What is this FAQ?
This FAQ summarises the situation regarding CLIPPERS as I understand it. I am not a medical professional and answers to any of these questions reflect my own understanding and interpretation of complex medical issues. Please do not use any of the information here as a substitute for expert medical advice.
Who wrote this blog?
Who wrote this blog?
I did - Bill Crum that is. You can read a bit about me here. If you want to contact me about something CLIPPERS related you can email me at wrcrum@hotmail.com (put CLIPPERS in the subject field). I can't advise about individual patient management or treatment though.
Where do I start?
To get the briefest possible introduction to CLIPPERS read on down the page. For the full story read the blog entries in chronological order. I started writing after my first bout of treatment but it tells the story from when I first got symptoms. I can be quite long-winded - just ask my wife (or anyone else who knows me!) - so you you might want to jump straight to the most important entries:
After I'd been doing this for a year, I posted some reflections on how the blog started and where it might go in the future. This 2014 paper gives an excellent overview of CLIPPERS including symptoms, diagnosis and treatments. It is aimed at medics but may well be helpful to a wider audience. CLIPPERS now also has an entry on this web-site also aimed at doctors.
After I'd been doing this for a year, I posted some reflections on how the blog started and where it might go in the future. This 2014 paper gives an excellent overview of CLIPPERS including symptoms, diagnosis and treatments. It is aimed at medics but may well be helpful to a wider audience. CLIPPERS now also has an entry on this web-site also aimed at doctors.
In 2022 I collected and enhanced the most pertinent blog posts into A Book on CLIPPERS which tells my story including chapters about symptoms, diagnosis, treatment and research.
What is CLIPPERS?
What is CLIPPERS?
CLIPPERS is a recently described inflammatory brain disorder which produces a characteristic pattern of spots (lesions) seen in contrast-enhanced brain MRI. Some common symptoms include double vision, balance/coordination problems and skin numbness/tingling in face or limbs. It is known to respond to corticosteroid treatment.
What does CLIPPERS stand for?
Originally, CLIPPERS stood for "Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids". In 2012, it was proposed to amend this to "Chronic Lymphocytic Inflammation with Pontocerebellar Perivascular Enhancement Responsive to Steroids" to better reflect the range of features seen in contrast-enhanced MRI. Currently both forms remain in use. The name says what the condition is (Chronic Lymphocytic Inflammation), what it looks like in contrast-enhanced MRI (Pontine Perivascular Enhancement) and how it can be treated (Responsive to Steroids).
Can I catch CLIPPERS from someone/something?
CLIPPERS is not a transmittable disease, more a condition. Think of the difference between the common cold which is infectious and eczema which isn't. CLIPPERS is more akin to eczema (but I stress only by analogy!).
How do I keep up with CLIPPERS research?
I try and report anything I find promptly on this blog and include up-to-date references. Good places to start looking elsewhere on the web are Google Scholar and PubMed with search terms like "CLIPPERS syndrome" or "CLIPPERS brain".
How do I get hold of CLIPPERS research papers?
I try and give links to papers listed on PubMed when I write about them or put links to them on the CLIPPERS Research Papers page. There is often a link to the electronic version of the paper from PubMed (if not, try searching for the title on Google). Some of these papers are freely available under the increasingly common Open Access agreements, however some are not. One common way to get hold of papers not freely available is to email the lead author and ask politely for a "pre-print". Authors are not obliged to provide these, but most journals allow pre-prints (final submitted versions of papers), and authors like their papers to be as widely read as possible.
How do I keep up with CLIPPERS research?
I try and report anything I find promptly on this blog and include up-to-date references. Good places to start looking elsewhere on the web are Google Scholar and PubMed with search terms like "CLIPPERS syndrome" or "CLIPPERS brain".
How do I get hold of CLIPPERS research papers?
I try and give links to papers listed on PubMed when I write about them or put links to them on the CLIPPERS Research Papers page. There is often a link to the electronic version of the paper from PubMed (if not, try searching for the title on Google). Some of these papers are freely available under the increasingly common Open Access agreements, however some are not. One common way to get hold of papers not freely available is to email the lead author and ask politely for a "pre-print". Authors are not obliged to provide these, but most journals allow pre-prints (final submitted versions of papers), and authors like their papers to be as widely read as possible.
Have I got CLIPPERS?
Almost certainly not if you consider the probability based on the number of reported cases against the world population. At present CLIPPERS is a rare, last resort diagnosis after many other more common conditions have been excluded, so statistically you are more likely to have something else. Few cases have been reported to date but there are likely to be more previously unidentified cases out there. That said some of us HAVE been diagnosed with CLIPPERS so as the other possibilities are ruled out it becomes more likely. It is very important to note that CLIPPERS cannot be self-diagnosed either using the internet or in any other way. At present diagnosis is very difficult even for experts and many other conditions have to be considered and excluded first.
How is CLIPPERS diagnosed?
CLIPPERS is diagnosed by first considering and excluding lots of other conditions. The pattern of brain lesions seen in MRI is distinctive for CLIPPERS but not enough on it's own to give a firm diagnosis. At the time of writing one of the contentious topics in the research literature is whether a brain biopsy (removal of a brain tissue sample) is required for a diagnosis of CLIPPERS. There have been a few cases reported where symptoms and MRI looked initially like CLIPPERS but then turned out to be something else. Direct examination of brain tissue samples offers the most information about what is happening at the microscopic level but is invasive and not without risk to the patient. However to offer the firmest possible diagnosis and extend our understanding of the disease, brain biosy is likely to form part of the diagnosis procedure in many cases.
How is CLIPPERS treated?
The typical reported treatment begins with a high dose corticosteroid administered intravenously (e.g. 1000mg methylprednisolone per day for 5 days in my case) followed by a much lower and reducing dose of orally administered corticosteroids (e.g. 60mg methylprednisolone in my case). I've also heard of "pulsed" therapy where high-dose corticosteroid is repeated every month or so. Often a complementary immunosuppressant is introduced as the corticosteroids are reduced in order to maintain the beneficial effect on the immune response without as much long-term risk. In my case this is Azathioprine (Imuran) but I've also seen reports of Methotrexate and Cyclosporin being used.
Can CLIPPERS be cured?
This is unclear at present. Most cases report improvement following steroid treatment but the amount of improvment is variable. It is quite common for symptoms to reappear when steroid treatment is discontinued. Either a maintenance, low-dose, corticosteroid or alternative immnosuppressant seems to be required to prevent relapse in many (most?) cases. So at present, CLIPPERS appears to be a chronic condition which can often be managed but has an unknown long-term prognosis.
Is there any other help out there on the InterWeb?
At present I have been unable to find much. CLIPPERS has an entry (note - currently, 2023, being revamped) at the NIH Office of Rare Diseases Research. Most countries seem to have some kind of organisation devoted to rare diseases. There is now a private Facebook group for affected families. If you find some other resources, feel free to tell me about them and I'll add them to this FAQ.
What can I do to help?
If you have been diagnosed with CLIPPERS you can volunteer to be included in a research programme at the Mayo Clinic, USA. The team there is gathering blood/tissue samples and medical records from as many CLIPPERS patients as possible to improve our understanding of the disease. Please see details of the study here or look for the CLIPPERS Repository link at the top of the blog.
I've been diagnosed - what do I call myself?
I don't know of an established term for people with CLIPPERS. Although not very grammatical, using CLIPPER as a noun (e.g. "I'm a CLIPPER", "me and my fellow CLIPPERS" etc) has a certain ring to it. My wife went through a phase of calling me "CLIPPY" which was a bit too off-hand in my opinion! Another regular correspondent calls herself a "CLIPPER-head". Anyone with a diagnosis of CLIPPERS probably has better things to worry about than the correct naming convention, but these are the best I can offer for now.
Is there any other help out there on the InterWeb?
At present I have been unable to find much. CLIPPERS has an entry (note - currently, 2023, being revamped) at the NIH Office of Rare Diseases Research. Most countries seem to have some kind of organisation devoted to rare diseases. There is now a private Facebook group for affected families. If you find some other resources, feel free to tell me about them and I'll add them to this FAQ.
What can I do to help?
If you have been diagnosed with CLIPPERS you can volunteer to be included in a research programme at the Mayo Clinic, USA. The team there is gathering blood/tissue samples and medical records from as many CLIPPERS patients as possible to improve our understanding of the disease. Please see details of the study here or look for the CLIPPERS Repository link at the top of the blog.
I've been diagnosed - what do I call myself?
I don't know of an established term for people with CLIPPERS. Although not very grammatical, using CLIPPER as a noun (e.g. "I'm a CLIPPER", "me and my fellow CLIPPERS" etc) has a certain ring to it. My wife went through a phase of calling me "CLIPPY" which was a bit too off-hand in my opinion! Another regular correspondent calls herself a "CLIPPER-head". Anyone with a diagnosis of CLIPPERS probably has better things to worry about than the correct naming convention, but these are the best I can offer for now.
