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.
This just happens to be the first ever COVID-related catchy title we will feature in this blog, and we surely hope it will be the last! It is however appropriate that we begin our 2020 run down with a COVID title, and our first choice is apt, literally and metaphorically. Published in the journal Headache, the paper is a brilliant review of the different headaches which follow in the trail of COVID 19, complete with prevalence, pathophysiology, and clinical characteristics. It is also full to the brim with scary facts, such as up to 10% of people with COVID19 will first manifest with a headache, and, more worryingly, we should expect some COVID headaches to linger long after the pandemic is over. Ominous!
Linking weight and obesity in a title doesn’t sound like creativity, but add evidence to the mix and what you see is inventiveness. The authors of this editorial, published in Multiple Sclerosis Journal argue that for a dreadful disease such as multiple sclerosis (MS), where the real cause remains elusive, it is critical to explore all its potentially modifiable risk factors and, well, weigh the evidence for them. The editorial was commenting on a study that did exactly this for obesity and found that a few pounds here, and a few inches there, do increase the odds of developing MS. Ingeniously, their marker for MS was atrophy of the prepapillary retinal nerve fibre layer thickness as measured during optical coherence tomography (OCT). And the evidence for this appears to be, well, somewhat weighty.
A cautionary title if ever there was one. This paper, full title being Cavernous malformations with DVA: hold those knives, is an editorial published in the journal Neurology. The authors were commenting on a paper in the same journal which investigated the risk of bleeding from cavernomas when they are associated with developmental venous anomalies (DVAs). The editorial is very enlightening, pointing out that DVAs are only associated with sporadic, and not with familial, cavernomas, and a whopping 25% of sporadic cavernomas are associated with DVAs. Even more surprising was the study finding that the risk of bleeding from a cavernoma is reduced if the cavernoma is associated with a DVA. Quite counterintuitive, but then this is the brain we are talking about. So the next time your eager-beaver neurosurgeon embarks on a knife-edge mission to chop out a cavernoma, flag this paper, and tell them to sheathe their scalpels.
This paper in the journal Epilepsia is all about seizures that occur in the setting of autoimmune encephalitis. ‘Fire’ and ‘kindling’ are entirely appropriate in this regard when one considers that autoimmune encephalitis has been symbolically depicted as Brain on Fire. The paper, full title Seizures in autoimmune encephalitis: kindling the fire, reviews the complex pathogenic mechanisms by which autoimmune encephalitis develops. And the picture the authors paint is of pyromaniac antibodies igniting neuro-inflammatory flames and stoking hyper-excitability blazes, one consequence of which is recalcitrant epilepsy. The question for neuroscience is therefore clear: how do we quench this inferno?
That is the full title, honest. And this is as short and catchy as a title can possibly be. Published in the journal Practical Neurology, the piece is not about how to detect fasciculations, but about how the etymology of the word ‘fasciculation‘ tells us that many clinicians are wielding it inappropriately. And this all stems from a tenuous battle of ideas that is raging in Newcastle neurology because some fussy older neurologists become very ‘twitchy’ when their presumably younger colleagues misuse and abuse the word. It appears, for example, that it is sacrilege to use the word in a plural form. In a detailed analysis of the word, the authors traced its fitful and flickering history, making allusions to, amongst other things, fascism! You will be very nervy at the end of the short article, but what a brilliant title!
The yellow brick road, in case you need reminding, is a fictional path in the famous children’s novel The Wonderful Wizard of Oz. If, like me, you never actually ever read the book, you would surely have watched one or the other incarnation of its screen version; there is therefore no excuse not to be familiar with the really oddball characters created by L. Frank Baum (I bet you thought it was Lewis Carroll!). I digress. The authors of our catchy headline, clearly fans of Baum, got their inspiration from his classical farcical adventure, from which they extracted differential diagnostic lessons to help neurologists distinguish between Alzheimer’s disease (AD) and frontotemporal dementia (FTD). The paper, full title Diagnosing the frontal variant of Alzheimer’s disease: a clinician’s yellow brick road, is a report of three patients who presented with typical features of behavioural variant FTD (bvFTD), but eventually turned out to be suffering from the frontal variant AD (fvAD). The authors characterise their differential diagnostic clues as the yellow brick road, an allusion to the road that leads to success in Oz. They metaphorically refer to bvAD as “the irritable, paranoid, and tremulous Scarecrow“, and bvFTD as “the heartless, ritualistic, and rigid Tin Man“. As for Lion, he probably lost his way on the yellow brick road…surely a metaphor for some other cognitive disorder.
The full title of this short article in the journal Neurology is Intracranial extramedullary hematopoiesis: blood disorders on the mind. The authors presented the case of a woman whose clinical presentation and striking brain MRI features are typical of a primary brain tumour…but for the fact that she has an underlying complex blood disorder: polycythemia vera and myelodysplastic/myeloproliferative overlap. The histology of her dural lesions revealed extramedullary hematopoiesis (EMH). You must mind the blood…even if you are in the brain!
This really insightful editorial, also from the journal Neurology, is a commentary on a paper in the same issue which explored the relationship between apathy and impulse control disorders (ICDs) in people with Parkinson’s disease (PD). Admitting that apathy is more a psychiatric than neurological concept, the editorial makes the strong case that neurologists need to shake off their indifference for the word that evokes disinterest. The editorial underscored the key lesson from the paper in question, that as contrasting as their manifestations are, apathy and ICDs are travelling companions in PD. And what strange bedfellows they make, a veritable parkinsonian Jekyll and Hyde! (Let’s not get started on another classic of literature).
If Agatha Christie had written a neurological murder mystery, this might well have been the title. Imagine a genetic mutation which alters the conformation of a chromosome from linear to circular, thereby unleashing indescribable havoc. That, in a nutshell, is ring 14 syndrome. But for the neurologist, it spells a trigger for drug-resistant epilepsy and a host of congenital anomalies. The intriguing paper that explores this is published in the journal Epilepsia, and its full title is Chromosome 14 deletions, rings, and epilepsy genes: a riddle wrapped in a mystery inside an enigma. The authors, just like Miss Marple or Hercule Poirot would have done, set out to solve the enigma of how the deletion could produce such a remarkable chromosomal metamorphosis, and why this should turn out to be so devastating. The only spoiler…the striking quotation is adapted from Winston Churchill, who was, in turn, referring to Russia. The plot thickens, you might say.
I admit that it doesn’t take genius to see the obvious link between the small vessel disease CADASIL, and its causative mutation on the NOTCH3 gene. But ‘NOTCHing‘ is what clinched it for me as a truly catchy title. This paper is an editorial, again in the journal Neurology, which reviews the studies that are steadily increasing our understanding of a disease that manifests with no less than three major neurological disorders: migraine, stroke, and dementia. The author was specifically commenting on a paper in the same journal which studied the diverse manifestations of ‘cysteine-altering NOTCH3 variants‘. What is remarkable is the range of manifestations of these variants, from an overwhelming burden of small vessel disease, to no clinical features whatsoever. We clearly need to do more than notch towards understanding…we need some speed!
I have always wondered when James Bond would burst into a neurology headline, and when he eventually did, it was was characteristic flair in the Annals of the Indian Academy of Neurology. The full title of the paper, Shaken not stirred: a pilot study testing a gyroscopic spoon stabilization device in Parkinson’s disease and tremor, is rather long-winded, but the question at its heart was quite straight-forward – to test the effectiveness of devices that claim to relieve the tremor of Parkinson’s disease. And the device the skeptical authors assessed is a gyroscopic stabilisation device (me too, never heard of it). Expectedly perhaps, the authors discovered that this “tremor cancellation technology” failed to meet up to its advertised billing; the shaken patients were not stirred one bit by the gyroscope: on the contrary, some of the patients even shook more vigorously after the device was switched on. Talk of licence to shake! Ian Fleming must be turning under his pillar.
As we come to the last goodbye to 2020, it is important that we make a triumphant exit, and this victorious editorial in the Lancet Neurology perfectly fits the bill. The author of the editorial was commenting on the truly ambitious, and aptly titled, CONQUER trial, which ticked all the boxes to make Archie Cochrane smile from his grave: it was a “multicentre, randomised, double-blind, placebo-controlled, phase 3b” study which was carried out over 64 sites across 12 countries. The CONQUERERS examined the effectiveness of Galcanezumab, a calcitonin gene related peptide (CGRP) monoclonal antibody (mab), in the prevention of migraine. And the editorialist concluded that Galcanezumab, along with the related Erenumab and Fremanezumab, “are safe, tolerable, and efficacious in many patients with frequent migraines, even in those with several non-successful preventive treatment attempts”. The emergence of the CGRP mabs is surely one bright spot in what has been a dark year. So let us bid farewell to 2020 on a positive note…and welcome 2021 with good cheer and virus-free expectations!