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.

 

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 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.


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