With a Bang or a Whimper?

 

A squawk

My CLIPPERS 2011 ended with more of a whimper - or at least a squawk -  than a bang. On Monday I was booked in at the Day Care Unit at NHNN for an MRI and a meeting with my consultant. On the previous Friday the MRI appointment was pulled forward so I had to be at the unit for 8:45AM. This meant leaving the house earlier than I normally do to get to work! On the first day of my Christmas holiday!! I shouldn't moan too much though - comprehensive monitoring is a good thing.

A Very Merry CLIPPERS Christmas!

 

As we move towards the Christmas holiday period there will be slightly less frequent updates here for a little while - not no update, just not quite the as-regular-as-clock-work twice-a-week schedule that hopefully one or two have got used to. For those who want to keep up to date without the hassle of checking back here, a quick reminder that you can Subscribe By Email from any page and receive the latest updates automatically.

In terms of news, I'm now maintained on 15mg/day Prednisolone and two weeks into Azathioprine which has now moved up to 50mg twice a day. I feel pretty good and everything is pretty normal as far as I can tell. I've just started a weekly schedule of blood tests to monitor the Azathioprine effects on my blood count and liver and kidney function. I thought that was it for 2011 ...

No, I won't look like Arnold Schwarzeneggar ...

... has been my response to several people when they heard I was taking steroids.

So let's deal with the biggest source of confusion right away.

• Body-builders and people who want to build muscle-mass take anabolic steroids.
• People with inflammatory conditions including CLIPPERS take corticosteroids.

It turns out taking corticosteroids can result in weight gain but mostly as fat around the face, neck and stomach, which would probably make me look even less like Arnold Schwarzneggar. On the plus side I'm unlikely to suffer from "Roid Rage" or testicular atrophy either. Phew.

WE Ask The Questions

Now that I've got over my acute CLIPPERS symptoms and having hit a stable patch supported by various pills there's been some more time for reflection. That is reflection on the state of CLIPPERS rather than the reflection of a few months ago which without, wanting to be melodramatic, was more focussed on questions like "Will I make it as far as Christmas?".

Thanks Sis!

Snapshot of a card I recently received.
http://www.edwardmonkton.com/
http://www.reallygood.uk.com/

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 Not So Curious Case of CLIPPERS?

This month another CLIPPERS case study appeared, a report about a 56 year old man who I'll call "Sheldon" for ease of discussion. Although these single subject studies are limited in the sense that small numbers of cases don't allow general conclusions about CLIPPERS to be drawn, they are still of interest given the relatively small number of published reports to date. To me the interest in the recent report from Biotti et al is that this case is unremarkable and seems to fit fairly well with the bulk of the other reports - and with my own experience.

CLIPPERS Progress Assessment

Just back from 2 days as a guest of the Day Care Unit at the NHNN, at Queen Square. This for reassessment, progress reports and a decision about future treatment options. The background is that I am now in the last week of my decreasing steroid taper but there is growing evidence that CLIPPERS is a chronic condition which must be carefully managed with continued drug treatment to prevent relapse.

Autumnal Queen Square

Once More Unto the Breach, Dear Friends

Six months ago I first got symptoms of what I now know is probably CLIPPERS. This week it's time for two days of reassessment, fortunately as an out-patient this time. This also coincides with the last few days of my reducing steroid course which so far has done it's job and kept CLIPPERS at bay.  So with the benefit of hindsight, time for a brief recap of the last few months.

You Oligoclonal What?

One thing related to diagnosis which was mentioned to me in hospital a couple of times was oligoclonal bands. I'd never heard of these, had no idea what they were and didn't even know if having them was a good thing or a bad thing. Since reading some CLIPPERS papers I noticed they always comment about oligoclonal bands so they must be important; I've also seen them often mentioned in connection with Multiple Sclerosis diagnosis. So in case you were wondering ...

A Curious Case of CLIPPERS?

One of the problems with diagnosing CLIPPERS is that there isn't a definitive test. So a case for CLIPPERS has to be constructed from the available evidence and by excluding other conditions. The definition of CLIPPERS is still somewhat vague with some open questions about whether cases which don't fit the established pattern are CLIPPERS, or some other unrecognised condition, or a combination. A case recently reported as CLIPPERS by Guillaume Taieb and colleagues demonstrates these problems.

Treatment - The Next 4 Weeks

Some readers have asked how my treatment has been going since the last update.

October - 40mg/30mg Prednisolone
Things are continuing to improve.  The speech "glitches" have virtually disappeared. The walking coordination glitches have reduced in intensity and frequency and are absent for large portions of the day. I would put my balance/coordination at around 95% of normal. Apart from insomnia, the most obvious (to me) remaining symptoms are the tightness in my right forearm which also locks up further with the walking coordination glitches. Also some intermittent tight feeling across my lower right ribs but this is at the level of discomfort rather than anything worse.

Prednisolone dosing plan

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

Frying Tonight !

I've worked in and around medical imaging for most of my career. In that time I've mostly worked around MRI applications and as a result had a number of MRI brain scans as a volunteer. You can do this with MRI quite safely as there are no known mechanisms by which the magnetic fields and radio-frequency pulses used in the machines can harm human tissue. In technical terms, MRI doesn't use ionizing radiation. Other common medical imaging techniques like X-ray, CT and PET do use ionizing radiation and there is a risk-benefit aspect to their use. Most people are very wary of "radiation" and tend to regard any exposure as far riskier than it actually is. This is probably due to associations with the nuclear arms race and accidents at places like Windscale, Three-Mile Island, Chernobyl and Fukushima - which isn't to say these weren't dangerous and highly undesirable events.

Why I've Got CLIPPERS ...

... as suggested by a variety of people.

When something unexpected happens it's a natural reaction to think about the cause. For medical conditions establishing cause is often part of the diagnostic process. However there are many illnesses for which there is no obvious cause. Put another way it's hard to find a common link between people which might either explain the cause or at least predict who is more likely to be affected. So far with CLIPPERS there hasn't been an identified cause or common link, just a description of symptoms, findings and treatment response. However that hasn't stopped many suggestions being put forward (by others) for why I probably have CLIPPERS. So just for the record here they are.

MRI of CLIPPERS - And Now the Science Bit

CLIPPERS results in lesions with a characteristic appearance, both in terms of where they are and what they look like, on brain MRI. Pittock et al describe them as "a characteristic pattern of punctuate and curvilinear enhancement peppering the pons and extending variably into the medulla, brachim pontis and mid-brain". The lesions are thought to result from some underlying inflammatory process which has yet to be identified. The exact number and location of lesions is different amongst different patients, presumably contributing to the variability in symptoms seen.

To BB Or Not To BB ...

... That Is The Question

During the investigations which resulted in my current diagnosis of "presumptive CLIPPERS" I declined a brain biopsy (BB) (the surgical removal of a brain-tissue sample for laboratory investigation). Here's why.

Life in the Slow Lane

I walk fast, I always have done. In part it's a physical thing as being tall means I need fewer steps to get somewhere than many other folk (I have a higher cadence).  On the other hand natural impatience also plays a part. I don't like to dawdle; I'm a rush-hour train-commuter on a schedule who doesn't like delay. Suffering coordination and balance disorders which impact on walking offers a unique and unwelcome insight into life in the slow lane. Maybe it's payback time for years of commuting intolerance.

If not CLIPPERS then what?

Regular readers will know that a CLIPPERS diagnosis is neither quick nor simple. A large part of the process is excluding other conditions with similar or overlapping symptoms because it is vital that treatment fits the diagnosis as far as possible. Some of these conditions are more amenable to diagnosis than CLIPPERS because they are more common and better understood. The remainder are conditions about which less is known because of their rarity making differential diagnosis even harder. Here are the conditions I have found mentioned in the research literature which have been considered before diagnosing CLIPPERS. I bet that like me, most of you haven't heard of many of these. There are a frightening number of obscure and less obscure things which can go wrong with the old grey (and white) matter. As always please treat my interpretations of these complex disorders with caution and always go to authoritative sources for more information.

Treatment - The First 5 Weeks

Benchmark

Walking "heel-to-toe" without falling over and without assistance: one step or less.

September 2011 - IV

My treatment began in early September 2011 after over 3 weeks as an in-patient at the NHNN and came as a relief after a much longer that expected period of investigation. I was started on 1g/day of prednisolone delivered intravenously for 5 days for an hour a day. This is a fairly common treatment strategy for CLIPPERS and corticosteroids (like prednisolone) are often used to treat inflammatory disorders in brain and elsewhere. I improved during the IV treatment but not miraculously so. My facial numbness resolved, my walking became more fluid but my slow speech did return to normal. (In fact for a couple of days my speech went into over-drive and I couldn't be shut-up!). There was no change in my double vision and I still had numb fingers and tightness around my midriff. I also developed a tightness in my lower arms, especially on the right side. Additionally I developed "glitches" where my limbs would stiffen and I would walk like a tin-soldier for a few seconds if I was distracted or starting a new activity (e.g. walking somewhere after sitting down for a while). My speech would stall in a similar fashion - the words were queued up and ready to go but I couldn't get them out. These glitches were often accompanied by brief rushes of light-headedness. Over-all though, some modest improvement and a follow-up MRI two days after the end of the IV confirmed reduction in lesion appearance. So I was discharged and continued treatment at home. Note that this was another diagnostic hurdle - no improvement either in symptoms or in MRI would have meant that I wasn't "Responsive to Steroids" and therefore probably not CLIPPERS.

Aside on Hospital Life

And now a brief aside from the world of CLIPPERS for some reflections on hospital life. Although I've worked in and around hospitals for much of my career I had never before been admitted to one as a patient. So all of a sudden I found myself as an in-patient for several days of unspecified tests and facing an uncertain outcome. So how did I feel? Bloody marvellous as it turned out.

Four Week Journey to Diagnosis

In August 2011 I went into the National Hospital for Neurology and Neurosurgery for a few days of investigations expecting to be diagnosed with Multiple Sclerosis. I was finally let out exactly a month later with a diagnosis of "presumptive CLIPPERS" after MS and lots of other conditions had been considered, investigated and rejected. This simplified account is based on memory and doesn't include the extensive discussions amongst the medical staff on my team which happened behind the scenes and included the detailed results of many more tests than I report here. I've never stayed in hospital before and my experience was that all the medical staff at the NHNN and especially the nurses on John Young Ward were  professional, patient, kind and tolerant sometimes in difficult or unpleasant circumstances.

In-patient Investigations

Below is a summary in chronological order (as best I can remember) of 3 weeks of in-patient investigations at the National Hospital for Neurology and Neurosurgery in August/September 2011. I'll describe the progression of diagnoses later. Note that most of these tests were either looking for non-CLIPPERS conditions or were to exclude non-CLIPPERS conditions.  Some tests were prompted by findings peculiar to me in other investigations so may not form part of the standard work-up. For comparison I also list the other major investigations reported in the literature for patients being investigated for CLIPPERS. I'm sure there was also lots of variation in the blood and cerebral-spinal fluid tests which I don't have knowledge of. As Sherlock Holmes said:

 "Eliminate all other factors, and the one which remains must be the truth" !

Something's not right - developing CLIPPERS

Holidaying on the Greek island of Rhodes in May 2011 was the turning point and the end of my pre-CLIPPERS existence (if that doesn't sound too dramatic). Below is a short account of the time between first overt symptoms and being admitted to hospital for investigation.

Double Vision
The week after I got back I was watching television when I noticed that the peripheral vision on my right side was going slightly double. It was worse the further away and the further over to the right I was looking (by moving my eyes not my head). As the doubling was slight and only affected the edge of my vision I wasn't too concerned at first. Also I wear contact lenses for short-sight and get my eyes checked out twice a year so I knew there wasn't anything that got flagged up at my last eye exam. I could watch tv without a problem and get to work OK albeit with that weird feeling that there's something happening in the corner of your eye that you can't quite see.

Deconstructing C.L.I.P.P.E.R.S.

So what is CLIPPERS anyway? Well the acronym doesn't give much away and expanding it out isn't immediately informative to the non-specialist. So I'll break CLIPPERS down into it's component parts to show how CLIPPERS is really just a description of the syndrome. As usual this is my interpretation of a complex phenomena about which I don't have special knowledge (apart from experiencing it first hand!) so a full explanation will require some more book work. (AKGTQENR3XWH)

About Living With CLIPPERS

This blog is about a recently characterised brain disorder called CLIPPERS which produces a characteristic pattern of lesions (spots) in the brain. These lesions interfere with normal functioning in many tasks related to coordination, balance and (double) vision amongst others. I am a 44 year-old unremarkably healthy male who was diagnosed with CLIPPERS in September 2011. CLIPPERS is controversial because it's diagnosis is circumstantial at the moment and so there isn't any straight-forward test which identifies it. This also means that a diagnosis of CLIPPERS can be changed if the observations no-longer fit the pattern. It it is quite possible that in a few years it will be considered a special case of another disorder or there will be turn out to be several different kinds of CLIPPERS with their own patterns of progression or response to treatment.