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.

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.

One of the more interesting aspects of research is when scientists have discussions in public via the letters pages of scientific journals. One such letter has appeared in response to a short paper which I discussed briefly last year. Essentially, in the original paper, the authors examined a CLIPPERS patient using some different types of MR image acquired on a modern scanner with a stronger magnetic field than in some previous studies ("3T").

In this letter ("CLIPPERS and its related disorders, relevance of brain 3.0 T MR"), Dr Taieb and his colleagues suggest that although some of the observed differences seen could be due to the stronger magnetic field and different types of images, they may still not be sufficient to distinguish CLIPPERS from two competing conditions (primary angitis of the CNS and B-cell CNS Lymphoma). They very sensibly suggest that more work needs to be done to confirm the original observations in more patients. Also they stress the need to compare the appearance of the MR images directly with that of tissue samples, so that a detailed understanding of how the tissue is affecting the MRI can be obtained.

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.

Darn it.

 Well, no sooner had I claimed there was no CLIPPERS news, then a mini-flurry of new reports has appeared. So the first (and probably least interesting from the patient perspective) is as follows:

3.0T MR investigation of CLIPPERS: 

Role of susceptibility weighted and perfusion weighted imaging. 

Pesaresi I, Sabato M, Desideri I, Puglioli M, Moretti P, Cosottini M., Unit of Neuroradiology, AOUP, Santa Chiara Hospital, Pisa, Italy.

This is a paper which investigates the appearance of CLIPPERS lesions on different kinds of MR image. Those of you who have had an MRI scan will probably remember the radiographer saying things like "First scan now for 2 minutes" or "Stay still, this one takes about 6 minutes". Each time, the scanner is being adjusted to collect different kinds of image which highlight different properties of brain tissue. Now, the images people most often report in CLIPPERS papers are the contrast-enhanced ones where an image is collected before and after the injection of a drug which changes the appearance of any lesions (because of damage to the Blood-Brain-Barrier). You can see images of my CLIPPER brain in this blog entry from 2011. 

I'm getting to the point now. It would be better if CLIPPERS lesions could be reliably identified using images which don't require injections. Also other kinds of MR images might show up new information about CLIPPERS in the brain. So these researchers in Italy looked at two different kinds of MR image on a 3T scanner (the number of T is the magnetic field strength and is usually 1.5T or 3T in hospitals). Although this sounds like a methods paper, it is really another case study as they only look at results from one patient - we're still a rare breed. However they do discuss what could be going on in the tissue to make the lesions visible in the two kinds of image which I'm sure will be of interest to the medics. More updates soon.

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.

News Updates

Some quick snippets this week.

• I had some informal feedback about my recent MRI. Apparently no sign of any new lesions and only a small bit of enhancing tissue (i.e. a bright spot) which has been there in all the scans so far. So no change.

• I got excited when I found out my case is being discussed at a big MRI conference in the US next week. It turns out it will probably just be one or two pictures in a Question and Answer educational session for radiologists and imaging scientists rather than a full conference paper but hey-ho!

• A new US/UK report on the impact of rare diseases on carers and sufferers was released last week; you can read it on-line here. In many ways the results aren't too surprising. For instance "Nearly all (96%/98% in the UK/USA) physicians stated more office vists are required to diagnose a rare disease patient". Still, I think that actually recording these things provides a baseline for policy-makers to look at and hopefully make better provision for those affected by rare diseases. At the moment CLIPPERS is still too rare to make it into the list of diseases in the report - it should be inserted between "Chromosome 9p deletion" and "Chronic lymphocytic leukemia" in the UK listing, but we're still too rare for that.

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.

 

This week was that time again, another trip to the good old NHNN for a brain-scan. This was a pre-emptive monitoring scan to see if there is anything untoward going on, as I haven't had one since last summer. I've no reason to think there is anything to see but, given the gap, it would be better to be able to intervene (with steroids etc) before overt symptoms appear. I've had enough of these now to know the routine pretty well. 1. Blood-test (beforehand), 2. Safety form, 3. Remove coins, keys, watch, belt, bank-cards, etc, 4. Scan 1, 5. Stick tube in arm for contrast, 6. Scan 2, 7. Wait for report. So I'm at 7 for now and it usually takes a week or two.

 

Interestingly, in the middle of the night after I had the scan, I woke up with a thumping headache and vomited three times over the next couple of hours (I should stress that no alcohol was involved ...). I did a bit of checking on the internet to see if the injected contrast ("Gadolinium") can have these effects. Nausea/vomiting are rarely reported but usually occur within minutes of the injection; reactions like itching, hives and rashes are more common. So I think it was probably coincidence; my doctor thought it sounded like a conventional migraine.

 

PS A new request for help on the forum this week from a CLIPPERS patient in the US. Does anyone out there have a similar experience?

 

Read other articles in this series at Living With CLIPPERS.

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International Rescue

George

Genetic and Rare Diseases Information Center (GARD)

Well this blog has passed the quality checks for resources linked to by GARD and is now listed here (under the Organizations tab). This might explain the slightly increased traffic I've seen here recently although Mark Zuckerberg still has nothing to lose sleep over. Still, if there is anyone out there with a probable/presumptive/possible CLIPPERS diagnosis who wants to share a bit of their story (anonymously if you like)  let me know (see the FAQ) and I'll start a summary page.

Prodding and Poking

Another couple of days of prodding and poking booked in for next week at the NHNN. As far as I know this is comfy chair,  "Brain MRI" and "Case Review", and no-doubt a bit of walking in a straight line and tracking various things being waved in front of my eyes. Also no doubt a medication review which could be important as on the current schedule I'll be off cortico-steroids completely in 6 weeks.

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.

First Treatment Update of 2012

Could this be why my liver test blipped recently?

Scantastic

You may remember me whinging a bit about being called for an MRI on short notice the week before Christmas. I didn't hear anything more about the scan and assumed everything was stable. As it happens I wasn't quite right about that - it turns out there has been some further improvement. 

According to the letter I received after Christmas I had some "further reduction in enhancement mainly in the pons along the Virchow-Robin spaces". Also, "the hyperintense signal  in the FLAIR images have also slightly regressed" and "there is overall improvement when compared to previous examinations". So this was a pleasant surprise (even though I had never heard of the Virchow-Robin spaces).

Festive Excess?

The other development over Christmas was that I had to step my Azathioprine dose back down from 75mg twice a day to 50mg following a blip on my weekly liver enzyme tests. After being retested after being on 50mg for a couple of weeks the blip had gone so I'm trying the 75mg dose again. Just had the second test after about a week and will see what shows up. I suspect the blip may have been a seasonal anomaly but I might be completely wrong! My target dose, subject to blood and liver tests staying clear, is 100mg twice daily at which point my medical team will consider reducing the maintenance Prednisolone.

So for now a relatively quite period and expecting another MRI before my clinic appointment in February. I'll just keep taking the tablets until then.

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.

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.

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.

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.