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)
- Lymphocytic - To do with lymphocytes which are a special kind of white blood cell which deal with immune responses in the body.
- Inflammation with - A reaction of tissue to something harmful. It is a natural process intended to protect and heal and is usually associated with tissue containing blood vessels - so-called vascular tissue.
- Pontine - Related to the Pons (number 4 in the image below), a part of the brain concerned with movement and posture including eye movement and also levels of sleep and arousal.
(John A Beal, PhD, Dep't. of Cellular Biology & Anatomy, Louisiana State University Health Sciences Center Shreveport, available under Wikimedia Creative Commons CC-BY)
- Perivascular - In the vicinity of blood vessels.
- Enhancement - This refers to visibility in a special kind of MRI brain scan ("contrast-enhanced") where an injection is given which makes areas of inflammation brighter than normal tissue.
- Responsive to - The name includes something about how it responds to treatment!
- Steroids - Or more specifically corticosteroids which generally have a role in controlling inflammation.
This is all becoming a bit circular. At present to be labelled with CLIPPERS requires that your condition responds to treatment in a way consistent with CLIPPERS! In an ideal world diagnosis would proceed based on tests and then treatment would follow. Here treatment response is part of the label. In other words, if your condition doesn't respond to treatment with steroids as would be used for CLIPPERS then you haven't got CLIPPERS. In the next few years, and especially if many more cases appear, CLIPPERS is going to be pulled apart, redefined and possibly subsumed into another class of existing disorders. More specific diagnostic tests may emerge which mean that treatment response is no longer part of the definition. Things could change quite quickly and whatever happens, it should mean that the medical community understand more about the diagnosis, treatment and prognosis of this condition, whatever it is eventually called.
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.