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Archive for March, 2014

Variants of Guillain–Barré syndrome.

31 Mar, 14 | by Steve Vucic, Web Editor

Guillain-Barre syndrome (GBS) is a rare, but much taught, autoimmune disorder of the peripheral nerves.  Importantly, the variant of GBS are poorly recognized in the Western world, thereby leading to diagnostic and therapeutic delay.  In the superb review by Wakerley and Yuki, pharyngeal-cervical-brachial (PCB) variant of GBS is expertly discussed.  This must read review will tell you all you need to know about the PCB variant of GBS.

Pharyngeal-cervical-brachial variant of Guillain-Barre syndrome.  J Neurol Neurosurg Psychiatry. 2014 Mar;85(3):339-44. doi: 10.1136/jnnp-2013-305397. Epub 2013 Jun 26.

MUST READ ALERT!

 

 

Vitamin D and multiple sclerosis: what’s going on?

31 Mar, 14 | by Arun Krishnan, Web Editor

Most neurologists know very little general medicine. OK, minor correction- I no longer know very much general medicine and it is arrogant for me to assume that all my colleagues are the same (although I am pretty sure that they are…). Anyway, the one minor detail regarding endocrinology that I had managed to tuck away deep in some unsuspecting neurones of my hippocampus was that vitamin D was good for your bones. I tell most of my elderly patients and the ‘senior’ female members of my family that there is nothing better than drenching yourself in 15 minutes of the Australian sun every day. In my overly simplistic mind, this will mean that you are less likely to break a bone.

When I was at medical school, I did not think that vitamin D would have much to do with Neurology or more specifically with this wonderful material called myelin. Recently however, there has been a plethora of publications from all corners of the world that have explored the interaction between Vitamin D and multiple sclerosis risk. In this issue of JNNP, Lin et al (1) from Tasmania, Australia provide another contribution from their large ongoing study of the role of environmental factors in the development of MS. In short, their study suggests that there are genetic influences which may modify the effects of Vitamin D and its subsequent role in determining clinical activity in MS.

This is a very interesting paper but until randomised evidence is available, the question still remains: what are we to do in recommending vitamin D supplementation? There are so many things to remember when you are counselling MS patients that this is something that may be forgotten. So, exactly how important do you think it is? In most countries in the world, it is near impossible to maintain normal vitamin D levels all year around. Should all MS patients be on replacement? Do we repeat levels periodically and should we boost supplementation further if we are not hitting the high normal range? A patient of mine the other day had a MS relapse after many years of excellent control on disease-modifying drugs. She asked me if we could check her vitamin D level. It was low and she has put the relapse down to this. I told her that there was no evidence for thinking this way. Her response: ” then why on earth did you ask me to take vitamin D supplements”? I think she has a point…

I would love to hear from other neurologists out there. What exactly do you do and more importantly, why are you doing it?

(1)    Lin et al., Novel modulating effects of PKC family genes on the relationship between serum vitamin D and relapse in multiple sclerosis. J Neurol Neurosurg Psychiatry 2014;85:399-404

Are the sphingosine wars beginning?: Ponesimod-Promising new oral agent for RRMS

29 Mar, 14 | by Steve Vucic, Web Editor

Another oral agent has shown promise in a phase IIb trial for relapsing-remitting MS.  Ponesimod, a selective sphingosine 1-Phosphate Receptor 1 Agonist, reduced disease burden and activity as assessed by MRI and clinical measures (annualized relapse rates).   Safety and tolerability  were also assessed and proven to be good.   Larger trials are required to confirm the therapeutic utility of ponesimod in RRMS,  although choice is always good.  Read more in JNNP:

 

Olsson T, Boster A, Fernández O, Freedman MS, Pozzilli C, Bach D, Berkani O, Mueller MS, Sidorenko T, Radue EW, Melanson M. Oral ponesimod in relapsing-remitting multiple sclerosis: a randomised phase II trial. J Neurol Neurosurg Psychiatry. 2014 Mar 21. doi: 10.1136/jnnp-2013-307282.

 

 

Ponesimod structure- Are the sphingosine wars beginning?

Is one born to develop motor neuron disease?

28 Mar, 14 | by Steve Vucic, Web Editor

Motor neuron disease is fatal and rapidly progressive neurodegenerative disorder of the human motor system.  The disorder peaks in the late 50’s early 60’s and then declines after ager 80, an unusual statistic for a degenerative disease.   In addition, familial forms of MND are increasing recognised, although the penetrance of the genotype is at best 80% by age 80.  Further, the phenotype seems to be identical between different genotypes, at least in terms of the motor dysfunction, and “anticipation” has been reported.  Importantly, most of the genetic mutations seem to disrupt vital cellular function such as DNA/RNA metabolism.  So an important question to address is “why does it take 50 or 60 years for the disease to manifest in mutation carriers?”

In the must read review by Eisen and colleagues, the notion of a long pre-clinical period is raised with the possibility that ALS susceptibility is acquired in utero.  Exposure to environmental and other factors may then trigger the devastating process.  Such a construct has been suggested in other neurodegenerative diseases.  Development of robust techniques to detect pre-clinical dysfunction, if any, could be the magic therapeutic bullet. 

 Eisen A, Kiernan M, Mitsumoto H, Swash M.  Amyotrophic lateral sclerosis: a long preclinical period?  J Neurol Neurosurg Psychiatry. 2014 Mar 19. doi: 10.1136/jnnp-2013-307135.

Thoughts on facebook please!

To fly or not to fly doctor?

27 Mar, 14 | by Steve Vucic, Web Editor

The vexing question often posed of an unspepecting neurolgist is whether patients with a recent stroke, or risk of strokes, should fly.  The answer is obvious, being “only with a safe airline:))”.  However, all humour aside, this can be a difficult magaement questions.  In this must read issue of JNNP, Reynolds and colleagues grapple with this issue.  Subjects from the Carotid Occlusion Surgery Study (COSS) that travelled for a PET study to a regionla center were recruited.  While just over a thirs of the subjects exhibited ipsilateral cerebral ischemia, thankfully no events were reported (TIAs or atrokes).  So, I gues the answer must be Bon Voyage, but perhaps with an antiplatelet agent.

 

                     PLUS                      

 

                                                                                                      APPEARS TO BE OK

 

Reynolds MR, Kamath AA, Grubb RL, Powers WJ, Adams HP, Derdeyn CP; Carotid Occlusion Surgery Study Investigators.  The safety of aeroplane travel in patients with symptomatic carotid occlusion.  J Neurol Neurosurg Psychiatry. 2014 Apr;85(4):435-7. doi: 10.1136/jnnp-2013-306627. Epub 2013 Nov 18.

 

Another nail in the preverbial coffin for CCSVI theory?

26 Mar, 14 | by Steve Vucic, Web Editor

Chronic cerebrospinal venous insufficiency (CCSVI) was proposed as a potential pathogenic mechanism in MS.  While pathological data to support such a theory were elusive, a world-wide craze was sett-off, mostly amongst patients, some vascular physicians and some (minority) of neurologist.  It seamed appealing that a simple vascular problem was responsible for MS, but alas subsequent studies have refuted any meaningful link.  Unfortunately, a great deal of invaluable resources had been committed in disproving/proving  the CCSVI theory and the spin-off therapy.

 

In this issue of JNNP, van Zuuren and colleagues conducted a thorough and detailed analysis of studies assessing the effects of percutaneous transluminal angioplasty in MS, the so called liberation procedure.  Unfortunately no firm answer was provided either way, thereby prolonging the uncertainty and calling for more studies.

Is this liberating/helpful or not?? What do you think?

 

I suspect not.

Steve Vucic

Latest from JNNP

Latest from JNNP