Now that CLIPPERS has been recognised for a little while the number of new papers simply documenting cases has declined. There is at least some agreement about what constitutes CLIPPERS, even if there isn't a definitive test for it, so reporting new cases isn't, in itself, scientifically interesting. Of course, if the number of reported cases goes up, new research about the epidemiology of CLIPPERS may be required.
The new cases that are being reported seem to be unusual or novel in some way. A recent one from Dr Buttmann and colleagues in Germany concerns a 49 year old woman who has symptoms suggestive both of CLIPPERS and primary angiitis of the CNS (PACNS) (otherwise known as cerebral vasculitis, which was a candidate diagnosis for me for a short while). As well as the usual CLIPPERS symptoms this patient also had severe headaches and a seizure as well as other symptoms more associated with cerebral vasculitis than CLIPPERS. Dr Buttmann speculates whether this patient had unusual CLIPPERS, another condition which appeared like CLIPPERS or some combination of both conditions.
I have to say that, from my perspective, although these isolated cases are interesting, what I really want to see is something more detailed about number of reported cases and treatment options and responses in a large population. It is quite possible this information doesn't exist at present. It's a tricky situation as I obviously wouldn't wish CLIPPERS on anyone, or want the number of cases to rise. However, larger scale research may not happen when the number of cases stays small and there are already some treatments available.
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.
