CLIPPERS Cases Update

Trees by Bonfire Light

Dr Tobin and colleagues from the Mayo Clinic in the US and from Ghent in Belgium recently presented a paper at the ECTRIMS (European Committee for Treatment and Research in Multiple Sclerosis) conference. The paper is called "Defining a clinical, radiological and pathological signature of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids)" and that it was presented at a meeting primarily associated with Multiple Sclerosis emphasises that these brain disorders shouldn't be considered in isolation.  The work concerns efforts for a better working definition of CLIPPERS to aid with diagnosis and involves finding the key things which CLIPPERS patients have in common. In this still relatively small group of 34 subjects, eleven were excluded because of various findings which conflicted with what is known about CLIPPERS. This shows the difficulties of the whole diagnostic process for CLIPPERS.

One thing I found interesting, is that of the 23 subjects remaining, gait ataxia was the most common symptom (21/23) with diplopia (double vision), although the second most common symptom, lagging behind (13/23). In my case, diplopia was the first symptom with ataxia problems following, first with balance problems and later on with coordination and speech problems.

Another interesting thing is that all 11 patients in the study who stopped steroid treatment suffered symptom recurrence; it is not stated whether these patients were on other immuno-suppressant medication or not. I have managed to stay off steroids while taking Azathioprine but it is not clear whether I am just lucky or whether there is something which distinguishes my disease from others. I should avoid the temptation to read too much into this paper though, as conference presentations are usually early work in very short format which are followed up later by more substantial journal publications. Clearly though, this shows there are on-going collaborative efforts between researchers in the US and in Europe to move towards a better understanding of how CLIPPERS presents in patients.

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.

Another cautionary tale

Chewing the cud, or at least the hay, in Kent.

A recent article reminded me about the dangers of classing myself simply as "someone with CLIPPERS". Probabilities are funny things; when considering someone who is otherwise well, the lifetime chance of them getting diagnosed with CLIPPERS is very small indeed. The lifetime chance of them getting diagnosed with CLIPPERS AND some other condition is even smaller. However, for someone who already has a diagnosis of CLIPPERS, their chance of getting some other condition is just the same as anyone else (unless CLIPPERS has some mysterious protective effect which seems unlikely).

In their article "Stroke mimicking relapse in a patient with CLIPPERS syndrome" (unfortunately, not freely available), Dr Lefaucher and colleagues from Rouen describe exactly this latter set of circumstances.  A 52-year old man who had been diagnosed with CLIPPERS four years previously presented with double vision and ataxia, both common symptoms of CLIPPERS. After running some tests, a particular kind of stroke affecting a similar region of the brain as CLIPPERS was diagnosed and the patient was treated accordingly. In the paper, the authors briefly discuss whether disease processes associated with CLIPPERS could have made this kind of stroke more likely in this patient. They suggest that damage to small vessels after inflammatory disease (i.e. like CLIPPERS) could be a risk factor for subsequent stroke. However they also say, with a slightly odd choice of words, that the association between CLIPPERS and stroke in this case is simply "fortuitous" - I think I prefer the term "coincidental" but I'm pleased they concede that it could be just "one of those things" (my phrasing).

As someone in reasonable health, apart from CLIPPERS, and approaching middle-age this article reminded me that just because I drew the short straw in terms of rare cerebellar disease, doesn't mean that I am immune from any of the more common conditions that can appear as we age. So it's definitely worth doing the usual things to stay healthy to avoid as far as possible any other surprises.

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.

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.

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.

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 Now in Asia

Internal Medicine 51(9), pp 1115-1119, 2012

A new CLIPPERS case has just been reported in East Asia in the first CLIPPERS-related journal article I've seen for a while. You can read the full paper here. 

In brief though, it describes a 54 year-old woman with what sound like fairly typical symptoms of CLIPPERS (diplopia, paresthesia, gait disturbance) backed up by bright lesions towards the back and base of the brain visible in MRI. The authors are particularly keen to discuss possible allergy-based triggers for CLIPPERS. I have a history of mild allergies (occasional asthma, eczema etc) but wouldn't want to read too much into it at this stage. I'm trying to find out whether my "serum IgE" was elevated, as has been noted for several CLIPPERS patients, and will report back here

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.

Who Gets CLIPPERS?

There have been a few reported cases of CLIPPERS now and I wondered if there was any pattern. There's not enough cases or consistency of reported information to go into much detail but I thought it worth a look.

So simple things first, there's been an even split in the cases reported to date of 8 men and 9 women. Including me and the one female and one other male CLIPPERS patient I've heard about so far makes for 10 men and 10 women. On the limited information available it doesn't seem that men are any more or less likely to be diagnosed with CLIPPERS than women.

The next most obvious thing to look at is age at onset of symptoms.

Insomnia

I've never had significant problems with sleeping beyond the occasional restless night or early waking. But this year I've had two recent but different episodes of insomnia, one pre-CLIPPERS and one current, which go beyond simply "not being able to sleep". I don't know if they are stress-related, CLIPPERS-related or prednisolone-related. In both cases I do get some sleep and am not incapcitated like Al Pacino but the regularity of the sleep disturbance is strange.

If only it was this easy