One of the problems with diagnosing CLIPPERS is that there isn't a definitive test. So a case for CLIPPERS has to be constructed from the available evidence and by excluding other conditions. The definition of CLIPPERS is still somewhat vague with some open questions about whether cases which don't fit the established pattern are CLIPPERS, or some other unrecognised condition, or a combination. A case recently reported as CLIPPERS by Guillaume Taieb and colleagues demonstrates these problems.
Their patient aged 46 presented with some features of CLIPPERS. Specifically:
- gait ataxia (coordination problems associated with walking)
- dysarthria (problems with speech production)
- dysphagia (problems with swallowing)
- tetra-paresia (weakness in all four limbs)
- apathy
Unlike other reported cases this patient had suffered other problems since age 13 including:
- left/right peripheral facial palsy (paralysis of facial muscles)
- hearing impairment
- diplopia (double vision)
- hiccups
These various attacks were treated successfully with Prednisolone but several MRI scans showed no abnormalities until the latest episodes when aged 46 which again responded to Prednisolone. Professor Keegan (the senior author on the original CLIPPERS paper) replies by pointing out differences from the original CLIPPERS cohort (notably the long history of symptoms) and says "... CLIPPERS syndrome is likely to be far broader than the initial description that we have published".
So is this case CLIPPERS or not? It's certainly atypical compared with other reports but also shares many reported features. It will be interesting to see whether this case remains an isolated example or whether similar cases appear which justify a broadening of CLIPPERS. Already there has been one paper suggesting that the existing description of CLIPPERS be widened to more accurately describe the observed pattern of lesions in many cases.
Simon NG, Parratt JD, Barnett MH, Buckland ME, Gupta R, Hayes MW, Masters LT, Reddel SW
J Neurol Neurosurg Psychiatry. 2011 Nov 5. [Epub ahead of print]
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.
