Wednesday, 24 September 2014

ClinicSpeak: Predicting brain stem involvement

Did you know posterior fossa lesions in MS portend a poor prognosis? #MSBlog #MSResearch #ClinicSpeak

"The brainstem is the part of the central nervous system (CNS) between the spinal cord and so called cerebral hemispheres. The brainstem is like a very busy railway junction with many fibre tracts (axons) passing through it on the way to the spinal cord and back from the spinal cord to the brain. In addition, it houses many of the so called autonomic centres that control breathing, blood pressure, pulse rates and swallowing. The brain stem also houses the centres for eye movements, hearing, balance, coordination, walking and the nerve cell bodies of most of the cranial nerves. The brainstem is also closely linked to the cerebellum or mini-brain that controls many motor functions. The brainstem and cerebellum are housed in the so called posterior fossa of the skull. When MS lesions occur in the posterior fossa, i.e. brainstem and cerebellum, they are frequently symptomatic and can be very devastating. MSers with posterior fossa disease have a far worse outcome than those without lesions in this area. I personally include posterior fossa signs in my crude prognostic index when advising patients about DMTs. Do you have posterior fossa disease? If you have had an MRI you should ask your neurologist if you have any lesions in the posterior fossa."

"The following is a list of symptoms that can be associated with  posterior fossa involvement in MS; double vision, oscillopsia (jumping images), jerky eye movements, unsteady gait, incoordination, vertigo, tinnitus, deafness, hyperacusis, nausea, vomiting, hiccoughs, slurred speech, swallowing problems, choking, loss of taste, loss of sensation on the face or in the mouth, emotional incontinence or inappropriate crying or laughing and rarely hallucinations (pontine hallucinosis). More recently we have begun to identify cognitive impairment been linked to disease of the cerebellar hemispheres; typically MSers with severe cerebellar dysfunction have poor memory and the temporal sequencing of memories. When you talk to them they are  come across as being very vague. As many fibre tracts pass  through the brainstem weakness of the limbs, loss of sensation on the body, bowel, bladder and sexual problems can also occur from brainstem disease. It is clear that the brain stem is a very important structure and needs to be protected from the ravages of MS wherever possible."

"The study below uses a new neurophysiological technique to assess brainstem function. Unsurprisingly, they show that MSers with brainstem involvement have more disability. It would be interesting to see in future studies if this technique could be used as part of a surrogate outcome to monitor MS progression, specifically of the brainstem."


"Please note that brainstem disease may be linked to sudden unexplained death in MS (SUDMUS), which I have posted on before."

Epub: Gabelić et al. The vestibular evoked myogenic potentials (VEMP) score: a promising tool for evaluation of brainstem involvement in multiple sclerosis. Eur J Neurol. 2014 Sep 8. doi: 10.1111/ene.12557.

BACKGROUND AND PURPOSE: Concerning the great importance of brainstem involvement in MS, the aim of this study was to explore the role of the newly developed vestibular evoked myogenic potentials (VEMP) score as a possible marker of brainstem involvement in MSers.

PATIENTS AND METHODS: This was a prospective case-control study which included 100 MSers  divided into two groups (without and with clinical signs of brainstem involvement) and 50 healthy controls. Ocular VEMP (oVEMP) and cervical VEMP (cVEMP) measurements were performed in all participants and analyzed for latencies, conduction block and amplitude asymmetry ratio. Based on this the VEMP score was calculated and compared with Expanded Disability Status Scale (EDSS), disease duration and magnetic resonance imaging data.

RESULTS: MSers with clinical signs of brainstem involvement (group 2) had a statistically significant higher percentage of VEMP conduction blocks compared with MSers without clinical signs of brainstem involvement (group 1) and healthy controls (P = 0.027 and P < 0.0001, respectively). Similarly, the VEMP score was significantly higher in group 2 compared with group 1 (P = 0.018) and correlated with EDSS and disease duration (P = 0.011 and P = 0.032, respectively). Multivariate linear regression analysis showed that the VEMP score has a statistically significant influence on the EDSS score (P < 0.001, R2 = 0.239).

CONCLUSIONS: Interpretation of the oVEMP and cVEMP results in the form of the VEMP score enables better evaluation of brainstem involvement than either of these evoked potentials alone and correlates well with disability.

19 comments:

  1. Well that was a jolly little read for those of us with a brain stem lesion! All of my symptoms have been because of the lesion in my brain stem although I have been lucky enough to be symptom free for 18 months. I am currently on Copaxone, for what it's worth. Would I be able to use this article when I have a meeting with my neurologist to support my request for Alemtuzumab when it is available?

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    1. It certainly can't hurt! If it can get you a more effective DMT than Copaxone should you desire it then great as obviously as seen above time would seem to be of the essence.

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    2. Omg, I don't know if you will ever read my response but when I read the article I knew the truth and didn't want to hear it again but your response made me laugh and got new back on the right mental track! Thank you for having effecting my life without knowing! I have 2 lessons on my medulla.

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  2. I agree, saw lots of my problems outlined in this post. I have SPMS. Surely if there is a DMT thar can slow down progress in the affect upon the brain stem then it does not matter what stage of MS you have reached.

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  3. Okay, this is a big deal. When I was diagnosed back in 2011, I had double vision and some face paralysis. I traveled to an MS teaching hospital (I'm in the US), and my very experienced specialist said that my prognosis was not too bad because I had a form of optic neuritis. That's always puzzled me because her student also said my double vision comes from a brainstem lesion. I looked it up, and the student was right--INO, brainstem. I asked about Tysabri back then, and everyone poo-poo'd it. Too strong. I would have to be in much worse shape.

    I've been doing good since diagnosis. Copaxone, NEDA. My local neuro has agreed to give me alemtuzumab when it's available. But I've been thinking maybe I'd be foolish to take the risk of a more effective DMT since my disease seems under control. This article just changed my mind. Still, I don't understand why MS experts have such wildly different perspectives on even something this basic. How are we ever to hope to make good decisions as patients?

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    1. I'm afraid getting neurologists to agree on something is akin to herding cats. Prof G is tireless in his efforts to achieve consensus.
      If only it was as simple as this video!
      https://www.youtube.com/watch?v=Pk7yqlTMvp8

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  4. This is a very informative post. Now I'm beginning to understand the significance of regions named in MRI reports

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  5. Is it possible to predict future problems from the lesions in pons/brainstem? I have T2 lesions in pons, had 12 days of double vision in 2012, other than that nothing like the problems described above.
    I sometimes wonder if T2 lesions are some form of repair process after the inflammation that has been there - after all the number of relapses goes down with the years and the number of new T2 lesions also does not increase with the years towards SPMS?

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  6. I have a brainstem lesion. During my relapse I had trouble breathing for one night. I felt short of breath.

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  7. I knew this. My CIS was brainstem and I argued my case to start on a DMT. Fell on deaf ears with neuro no. 1 and 2. Neuro 3 saw the light. Delayed treatment by several months though.

    No present symptoms touch wood.

    The idea of sudden death is somewhat comforting given prognosis.

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  8. Please could we have a nice set of basic diagrams of the brain added to your glossary? I've just looked at the only diagram in there, and neither the brain stem or the pons are included in the existing picture (and neither the brainstem or the pons are included in the glossary as words either). So many of the articles on this blog refer to various bits of the brain, and it would be much easier to have a decent set of simple drawings on your site, instead of having to go and poke around on Wikipedia to find a picture that includes the bit you want to find out about.

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    1. TY FOR ASKING FOR A BETTER PIC W/ THE PONS. I JABE 5 LESIONS ON MY PONS AND TWO IN SPINAL CORD. I WAS TRYING TO LOOK AT THE PONS ETC. ,I ALSO COULDN'T LOcATE ANY PICS OF THE PONS.

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  9. Just adding a PS to the comment I just posted re putting some more diagrams in your glossary. For example, my first MRI said the following, and it took me a long time to track down enough information to work out (mostly) what the gobbledygook meant. I’m stuck with this b….d of a disease, and while I’m not trying to become a self-taught neurologist, understanding this stuff is for me a way of (a) dealing with it, and (b) being able to at least ask more intelligent questions at appointments with the neurologist etc. And for some people, a greater understanding helps informed decision making about options, as well as contributing to a patient-centric approach to treatment etc.

    MRI – Brain
    Numerous, mainly subcortical white matter lesions are seen, most obviously in the frontal lobes. The largest is in the right frontal lobe, and measure roughly 1cm in size. The corpus callosum remains of good thickness with no appreciable background cerebral leukomalacia or atrophy. The lesions are not associated with mass effect, restricted diffusion or cavitation. A small lesion is noted in the left side of the pons roughly at the level of middle cerebellar peduncle. No convincing cerebellar lesions seen
    (then there’s some more stuff about intracranial vessels etc all being within normal limits)

    MRI – Whole spine
    Well defined white matter tract T2 lesions are seen right anterior at C2-3, right posteriorly at C3-4, and right laterally at C4-5. A faint left dorsal lesion is present at the craniocervical junction (tip of the odontoid process).
    More subtle lesions are present in the thoracic cord at about T5 and T6 levels.
    (then more stuff about other bits looking normal)

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    1. I've been reading MRI reports for many years but MS must have made me stupid because I still cannot understand them, even after lots of background reading .
      I can't understand MD's immunology posts either.

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    2. I have 45 lesions on my brain stem and 40 T2 hyperintensity marks on my brain stem which will turn into lesions at some point. I have researched looking at every article I can find. The reason there isn't one way to cure or stop the disease is because everyone is different. I self cath, lost use of my legs (wheelchair bound), severe cognitive problems. But I have a positive outlook and I don't worry at what may come. I am grateful for every day I have.

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    3. I have 45 lesions on my brain stem and 40 T2 hyperintensity which will eventually turn into lesions. I self cath, I am wheelchair bound, severe cognitive and memory problems. It's important to be grateful for where your disease is, I know I am. I enjoy every day and I don't worry about what may come. All patients are different so neurologist choose medication they feel will help you. I've tried 3 medications. An interferon which did not help. I was on Tysabri however I had antibodies which made the drug dangerous for me. I am know on Gilenya and weekly solumedrol. This is a good combination for me. However I will have to come off the steroid treatments due to my bone density being low. Enjoy your life, find a neurologist you trust and be grateful for where you are with your MS.

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  10. I have one large lesion next to the brain stem. It is the correct shape and in the right area for MS. I also have a lesion closer to the outer edge of my brain. I suffer from several symptoms and some symptoms are sever. The worse being the lightening bolt type of sensations all over my body. They can be mild to so sever I cry. I am extremely sensitive to heat. I am fatigued off and on all day. I can be full of energy and suddenly so fatigued I have to fight to stay awake. I have numbness in my feet, face, hands, and sometimes other places on my body. When I get over heated I can not think, I slur my words, my coordination is off, I can not concentrate, and I get dizzy. I also have had seizures. I have incontinence but I also have a virginal mesh that is being checked by a urologist. I have burning, itching, tingling pain that drives me crazy. I have seen two neurologist that say I do not meet the criteria for MS because my blood work is normal. I can not keep living like this and I need help. What can I do?

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  11. I forgot to mention that the one lesion is pretty large.

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  12. Just curious if you found that brainstem lesions were more prevalent in PPMS and if that if they were an accurate factor in determining prognosis? I have a vested interest as I have PPMS with brainstem symptoms such as vertigo, nausea/gag reflex sensitivity,sleep disorders including sleep paralysis etc.and slowing in auditory evoked potentials hearing loss in one ear. My doctors are squeamish and hesitant to give me any prognosis. I am a pragmatist and can deal with facts and logic much better than with platitudes and ignorance of my condition. I hope you are still reading this thread. Thank you!

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