smart aleck = someone who is pretentious about their own cleverness
Just For the Record
A couple of months ago I posted an entry here moaning about a CLIPPERS case study which I felt confused cause and effect and made far too much of a supposed link between CLIPPERS and flu vaccination. Anyway I felt strongly enough to write a letter to the journal in question to point out the weaknesses in the paper. Now letters to journals are sent for external review before publication which took a while, but I thought it a good sign that they didn't immediately reject it. In the end they waited for a review to come back ... and then rejected it, which is of course their privilege. As I put several hours work into this letter and because I think I made some legitimate points I've put a copy below for posterity. In the interests of balance, I've also included the reviewer's comments so you can see why they rejected it and my subsequent email to the editor of the journal saying why I thought the reviewer was wrong too. If you work in academia you frequently think reviewers are wrong and sometimes they are, but often it's just you being a smart aleck ... ;-)
Regarding ”Chronic Lymphocytic
Inflammation With Pontine Perivascular Enhancement Responsive to Steroids
Following Influenza Vaccination” by Hillesheim et al
To the Editor. – The article titled
”Chronic Lymphocytic Inflammation With
Pontine Perivascular Enhancement Responsive to Steroids Following Influenza
Vaccination” by Paul Hillesheim et al (1) reports a new case of
CLIPPERS, a recently characterised inflammatory condition of the central
nervous system (2). This is an interesting case
which adds to the description of CLIPPERS-like disorders but raises questions
about the definition and diagnosis of CLIPPERS. The authors also make an
unjustified association between CLIPPERS and recent flu vaccination which is
not supported by the available data and is potentially misleading.
This case is somewhat atypical; in 19 reported cases to date only 3 others did not suffer diplopia. In addition, the present case does not exhibit the distributed pattern of enhancing punctuate lesions seen previously but has a larger focal lesion within the central pons. The current case is also at the upper end of the reported age range – only one older case has been described, with 13 out of 19 cases aged between 45 and 75 (Figure 1). Clearly though, this case is CLIPPERS-like and could simply represent population variation in a condition which is currently poorly understood.
 | ||||
| Figure 1 |
The connection with influenza vaccination warrants further investigation. Dr Hillesheim associates vaccination with CLIPPERS in the article title but I am of the strong belief that this is misleading and unwarranted. The authors make several statements which are technically true but not defendable in this context. For instance, “… this is the first reported case of CLIPPERS after vaccination” and “vaccination … raises the tantalizing possibility that this may be the triggering event”. While it is strictly true that the patient was vaccinated shortly before symptom onset, no evidence for a linkage with CLIPPERS is presented and no mechanism is suggested. It is quite right that the vaccination is recorded, but its significance cannot be established from this single case. Finally, the authors list existing neurologic complications of vaccination and state “CLIPPERS is another complication that should be added to this list”. There is no evidence to support this assertion and indeed no other reported CLIPPERS cases have mentioned vaccination. Therefore it is premature to make this connection on the basis of a single case and to link CLIPPERS with vaccination in the article title.
I should declare
an interest; I was diagnosed with CLIPPERS in September 2011 and am currently
well on tapering Prednisolone and Azathioprine. I am not one of the literature
cases. The first and only flu vaccination I received was in November 2011.
1. Hillesheim PB, Parker
JR, Parker JC, Jr., Escott E, Berger JR. Chronic lymphocytic inflammation with
pontine perivascular enhancement responsive to steroids following influenza
vaccination. Arch Pathol Lab Med. 2012;136(6):681-5. doi:
10.5858/arpa.2011-0428-CR.
2. Pittock
SJ, Debruyne J, Krecke KN, Giannini C, van den Ameele J, De Herdt V, et al.
Chronic lymphocytic inflammation with pontine perivascular enhancement
responsive to steroids (CLIPPERS). Brain. 2010;133:2626-34. doi:
10.1093/brain/awq164.
Comments for the Author
The author does make a valid point in that it cannot be
assumed that influenza vaccination directly leads to CLIPPERS; however, I do
not believe that Hillesheim et al are implying an absolute cause and effect.
They are merely reporting a case of a patient who developed CLIPPERS after
receiving the vaccination. With this being such a rare disease with no definitive
undeniable cause/trigger or mechanism only associations can be made between the
disease and its possible causes, which is what the article is doing, similar to
the other reports of CLIPPERS in the literature, until enough cases are
reported to find investigable commonalities.
My Response
I still believe Dr Hillesheim over-emphasises the
vaccination aspect in his original paper but accept this is a matter of
opinion. However as noted in my letter, his statement that CLIPPERS "is
another complication ..." (of influenza vaccination) "... which
should be added to this list" surely goes too far; however the reviewer
disagrees and so I must defer.
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.
