-bathing!
A couple more brief papers appeared recently showing that research and discussion into the possible mechanisms involved in CLIPPERS is on-going.

The first considers the relationship of CLIPPERS to a form of cerebral vasculitis - this was on the list of possible diagnoses I encountered in 2011. Interestingly, this paper (CLIPPERS With Chronic Small Vessel Damage: More Overlap With Small Vessel Vasculitis) is written from the perspective of the neuropathologist and makes the point that to-date there has been perhaps more emphasis on neuroimaging rather than direct examination of tissue. Presumably, this is partly because of the practical challenges of brain biopsy in the regions typically affected in CLIPPERS. The key finding in their described CLIPPERS case is that "small vessel injury" could be seen despite vasculitis not forming part of the original disease description. They do concede that vasculitis might not be the root cause of the observed vessel damage but carefully consider the evidence for this and other causes.

The second paper, Diffusely Infiltrating Central Nervous System Lymphoma Involving the Brainstem in an Immune-Competent Patient,  considers a case initially thought to be CLIPPERS but, after failing to respond to steroids, subsequently found to be lymphoma. I have to say, that the contrast-enhanced imaging in this case, particularly figure 1c, does not look quite the same as similar images of other CLIPPERS patients I have seen but .... I am not a doctor. The whole issue of the relationship of CLIPPERS to CNS lymphoma is vital to resolve. It is not clear to me whether it is simply a diagnostic issue or whether, as some have suggested, there may be a biological relationship between the two conditions.

Read other articles in this series at Living With CLIPPERS.

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