12 catchy neurology headlines to cast out a year of infamy

2020 was characterised by horror of epidemic proportions. Spawned in 2019, COVID did not attain plague status until 2020. Unfurling its envelope, and baring its spikes, it struck with ruthless efficiency. But the chilling terror it unleashed was not enough to freeze the ink of neurologists. With flowing prose and radiant captions, neurological penmanship flourished in defiance of SARS-CoV-2. So, as we say goodbye to a year we can’t wait to forget, let us take a moment to marvel at the outstanding neurology titles of the last 12 months. Here then are 12 catchy neurology headlines to cast out a year of infamy.

CC BY-SA 4.0, Link

What is the place of magnesium in the treatment of migraine?

Magnesium is a rather understated metal which however plays such a significant role in health. This should not be surprising as it is the eighth most common metal in the Earth’s crust, and the fourth most abundant mineral in the human body. Explaining why magnesium is so central to health, Uwe Gröber and colleagues, in their paper titled Magnesium in prevention and therapy, point out that this underrated element is a cofactor in more than 300 enzyme systems which regulate such diverse biochemical reactions ranging from protein synthesis to neuromuscular transmission.

By Maral10Own work, Public Domain, Link

With such an important physiological function, it is alarming that the body can very easily run out of magnesium. But this is exactly what Gröber and colleagues demonstrated in their paper, which was incidentally published in the journal, Nutrients, in 2015; they showed that magnesium deficiency can result from a myriad of medical disorders such as alcoholism, malabsorption, endocrine disorders, chronic kidney diseases, and dialysis, or from the use of drugs such as antibiotics, chemotherapeutic agents, diuretics, and proton pump inhibitors.

CC BY 3.0, Link

Because of its diverse and important role, magnesium deficiency doesn’t lie down quietly in the corner and mope; rather it screams out in many tongues. Low magnesium therefore presents with symptoms such as lethargy, vomiting, fatigue, cramps, tremor, carpopedal spasm, tetany, seizures, and cardiac arrhythmias. Even more astonishing is the list of disorders that may be triggered by magnesium deficiency, from asthma, diabetes, hypertension, and osteoporosis, to stroke, attention deficit hyperactivity disorder (ADHD), Alzheimer’s disease, of all things, and of course migraine. Obstetricians will of course remind us of the indispensability of magnesium for eclampsia.

By LeiemOwn work, CC BY-SA 4.0, Link

With the foregoing in the background, it is easy to understand why researchers have thoroughly investigated the possible place of magnesium in the treatment of migraine. Exploring its prophylactic role, for example, Hsiao-Yean Chiu and colleagues touted the virtues of magnesium in their paper titled Effects of intravenous and oral magnesium on reducing migraine: a meta-analysis of randomized controlled trials. Publishing in the journal Pain Physician in 2016, the authors reviewed 10 key studies, with a combined number of 789 subjects, which assessed the ability of magnesium to prevent migraine, and they concluded that “oral magnesium significantly alleviated the frequency and intensity of migraine“. The authors felt confident enough in their findings to recommend oral magnesium as a part of a “multimodal approach to reduce migraine”.

By KulmalukkoOwn work, CC BY-SA 3.0, Link

Even more authoritative about the role of magnesium in migraine prophylaxis is the conclusion of the systematic review published in the journal Headache in 2017 titled Magnesium in migraine prophylaxis-is there an evidence-based rationale? a systematic review. The authors, Alexander von Luckner and Franz Riederer, found grade C, or possibly effective, evidence in support of the preventative role of magnesium in migraine. Going further, Charly Gaul and colleagues, publishing in the Journal of Headache and Pain in 2015, reported that adding riboflavin and coenzyme Q10 significantly increased the beneficial effect of magnesium.

Magnesium. fdecomite on Flickr. https://www.flickr.com/photos/fdecomite/6257573610

There is however a dampener to the celebrity status of magnesium in the migraine prophylaxis saga:  some reports simply found insufficient evidence for it. One such paper, published in the journal Cephalalgia in 2014 is titled An evidence-based review of oral magnesium supplementation in the preventive treatment of migraine. The authors, Levi Teigen and Christopher Boesy, reviewed 16 relevant studies and concluded that “the strength of evidence supporting oral magnesium supplementation is limited at this time“. But even then, they appreciate that absence of evidence is not the evidence of absence. They therefore did not dismiss the potential benefit of magnesium in migraine, and had no objection to migraineurs supplementing their dietary magnesium intake. As this paper was published in 2014, a lot has clearly passed under the bridge since then.

By Ben MillsOwn work, Public Domain, Link
Magnesium in the acute treatment of migraine has also been under scrutiny, and one such searchlight was shone by Hsiao-Yean Chiu and colleagues in their paper cited above. After reviewing 11 relevant studies comprising 948 subjects, they found that “intravenous magnesium significantly relieved acute migraine“. It is reassuring that two older papers also came to the same conclusion; the first, by ME Bigal and colleagues, was published in the journal Cephalalgia in 2002, and the second, by Şeref Demirkaya and colleagues, is reported in the journal Headache in 2004. Both papers revealed that 1000mg of magnesium sulfate intravenously was effective in aborting acute migraine attacks, especially if the attacks are associated with auras. Furthermore, writing in the journal Clinical Neurology and Neurosurgery in 2019, Fanny Xu and colleagues found that magnesium is effective even in status migrainosus, the most pernicious form of acute migraine.
By LoethlinOwn work, CC BY-SA 4.0, Link

But, as you guessed, the verdict on the benefit of magnesium in acute migraine is far from unanimous. For example, Y Cete and colleagues, publishing their case series of emergency department patients in the journal Cephalagia in 2005, reported that magnesium is no better than placebo for acute migraine. Furthermore, Hyun Choi and Nandita Parmar in their meta-analysis, published in the European Journal of Emergency Medicine in 2014, said intravenous magnesium “failed to demonstrate a beneficial effect” in acute migraine. Arpad Pardutz and Laszlo Vecsei, commenting in the Journal of Neural Transmission in 2012, even discouraged the use of magnesium because there are more effective treatment options.

By Pixelmaniac pictures (Leave a reply) – Own work, CC0, Link

Why are there such conflicting conclusions about the value of magnesium in acute migraine? One answer may lie in the almost prehistoric observation by Alexander Mauksop and colleagues; writing way back in 1996, in the journal Headache, they suggested that only a subset of migraine sufferers are susceptible to low magnesium levels. The authors go further to argue that low magnesium may be a trigger, not just for migraine, but for tension type headaches and cluster headaches; they therefore recommended that magnesium levels should be assessed in patients presenting with significant headaches, whatever the cause.

By 2×910Own work, CC BY-SA 4.0, Link

In conclusion, the evidence for the use of oral magnesium in migraine prophylaxis justifies its clinical use. The evidence for the use of intravenous magnesium for acute migraine is however less clear-cut, and future studies may help to clarify the ambiguity. In the meantime, it may be worth checking magnesium levels when a migraine attack defies conventional treatment: a top-up might just make the difference. And for the researchers, it may be time to look more closely at precision migraine medicine – it might just help to define those migraine sufferers who will benefit from that magic shot of magnesium.

By Warut RoonguthaiOwn work, CC BY-SA 3.0, Link

The emerging influential role of microglia in neurology

By GerryShawOwn work, CC BY-SA 3.0, Link

The most important clinical fallout of dysfunctional microglia appears to be the emergence of dementia. It is indeed speculated that microglia may hold the key to stopping the notorious Alzheimer’s disease (AD). This is because microglia seem to play a role in eliminating the amyloid plaques which are thought to contribute to the disease process. Experiments suggest that there is excessive microglial activation in AD, and these supercharged microglia destructively eat up’ synapses, the all-important junctions where nerve cells communicate with each other. It is also relevant that microglial activation is particularly prominent in the hippocampus, a structure critical for memory formation. Because synaptic loss is such a key feature of AD, it is hoped that a better understanding of microglial function may lead to therapeutic tools that modulate AD microglial activation.

Microglial cells and photoreceptors. NIH Image Gallery on Flickr. https://www.flickr.com/photos/nihgov/46571706425

Microglial activation also seems to play a role in another prominent neurodegenerative disease, Parkinson’s disease (PD). It is also speculated that microglia are activated in PD as a response to environmental triggers, and the activated microglia cause neuronal damage by producing toxic substances. Because this is presumably an inflammatory process, there is the hope that a better understanding of the process will open up new therapeutic possibilities.

Microglia. NIH Image Gallery on FLickr. https://www.flickr.com/photos/nihgov/42301918151

Another disorder in which microglia may play a pathogenetic role is frontotemporal dementia (FTD) in which chronic microglial activation has been reported. It is significant that the microglial activation is most evident in the frontal cortex as this correlates with the behavioural and speech disorders which characterise FTD. More intriguingly, the activated microglia seem to express the progranulin (PGRN) gene mutations that are known to be associated with FTD. Enough clues one might say.

By Mary AntipovaOwn work, CC BY 4.0, Link

The reach of microglial dysfunction however goes way beyond the big three of AD, PD, and FTD. For example, microglia are acutely activated in traumatic brain injury (TBI), and this may be responsible for the damage that results from this. Microglia also appear to be relevant in cerebrovascular disorders because microglial activation has been reported in ischaemic stroke and in haemorrhagic stroke. And the cherry on top is surely the report that microglia play a role in prion disorders. It may well turn out that neuroscientists are just opening up the microglial can of worms.

Abraçada de microglia Patricia Bogdanov-Cristina Sola-Joel Sampedro- Marta Valeri. Vall d’Hebron Institut de Recerca VHIR on Flickr. https://www.flickr.com/photos/vhir/31615774702

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Want to find out more on microglia? You may want to explore these links:

Review: Microglia in motor neuron disease

Motor cortex transcriptome reveals microglial key events in amyotrophic lateral sclerosis

25 non-eponymous neurological disorders… and the names behind them

Medicine is as much defined by diseases as by the people who named them. Neurology particularly has a proud history of eponymous disorders which I discussed in my other neurology blog, Neurochecklists Updates, with the title 45 neurological disorders with unusual EPONYMS in neurochecklists. In many cases, it is a no brainer that Benjamin Duchenne described Duchenne muscular dystrophy, Charle’s Bell is linked to Bell’s palsy, Guido Werdnig and Johann Hoffmann have Werdnig-Hoffmann disease named after them. Similarly, Sergei Korsakoff described Korsakoff’s psychosis, Adolf Wellenberg defined Wellenberg’s syndrome, and it is Augusta Dejerine Klumpke who discerned Klumpke’s paralysis. The same applies to neurological clinical signs, with Moritz Romberg and Romberg’s sign, Henreich Rinne and Rinne’s test, Joseph Babinski and Babinski sign, and Joseph Brudzinski with Brudzinki’s sign.

Yes, it could become rather tiresome. But not when it comes to diseases which, for some reason, never had any names attached to them. Whilst we can celebrate Huntington, Alzheimer, Parkinson, and Friedreich, who defined narcolepsy and delirium tremens? This blog is therefore a chance to celebrate the lesser known history of neurology, and to inject some fairness into the name game. Here then are 25 non-eponymous neurological diseases and the people who discovered, fully described, or named them.

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Amyotrophic lateral sclerosis (ALS)

Jean-Martin Charcot

Készítette: Unidentified photographerhttp://resource.nlm.nih.gov/101425121, Közkincs, Hivatkozás

Aphantasia

Francis Galton (and Adam Zeman)

By Eveleen Myers (née Tennant) – http://www.npg.org.uk/collections/search/portrait/mw127193, Public Domain, Link

Chronic inflammatory demyelinating polyneuropathy (CIDP)

Peter J Dyck

By Dr. Jana – http://docjana.com/#/saltatory ; https://www.patreon.com/posts/4374048, CC BY 4.0, Link

Corticobasal degeneration (CBD)

WRG Gibb, PJ Luthert, C David Marsden

https://upload.wikimedia.org/wikipedia/commons/c/cd/ProteineTau.jpg

Epilepsy

Hippocrates

Hippocrates. Eden, Janine and Jim on Flickr. https://www.flickr.com/photos/edenpictures/8278213840

Essential tremor

Pietro Burresi

By UndescribedOwn work, CC BY-SA 4.0, Link

Frontotemporal dementia (FTD)

Arnold Pick

By Unknown authorhttp://www.uic.edu/depts/mcne/founders/page0073.html, Public Domain, Link

Inclusion body myositis (IBM)

E J Yunis and F J Samaha

CC BY-SA 3.0, Link

Meningitis

Vladimir Kernig and Jozef Brudzinski

By A. F. Dressler – Festschrift zum 70. Geburtstag Dr. Woldemar Kernig’s: Von Verehrern und Schülern herausgegeben als Festnummer der St. Petersburger medicinischen Wochenschrift St. Petersburger medizinische Wochenschrift, Bd. 35, Nr. 45. (1910), Public Domain, Link

Migraine

Aretaeus of Cappadocia

By Cesaree01Own work, CC BY-SA 4.0, Link

Multiple sclerosis (MS)

Jean-Martin Charcot

Journal.pone.0057573.g005http://www.plosone.org/article/info:doi/10.1371/journal.pone.0057573#pone-0057573-g005. Licensed under CC BY 2.5 via Wikimedia Commons.

Multiple system atrophy (MSA)

Milton Shy and Glen Drager

By Kenneth J. Nichols,Brandon Chen, Maria B. Tomas, and Christopher J. Palestro – Kenneth J. Nichols et al. 2018. Interpreting 123I–ioflupane dopamine transporter scans using hybrid scores., CC BY 4.0, Link

Myasthenia gravis (MG)

Samuel Wilks

By Unknown authorhttp://ihm.nlm.nih.gov/images/B25782, Public Domain, Link 

Myotonic dystrophy

Hans Gustav Wilhelm Steinert

By Unknown author – reprinted in [1], Public Domain, Link 

Neurofibromatosis

Friedreich Daniel von Recklighausen

By Unknown authorIHM, Public Domain, Link 

Narcolepsy

Jean-Baptiste-Edouard Gélineau

https://upload.wikimedia.org/wikipedia/commons/7/74/Jean_Baptiste_Edouard_G%C3%A9lineau.jpg

Poliomyelitis

Michael Underwood

By Manuel Almagro RivasOwn work, CC BY-SA 4.0, Link

Progressive supranuclear palsy (PSP)

John Steele, John Richardson, and Jerzy Olszewski

By Dr Laughlin Dawes – radpod.org, CC BY 3.0, Link

Restless legs syndrome (RLS)

Karl Axel Ekbom

By Peter McDermott, CC BY-SA 2.0, Link

Stiff person syndrome (SPS)

Frederick Moersch and Henry Woltmann

By PecatumOwn work, CC BY-SA 4.0, Link

Synesthesia

Georg Sachs and Gustav Feschner

Synaesthesia. aka Tman on Flickr. https://www.flickr.com/photos/rundwolf/7001467111/

Stroke

Hippocrates

By editShazia Mirza and Sankalp GokhaleSee also source article for additional image creators. – editShazia Mirza and Sankalp Gokhale (2016-07-25). Neuroimaging in Acute Stroke.Attribution 4.0 International (CC BY 4.0), CC BY 4.0, Link

Tabes dorsalis

Moritz Romberg

By https://wellcomeimages.org/indexplus/obf_images/39/1d/edecf5a530781f5c10603a50fa35.jpghttps://wellcomecollection.org/works/gctr3stg CC-BY-4.0, CC BY 4.0, Link

Trigeminal neuralgia

John Fothergill

By Gilbert Stuarthttp://www.pafa.org/Museum/The-Collection-Greenfield-American-Art-Resource/Tour-the-Collection/Category/Collection-Detail/985/mkey–1923/, Public Domain, Link

Tuberous sclerosis

Désiré-Magloire Bourneville

By Unknown author – Bibliothèque Interuniversitaire de Médecine – http://www.bium.univ-paris5.fr/images/banque/zoom/CIPB0452.jpg, Public Domain, Link

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Reunion of neurologists at the Salpêtrière hospital. Photograph, 1926 https://commons.wikimedia.org/w/index.php?curid=36322408

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Let us then celebrate the pioneers…

Eponymous and anonymous alike

The case for testing serum neurofilament light protein in MS

I am yet to request serum neurofilament light protein (NfL) in my practice. I am not sure yet why I should, but until now I confess I really haven’t looked for a reason to do so. I however know that some MSologists now tick it, along with other blood tests, when they investigate people they suspect may have multiple sclerosis (MS). NfL are proteins that are released by damaged neurones. Should I be requesting NfL in my clinical practice? I sniffed around to find the case for testing serum NfL, and below is what I found.

By GerryShaw – Standard tissue culture and immunofluorescencePreviously published: Unpublished, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=26518273

Many studies have looked at the value of NfL in MS. One such very well-planned study that addresses many of my questions is that by Guili Disanto and colleagues, published in the journal Annals of Neurology in 2017. In the paper, titled Serum Neurofilament light: a biomarker of neuronal damage in multiple sclerosis, the authors studied >380 people with MS and >150 healthy controls, and report four important findings.

By GerryShaw – Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=17500647
  1. The levels of NfL in serum strongly correlate with the levels in cerebrospinal fluid (CSF) of people with MS.
  2. People with more active and more severe MS had higher levels of NfL.
  3. People with MS on disease modifying treatment (DMT) had lower NfL levels than those who were not on treatment.
  4. In people with MS who had their serum NfL tested serially over time, the level of NfL predicted those who will develop frequent relapses or progressive MS.

The authors concluded, with enough justification I think, that serum NfL is a “sensitive and clinically meaningful blood biomarker to monitor tissue damage and the effects of therapies in MS“.

Culture rat hippocampal neuron. ZEISS Microscopy on Flickr. https://www.flickr.com/photos/zeissmicro/24327909026

The strong correlation between cerebrospinal fluid (CSF) and serum NfL was also confirmed by a study published in the journal Neurology, by Lenka Novakova and colleagues titled Monitoring disease activity in multiple sclerosis using serum neurofilament light protein. As the title indicates, they discovered that serum NfL is as good as CSF NfL in monitoring the progression of MS.

Neuron. NICHD on Flickr. https://www.flickr.com/photos/nichd/21086076575

The observation that NfL predicts the course of MS is supported by many other studies, such as the one by Kristin Varhaug and colleagues in the journal Neurology Neuroimmunology and  Neuroinflammation whose title is also self-explanatory: Neurofilament light chain predicts disease activity in relapsing-remitting MS. A more recent paper, also published in Neurology, further reinforces the benefit of serum NfL in disease course prediction. It is titled Blood neurofilament light chain as a biomarker of MS disease activity and treatment response. In this paper, Jehns Kuhle and colleagues practically confirm all the above stated benefits of NfL, concluding that “our results support the utility of blood NfL as an easily accessible biomarker of disease evolution and treatment response”.

“Neuron” by Roxy Paine. Christopher Neugebauer on Flickr. https://www.flickr.com/photos/chrisjrn/4745660322

As for long term outcome, the 10 year follow up study by Alok Bahn and colleagues, published in the Multiple Sclerosis Journal in 2018, is most informative. In their paper titled Neurofilaments and 10-year follow-up in multiple sclerosis, the authors noted that “CSF levels of NfL at the time of diagnosis seems to be an early predictive biomarker of long-term clinical outcome and conversion from RRMS to SPMS”. Further support for the long term prognostic value of serum NfL comes from a paper published in 2018 in the journal Brain titled Serum neurofilament as a predictor of disease worsening and brain and spinal cord atrophy in multiple sclerosis. The authors, Christian Barro and colleagues, studied more than 250 people with MS and concluded that “the higher the serum neurofilament light chain percentile level, the more pronounced was future brain and cervical spinal volume loss“.

Nervous Tissue: Spinal Cord Motor Neuron. Berkshire Community College on Flickr. https://www.flickr.com/photos/146824358@N03/41850849912/in/album-72157666241437517/

It is pertinent to note that the MS sphere is not the only one in which NfL is making waves. It has been found to be elevated in many other disorders such as motor neurone disease (MND), multiple system atrophy (MSA), hereditary spastic paraplegia (HSP), stroke, active small vessel disease, and peripheral neuropathy (PN). With these disclaimers in place, it may just be time to start ticking that NfL box.

 

By GerryShaw – Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=17502311

 

What is the last word on migraine and PFOs?

This is surely one of neurology’s bug bears, the old chestnut. Just when one generation of neurologists thinks it has buried and sealed it in an impervious crypt, it resurrects to haunt the next breed. This cyclical and macabre dance of migraine and PFOs evokes a sense of deja vu every time it comes around. And each spawn of neurologists predictably picks up the gauntlet, answers the call to arms, and sets out to slaughter the ghost of migraines past.

By Lille1982 – Own work, Public Domain, https://commons.wikimedia.org/w/index.php?curid=9682382

But let’s take a step back to basics with some definitions. The foramen ovale is just a ‘hole in the heart‘ between the right and left atria, or upper heart chambers. It is essential in foetal life because it enables circulating blood to bypass the superfluous foetal lungs (apologies to readers across the Atlantic for the superfluous ‘o’!). However, after birth, when blood needs to circulate through the now indispensable lungs, the foramen ovale becomes irrelevant. In most people, the foramen ovale humbly accepts its fate, crawls to a corner, and closes shop. But foramen ovales in some people are recalcitrant; standing their ground, they endure and survive as PFOs.

By DrJanaOfficial – Own work, CC BY-SA 4.0, https://commons.wikimedia.org/w/index.php?curid=50477765

It is not clear how the myth started, but stories of migraine cure following surgical closure of PFO spread rapidly and widely. Very soon, migraineurs where demanding echocardiograms to check if they have PFOs lurking in their tickers. Research trials investigating this issue started as a trickle, and very soon become a flood. But rather than shed light, the conflicting results intensified the darkness. That is, until some indignant and determined neurologists and cardiologists set out to settle the matter once and for all. And the onslaught came in three waves of studies.

MIGRAINE. aka Tman on Flickr. https://www.flickr.com/photos/rundwolf/331545021

The first wave in the attempt to slay the beast of migraine and PFO was a trial published in the journal Headache. Davinia Larrosa and colleagues studied 183 people with migraine and found that, whilst PFOs were larger and more permanent in people with migraine, there was no relationship between patent foramen ovale and migraine frequency.

Migraine spectrum. JoanDragonfly on Flickr. https://www.flickr.com/photos/joandragonfly/26221136058

The second wave was a study published in the European Heart Journal by Heinrich Mattle and colleagues. In their PRIMA trial (Percutaneous Closure of PFO in Migraine with Aura), they blindly allocated or randomized half of their subjects with refractory migraine and PFO to have PFO closure surgery. And their verdict was, PFO closure did nothing to reduce the frequency of migraine.

Open the key to your heart. Maria Eklind on Flickr. https://www.flickr.com/photos/mariaeklind/24659701809

The third wave, launched by Nauman Tariq and colleagues, took a different tack. They carried out a detailed review of practically all the studies that had addressed the subject. Their brilliantly titled paper, “Patent foramen ovale and migraine: closing the debate, reflected their ambition to settle the question once and for all. After an arduous trawl through the literature, and a mind-blowing crunching of statistics, the authors came to the conclusions that “there is no good quality evidence to support a link between migraine and PFO“, and “closure of PFO for migraine prevention does not significantly reduce the intensity and severity of migraine“.

By Patrick J. Lynch, medical illustrator – Patrick J. Lynch, medical illustrator, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=1488277

The final word therefore is, leave PFOs alone! But there is a big caveat. There is one situation where PFO is associated with migraine, and that is when it is accompanied by another heart anomaly called an atrial septal aneurysm (ASA). The evidence for this comes from Roel Snijder and colleagues, and the title of their paper says it all: Patent foramen ovale with atrial septal aneurysm is strongly associated with migraine with aura: a large observational study. We already knew that PFO associated with ASA increases the risk of stroke; we can now add to this, the risk of migraine with aura.

By H. Airy – Flatau 1912 “Migrena” monograph after previous publication of H. Airy, Public Domain, https://commons.wikimedia.org/w/index.php?curid=7814450

The debate is now hopefully sealed and settled. But don’t hold your breath for too long: the phantom of migraine and PFO may just rear its hideous head again at a neuroscience centre near you.

 

15 more creative and catchy neurology headlines for 2019

Regular visitors to this blog know that we love catchy article titles. It is always heartwarming to see how some authors create imaginative and inventive headlines. This skill involves the ability to play with words, and the capacity to be double-edged. This is why this blog keeps a lookout for fascinating neurology titles. And in line with this tradition, and in no particular order of inventiveness, here are 15 more catchy neurology titles!

By Andrikkos – Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=33725735

15. Who do they think we are? Public perceptions of psychiatrists and psychologists

This paper, for some unfathomable reason, set out to ask if the public knows the difference between what psychiatrists and psychologists actually do. And the authors discovered that “there is a lack of clarity in the public mind about our roles”. More worryingly, or reassuringly (depending on your perspective), they also found out that “psychologists were perceived as friendlier and having a better rapport“. Not earth-shattering discoveries, but what a great title!

By Laurens van Lieshout – Own work, Public Domain, https://commons.wikimedia.org/w/index.php?curid=2059674

14. OCT as a window to the MS brain: the view becomes slightly clearer

Optical coherence tomography (OCT) is a cool tool which measures the thickness of the retinal fiber layer (RFL). And it has the habit of popping its head up in many neurological specialties. In this case, the specialty is multiple sclerosis, and the subject is how OCT influences its diagnosis and surveillance. Surely a window into the brain is easier to achieve than one into the soul.

Optical coherence tomography of my retina. Brewbooks on Flickr. https://www.flickr.com/photos/brewbooks/8463332137

13. A little man of some importance 

The homonculus is the grotesque representation of the body on the surface or cortex of the brain. This paper reviews how formidable neurosurgeons such as Wilder Penfield worked out the disproportionate dimensions of this diminutive but influential man. He (always a man for some reason) has giant hands, a super-sized mouth, very small legs, and a miniature trunk. The clever brain doesn’t readily allocate its resources to large body parts that perform no complex functions! But be warned, this article is no light-weight reading!

The Homunculus in Crystal Palace (Moncton). Mark Blevis on Flickr. https://www.flickr.com/photos/electricsky/1298772544

12. Brain-focussed ultrasound: what’s the “FUS” all about? 

This title is a play on words around MR-guided focussed ultrasound surgery (MRgFUS), an emerging technique for treating disorders such as essential tremor and Parkinson’s disease (PD). This review looks at the controversial fuss that this technique has evoked.

By Luis Lima89989 – Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=19162929

11. The Masks of Identities: Who’s Who? Delusional Misidentification Syndromes

This paper explores the interesting subject of delusional misidentification syndromes (DMSs). The authors argue that few concepts in psychiatry can be as confusing as DMSs. And they did an excellent job of clearing our befuddlement around delusions such as Capgras and Fregoli. Very apt title, very interesting read.

no identity. HaPe-Gera on Flickr. https://www.flickr.com/photos/hape_gera/2929195528

 

10. Waking up to sleeping sickness.

This title belongs to a review of trypanosomiasis, aka sleeping sickness. It is a superb play on words, one that evokes several levels of meaning. It is simple and yet complex at the same time. Great imagination.

https://picryl.com/media/the-sleeping-sickness-gordon-ross

09. Brains and Brawn: Toxoplasma Infections of the Central Nervous System and Skeletal Muscle

This paper discusses two parts of nervous system that are affected by toxoplasmosis. Playing on the symbolic  contradiction between intellect and strength, the authors show how toxoplasmosis is an ecumenical abuser: it metes out the same fate to both brain and brawn.

Brain vs. Brawn. Yau Hoong Tang on Flickr. https://www.flickr.com/photos/tangyauhoong/4474921735

08. Shedding light on photophobia

A slightly paradoxical title this one. Ponder on it just a little more! And then explore the excellent paper shedding light on a condition that is averse to light.

Photophobia (light sensitivity). Joana Roja on Flickr. https://www.flickr.com/photos/cats_mom/2772386028/

07. No laughing matter: subacute degeneration of the spinal cord due to nitrous oxide inhalation

Nitrous oxide, or laughing gas, is now “the seventh most commonly used recreational drug”. But those who pop it do so oblivious of the risk of subacute combined degeneration. This damage to the upper spinal cord results from nitrous oxide-induced depletion of Vitamin B1 (thiamine). Not a laughing matter at all!

Empty Laughing Gas Canisters. Promo Cymru on Flickr. https://www.flickr.com/photos/promocymru/18957223365

06. To scan or not to scan: DaT is the question

Dopamine transport (DaT) scan is a useful brain imaging tests that helps to support the diagnosis of Parkinson’s disease and other disorders which disrupt the dopamine pathways in the brain. It is particularly helpful in ruling out mimics of Parkinson’s disease such as essential tremor. When to request a DaT scan is however a tricky question in practice. This paper, with its Shakespearean twist, looks at the reliability of DaT scans.

Dopamine. John Lester on Flickr. https://www.flickr.com/photos/pathfinderlinden/211882099

05. TauBI or not TauBI: what was the question?

It should be no surprise if Shakespeare rears his head more than once in this blog post. Not when the wordsmith is such a veritable source of inspiration for those struggling to invent catchy titles. This paper looks at taupathy, a neurodegeneration as tragic as Hamlet. It particularly comments on an unusual taupathy, one induced by traumatic brain injury. Curious.

By Lafayette Photo, London – This image is available from the United States Library of Congress‘s Prints and Photographs divisionunder the digital ID cph.3g06529.This tag does not indicate the copyright status of the attached work. A normal copyright tag is still required. See Commons:Licensing for more information., Public Domain, Link

04. Mind the Brain: Stroke Risk in Young Adults With Coarctation of the Aorta

What better way to call attention to a serious complication than a catchy title like this one. This paper highlights the neurological complications of coarctation of the aorta, a serious congenital cardiovascular disease. And the key concerns here are the risks of stroke and cerebral aneurysms. Cardiologists, mind the brain!

Own work assumed (based on copyright claims)., Public Domain, https://commons.wikimedia.org/w/index.php?curid=803943

03. Diabetes and Parkinson disease: a sweet spot?

This paper reviews the unexpected biochemical links between diabetes and Parkinson’s disease. And this relationship is assuming a rather large dimension. Why, for example, are there so many insulin receptors in the power house of Parkinson’s disease, the substantia nigra? A sweet curiosity.

Insulin bubble. Sprogz on Flickr. https://www.flickr.com/photos/sprogz/5606839532

02. PFO closure for secondary stroke prevention: is the discussion closed?

The foraman ovale is a physiological hole-in-the-heart which should close up once a baby is born. A patent foramen ovale (PFO) results when this hole refuses to shut up. PFOs enable leg clots to traverse the heart and cause strokes in the brain. This paper reviews the evidence that surgically closing PFOs prevents stroke. Common sense says it should, but science demands proof. And the authors assert that they have it all nicely tied up. Hmmm.

By Kjetil Lenes – Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=3705964

01. Closure of patent foramen ovale in “cryptogenic” stroke: Has the story come to an end?

Not to be beaten in the catchy title race is another brilliant PFO review article. Why do I feel the answer here is ‘no’? This is science after all.

https://www.flickr.com/photos/fliegender/293340835

 

A few more catchy neurology article titles to start the year

The Neurology Lounge is addicted to journal articles whose titles show that a lot of thought and attention went into constructing them. I have reviewed some of these in my previous blog posts titled The Art of Spinning Catchy Titles, and The Art of Spinning Catchy Neurology Headlines. To keep the tradition alive, here are a few more recent catchy titles.

Journal Entry. Joel Montes de Oca on Flikr. https://www.flickr.com/photos/joelmontes/4762384399

Stoop to conquer: preventing stroke and dementia together

This comes from an editorial in Lancet Neurology urging a joint approach to preventing stroke and dementia, a strategy the author calls ‘the lowest hanging fruit in the fight against these two greatest threats to the brain’. He argues that ‘at the moment, the fruit might be hanging too low for our gaze, and we are wrongly fixated on the distant future of Alzheimer’s disease treatment. We might have to stoop to conquer‘.

By Gavarni – Le voleur, n°95, 27 août 1858, page 265. Reproduction d’une gravure extraite des Toquades de Paul Gavarni, éditées par Gabriel de Gonet, Paris 1858., Public Domain, Link

Romberg’s test no longer stands up

This opinion piece in Practical Neurology takes a stab at the age-old neurological test of sensory impairment. Subject are asked to stand up and try to maintain their balance with their eyes shut. The author asserts that this, the Romberg’s test, ‘lacks essential specificity’, ‘risks physical injury’, and is ‘redundant’. He argues that there are much better, and safer, ways of testing for sensory ataxia. There goes an interesting test!

By Mikhail KonininFlickr: Meerkat / At the zoo / Novosibirsk / Siberia / 24.07.2012, CC BY 2.0, Link

Dacrystic seizures: a cry for help

This is from a case report of a 69-year old man in the journal Neurology. He presented with unusual crying spells which turned out to be dacrystic (crying) seizures. This case is eventually revealed to be a case of….sorry, no spoilers. Click on the link to find out.

HeartBroken-Tears are the Baptism of the Soul. Anil Kumar on Flikr. https://www.flickr.com/photos/87128018@N00/139136870

Game of TOR -the target of rapamycin rules four kingdoms

I am no fan of Game of Thrones, but it is an in-your-face television series which provides the setting for this catchy title. The mechanistic target of rapamycin (mTOR) pathway is underlies the pathology of tuberous sclerosis. It is therefore the target of many therapeutic strategies in the form of mTOR inhibitors. And the 4 kingdoms? You have to read the piece from the New England Journal of Medicine…perhaps after you have watched the TV series!

Stack. Wendy on Flikr. https://www.flickr.com/photos/wenzday01/4332780839

Restless legs syndrome: losing sleep over the placebo response

This editorial, also from Neurology, addresses the disturbing report in the same journal warning of the high placebo response of interventions for restless legs syndrome (RLS). The title couldn’t be more apt. 

By Edvard Munch – The Athenaeum: pic, Public Domain, Link

 


…and some not very catchy titles

Unfortunately many neurology titles are not as catchy as the ones above. Many article titles appear to be half-baked and fall short. Here are a few:

And the prize for the silliest title in neurology must go to this paper in the Journal of Neural Transmission that is simply…unreadable!

Do statins really increase the risk of Parkinson’s disease?

Statins are famous, and their fame lies in their ability to bust cholesterol, the villain in many medical disorders such as heart attack (myocardial infarction) and stroke. Some may add that statins are infamous, and this is partly because of their side effects such as muscle pain. Love them or hate them, we can’t get away from statins…even as the debate rages about their benefits and downsides.

By ChiltepinsterOwn work, CC BY-SA 3.0, Link

It is not surprising therefore that the statin debate will filter into neurology. The sticking point here however has nothing to do with cholesterol busting, but all to do with whether statins increase or reduce the risk of developing Parkinson’s disease (PD). Strange as it may seem, statins and PD have a long history. And a positive one generally, I hasten to add. There is a large body of evidence to suggest a protective effect of statins on PD as reflected in the following studies:

  1. Confounding of the association between statins and Parkinson disease: systematic review and meta-analysis 
  2. Statin therapy prevents the onset of Parkinson disease in patients with diabetes
  3. Statin use and risk of Parkinson’s disease: A meta-analysis 
  4. Statin use and its association with essential tremor and Parkinson’s disease
  5. Statin use and the risk of Parkinson’s disease: an updated meta-analysis
  6. Long-term statin use and the risk of Parkinson’s disease
  7. Discontinuation of statin therapy associated with Parkinson’s disease
Modeling the Molecular Basis of Parkinson’s Disease. Argonne National Laboratory on Flikr https://www.flickr.com/photos/argonne/4192798573

It was therefore with some consternation that a recent study, published in the journal Movement Disorders, really put the cat among the pigeons. The paper is titled:

Statins may facilitate Parkinson’s disease: insight gained from a large, national claims database,

The authors of this paper set out to investigate ‘the controversy surrounding the role of statins in Parkinson’s disease’. In this retrospective analysis of over 2,000 people with PD, and a similar number of control subjects, the authors found that statins significantly increased the risk of developing PD. This is clearly a conclusion looking for a fight!

By Col. Albert S. Evans – internet archives, Public Domain, Link

I must admit I was totally unaware there was any controversy about statins and PD. I was therefore curious to find out what studies are out there fuelling it. Which other trials have bucked the trend and reported an increased risk of PD from statins? And where best to find the answers but in PubMed, the repository of all human knowledge! And I found that there were only a few studies that did not report a protective effect of statins on PD, and these studies concluded, quite reasonably, that they found no relationship between PD and statins. Here are a few of the studies:

  1. Statin adherence and the risk of Parkinson’s disease: A population-based cohort study. 
  2. Use of statins and the risk of Parkinson’s disease: a retrospective case-control study in the UK. 
  3. Statin use and the risk of Parkinson disease: a nested case control study. 

These papers reporting the absence of evidence seem happy to engage in an amicable debate to resolve the question.

By DavidKF1949Own work, CC BY-SA 3.0, Link

One study however stood out like a sore thumb because it positively reported a negative effect of statins on PD (try and work that out!). This 2015 study, also published in Movement Disorders, is titled Statins, plasma cholesterol, and risk of Parkinson’s disease: a prospective study. The paper concludes that “statin use may be associated with a higher PD risk, whereas higher total cholesterol may be associated with lower risk“. Not only are the authors arguing that statins are bad for PD, they are also suggesting that cholesterol is good! This is a paper that was itching for fisticuffs.

By Jan SteenWeb Gallery of Art:   Image  Info about artwork, Public Domain, Link

What is a jobbing neurologist to do? What are the millions of people on statins to do? Whilst awaiting further studies, I will say stay put. Go with the bulk of the evidence! And keep track of The Simvastatin Trial, funded by The Cure Parkinson’s Trust. This trial is looking at the benefit of statins in slowing down PD. And surely, very soon, the science will lead to a resolution of the argument-all you need to do is keep track of everything PD in Neurochecklists.

By Léon Augustin Lhermittehttp://wellcomeimages.org/indexplus/obf_images/fc/7f/643258ab30237374aaea5ac15757.jpgGallery: http://wellcomeimages.org/indexplus/image/L0006244.html, CC BY 4.0, Link

 

Primary angiitis of the CNS: unusual presentations of a rare and dangerous disorder

Primary angiitis of the central nervous system (PACNS) is inflammation of the blood vessels of the central nervous system (stating the obvious you might say). It differs from other forms of angiitis or vasculitis, such as lupus and giant cell arteritis (GCA), which respect no boundaries. PACNS is as dangerous a neurological disorder as they come, and just as rare. It requires aggressive, and paradoxically equally life-threatening, immunosuppressive treatment. Between the devil and deep blue sea-that’s exactly where the neurologist managing a patient with PACNS will be found.

BRAINADE! the Brain Grenade. Emilio Garcia on Flikr. https://www.flickr.com/photos/lapolab/11929014084
BRAINADE! the Brain Grenade. Emilio Garcia on Flikr. https://www.flickr.com/photos/lapolab/11929014084

The clinical features of PACNS are unfortunately very non-specific and include headaches, seizures, stroke, and cognitive changes. This makes PACNS is a challenge to diagnose. Even when suspected, PACNS may evade detection even by the special scan of the blood vessels called angiography. More frequently, the only certain way of confirming this disease in life is with a brain biopsy. Did I say ‘certain’? I take that back. Alas, even brain biopsy is not guaranteed to make the diagnosis of PACNS. A high degree of confidence and teeth-gritting is therefore an absolute requirement in any neurologist unfortunate enough to come face-to-face with this menace.

By The original uploader was Glitzy queen00 at English Wikipedia - Transferred from en.wikipedia to Commons., CC BY-SA 3.0, Link
By The original uploader was Glitzy queen00 at English Wikipedia – Transferred from en.wikipedia to Commons., CC BY-SA 3.0, Link

To make complicated matters even worse for the unwary neurologist, there are now reports suggesting that PACNS presents in even rarer and atypical ways. For the neurological Sherlocks and Poirots, here are 2 unusual presentations of PACNS.

Isolated spinal cord involvement

Spinal Cord 2. Green Flames 09 on Flikr. https://www.flickr.com/photos/greenflames09/116396742
Spinal Cord 2. Green Flames 09 on Flikr. https://www.flickr.com/photos/greenflames09/116396742

This is a case report from the Journal of Neurology of a 44-year old woman who presented with PACNS but with purely spinal cord involvement and completely sparing the brain. The diagnosis in this case was only confirmed with a spinal cord biopsy. The authors reviewed the literature and only found 8 previous reports of PACNS beginning in the spinal cord, and half of these progressed to involve the brain. 

Unilateral cerebral presentation

Keep Left. Howard Lake on Flikr. https://www.flickr.com/photos/howardlake/4440588147
Keep Left. Howard Lake on Flikr. https://www.flickr.com/photos/howardlake/4440588147

Most cases of PACNS evenly involve both sides of the brain. This report, again from Journal of Neurology, bucks this trend with the report of a 55-year old man who had PACNS which only involved the left side of his brain. This unilateral hemispheric PACNS is a reminder that an entity called focal PACNS exists.

https://pixabay.com/en/light-bulb-brain-absorbed-light-1599359/
https://pixabay.com/en/light-bulb-brain-absorbed-light-1599359/

Do you have any sightings of unusual cases of PACNS? Please drop a comment

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