The Tip of the Iceberg?

General Wolfe, originally from my neck of the woods.

 A paper reviewing the state of CLIPPERS was presented at the 2013 meeting of the American Academy of Neurology. It was called "CLIPPERS: Rare Disease or Tip of the Iceberg? A Case Report and Literature Review".

The abstract linked to above is actually much less revealing than is implied by the title. The main part of the paper seems to be another CLIPPERS case study (at least as much as I can deduce from the abstract). The patient in this case was a 70 year old woman who as usual did not find an alternative diagnosis and did respond to high-dose Prednisolone. Frustratingly, there is a lack of follow-up detail here as, apparently the patient had no further steroids and was OK at 8 weeks, but from the abstract we don't know if she remained well longer term.

The promised critical review of the literature concluded that "CLIPPERS syndrome may be more common than realized" which has been the conclusion of many of the more extensive published papers. So I conclude we will have to wait a while for a more definitive assessment of the prevalence of CLIPPERS.

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.

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