One patient's story

This month I was privileged to hear from Keith whose wife Lisa has recently been diagnosed with CLIPPERS. She has had a tough time as she had a much more acute onset of symptoms than I did and suffered several relapses before the CLIPPERS diagnosis enabled her to receive the right treatment regime. Keith has been keeping an on-line journal about his experience which is updated pretty much in real time. You can read their story here (note that the entries are most-recent first). Keith is also keen to find out as much as possible about how best to support a recovering CLIPPERS patient through treatment.

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 Brazillian CLIPPERS case

The Peak District in September

To date, the majority of reported CLIPPERS cases have been from the United States, Europe and Australia. As awareness of the condition spreads, cases are being reported from other parts of the world. Recently the first Brazillian case report has been published where a 49 year-old first presented with CLIPPERS-like symptoms in 2001; of course CLIPPERS wasn't first published in a scientific journal until 2010. 

Interestingly, straight away this patient received pulse-steroid treatment which is now fairly standard when CLIPPERS is suspected; his symptoms improved. Unfortunately, Multiple Sclerosis was suspected and so other drug treatments which were not effective were subsequently used. In 2014, after significant deterioration, treatment for CLIPPERS was begun (Prednisolone and Azathioprine). There was then marked improvement of the lesion appearance in MRI but much less clinical (i.e. patient) improvement.

Although this is only a single case, it adds to the evidence that early diagnosis and treatment is important in CLIPPERS, as there may be progressive damage over time which cannot be completely reversed if treatment is delayed. I began steroid  treatment 3 months after I had my first symptoms which I think was quite fast. In my case my first symptom was diplopia and I was lucky to be spotted by someone in an eye clinic (once I'd been back a couple of times) who was worried about my worsening symptoms and referred me quickly to a neuro-opthalmology clinic. From there, I was lucky to be seen by a neurologist who was also sufficiently worried - especially when I failed all the balance tests - to get me admitted quickly to a neurology ward for in-patient investigation. It still took a month in hospital (in 2011) before they realised CLIPPERS was the likely culprit and began treatment. I think given that CLIPPERS was not widely recognised in 2011, this was still very fast and I am profoundly grateful to the people who pushed me rapidly through the healthcare system.

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.

Still in remission from cerebellar disease ...

... is the official word from my neurologist who I saw in his clinic in London last week. I did a few familiar tests such as walking "heel-to-toe"; he remembered that I had previously said I practiced this one at home to check if I had symptoms so he also wanted to make sure I couldn't still do it simply because I had been practicing so often! The other two tests were to do with eye coordination. One involved moving my finger from my nose to  his finger (and back again) while he waved his finger around in front of me. The other involved holding my head still and tracking his finger movement (left-right-up-down and then swiftly left-to-right) by only moving my eyes.

A medical student was present who helped with some of these tests and said I had nystagmus. I was surprised as I knew I had a lingering very mild nystagmus for a while, but I thought that had resolved and hoped it wasn't a sign of recurring CLIPPERS symptoms. Fortunately, it turned out that the student had been moving his finger too far/fast beyond the extremes of my left/right vision; everyone gets nystagmus if you try and track at these extremes.

We had a short discussion about continuing treatment. Regular and long-term readers of this blog will know that I have been maintained on Azathioprine alone (100mg twice a day) for three years now. We agreed that because of the uncertainty surrounding the disease, the possible catastrophic consequences of a relapse and my good tolerance so far of this drug, that this treatment should continue.

Interestingly, I recently came across a letter (unfortunately not freely available to read) about long-term CLIPPERS management which mentioned one or two cases of patients on various drug combinations staying well, or at least staying stable, for several years. Long term management is bound to become more of an issue in the future but it is not at all clear whether there is a single optimal treatment strategy or whether different treatments should be tailored for different patients. It may be as much luck as anything else that I have benefited from Azathioprine so far.

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.