This month another CLIPPERS case study appeared, a report about a 56 year old man who I'll call "Sheldon" for ease of discussion. Although these single subject studies are limited in the sense that small numbers of cases don't allow general conclusions about CLIPPERS to be drawn, they are still of interest given the relatively small number of published reports to date. To me the interest in the recent report from Biotti et al is that this case is unremarkable and seems to fit fairly well with the bulk of the other reports - and with my own experience.
Symptoms and Diagnosis
So essentially in this report "Sheldon" suffered progressive ataxia and "binocular horizontal diplopia", both symptoms I had before being admitted to hospital. He also had normal opthalmoscopy as did I and lots of other investigations. As his medical team judged that Sheldon's presentation was typical of CLIPPERS with no indications of other conditions they decided that brain biopsy was not worth the risk. Sheldon also had an absence of oligoclonal bands which was also the same for me.
So essentially in this report "Sheldon" suffered progressive ataxia and "binocular horizontal diplopia", both symptoms I had before being admitted to hospital. He also had normal opthalmoscopy as did I and lots of other investigations. As his medical team judged that Sheldon's presentation was typical of CLIPPERS with no indications of other conditions they decided that brain biopsy was not worth the risk. Sheldon also had an absence of oligoclonal bands which was also the same for me.
Treatment
Sheldon was treated with 5 days of 1g intra-venous Prednisolone followed by oral Prednisolone at what sounds like an even higher dose than me (1mg/kg body weight which would be 76mg for me compared with my 60mg taper). Unlike me he wasn't treated with a steroid taper but moved straight onto Azathioprine which I am now taking in conjunction with Prednisolone. The encouraging thing is that Sheldon has had no relapse up to his 7 month follow-up and resolution of most symptoms.
Long Term
What we need now is a move to longer term studies with more subjects. Easier said than done I know but now there is an emerging concensus on CLIPPERS presentation and some common treatment strategies which seem to work in the short-term, long-term prospects are what we need to know about.
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.