The Neurology Lounge

August 8, 2020 Ibrahim Imam GENERAL

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, Jules 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.

***

Amyotrophic lateral sclerosis (ALS)

Jean-Martin Charcot

Készítette: Unidentified photographer – http://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

Essential tremor

Pietro Burresi

By Undescribed – Own work, CC BY-SA 4.0, Link

Frontotemporal dementia (FTD)

Arnold Pick

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

Inclusion body myositis (IBM)

E J Yunis and F J Samaha

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 Cesaree01 – Own work, CC BY-SA 4.0, Link

Multiple sclerosis (MS)

Jean-Martin Charcot

“Journal.pone.0057573.g005” http://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 author – http://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 author – IHM, 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 Rivas – Own 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

Stiff person syndrome (SPS)

Frederick Moersch and Henry Woltmann

By Pecatum – Own 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.jpg https://wellcomecollection.org/works/gctr3stg CC-BY-4.0, CC BY 4.0, Link

Trigeminal neuralgia

John Fothergill

By Gilbert Stuart – http://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

***

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

***

Let us then celebrate the pioneers…

Eponymous and anonymous alike

August 3, 2020 Ibrahim Imam GENERAL

How does CSF analysis distinguish MS from neurosarcoidosis?

Distinguishing neurosarcoidosis from multiple sclerosis based on CSF analysis: A retrospective cohort study. Arun T, Pattison L, Palace J. Neurology 2020 (online ahead of print) Abstract Objective: To characterize a cohort of patients with neurosarcoidosis with particular focus on CSF analysis and to investigate whether CSF values could help in distinguishing it from multiple sclerosis […]

via How does CSF analysis distinguish MS from neurosarcoidosis? — Neurochecklists Blog

July 25, 2020 Ibrahim Imam GENERAL

Is thrombolysis beneficial when time of stroke onset is unknown?

Efficacy and safety of thrombolytic therapy for stroke with unknown time of onset: a meta-analysis of observational studies. Zhu RL, Xu J, Xie CJ, Hu Y, Wang K. J Stroke Cerebrovasc Dis 2020 (Epub ahead of print). Abstract BACKGROUND: Recombinant tissue plasminogen activator (rt-PA) is one of the most effective therapies available for patients with […]

via Is thrombolysis beneficial when time of stroke onset is unknown? — Neurochecklists Blog

July 21, 2020 Ibrahim Imam GENERAL

Does breastfeeding influence the risk of postpartum MS relapses?

Association between breastfeeding and postpartum multiple sclerosis relapses: a systematic review and meta-analysis. Krysko KM, Rutatangwa A, Graves J, Lazar A, Waubant E. JAMA Neurol 2019 (Epub ahead of print). Abstract BACKGROUND: Multiple sclerosis (MS) relapses may be increased in the postpartum period, and whether breastfeeding is associated with reduction in the risk of postpartum […]

via Does breastfeeding influence the risk of postpartum MS relapses? — Neurochecklists Blog

July 20, 2020 Ibrahim Imam GENERAL

Patient H.M.

Patient H. M. Author: Luke Dittrich Synopsis This is a unique and even controversial biography of a person the author described as ‘the most studied individual in the history of neuroscience’ (page 8). Patient H.M., now known to be Henry Molaison, perhaps more than any other patient, helped to delineate how the brain functions. What […]

via Patient H.M. — The Doctors Bookshelf

July 18, 2020 Ibrahim Imam CATCHY ARTICLE TITLES

10 more catchy titles from the recent neurology literature

The art of spinning catchy titles

The art of spinning catchy neurology headlines

A few more catchy neurology article titles to start the year

15 more creative and catchy neurology headlines for 2019

A few more catchy titles from the world of neurology

Going forward then, here are 10 more catchy titles from the recent neurology literature-almost all from the last 12 months.

***

A new hose to extinguish the FIRES?

This is the title of a 2019 editorial in the journal Epilepsy Currents, and FIRES here refers to febrile infection related epilepsy syndrome (FIRES). FIRES is itself a form of new onset refractory status epilepticus (NORSE) in which there is associated fever. NORSE and FIRES are notoriously resistant to treatment, and the editorial points out the abject failures of steroids, IVIg, and plasma exchange, and the limited benefit of ketogenic diet and anakinra. This is where the hose comes in: it is the interleukin‐6 receptor inhibitor tocilizumab; the original research paper, published in the journal Annals of Neurology in 2018, reported that seven patients treated with Tocilizumab had very good outcomes. Take home messages: perhaps Tocilizumab is the right fire-extinguisher for this blazing inferno, and there must be an acronym-generating machine in the epilepsy universe!

Aircraft rescue firefighters battle blazes, sustain skills. DVIDSHUB on Flickr. https://www.flickr.com/photos/dvids/6521127091

Do good things come to those who wait?

The full title of this paper is “Dosing interval of natalizumab in MS: do good things come to those who wait?” It comes from a 2019 issue of the journal Neurology, and it is itself an editorial on a paper in the same journal which touted the benefits of extended dosing interval of natalizumab in multiple sclerosis. That paper showed that the risk of progressive multifocal leukoencephalopathy (PML) can be reduced by reducing the frequency of Natalizumab infusions. The authors used a 6 weekly, rather than the conventional 4 weekly, dosing interval of Natalizumab, and their gamble had paid off; they reported a “remarkable” risk reduction of developing PML by 88-99%. Enough justification for a catchy title, and proof that procrastination pays off…sometimes.

Faces. Jeanne Menjoulet on Flickr. https://www.flickr.com/photos/jmenj/11669577265

Don’t fear the reefer

The full title of this paper is “Don’t fear the reefer-evidence mounts for plant-based cannabidiol as treatment for epilepsy”. Published in Epilepsy Currents in 2019, it set out to reassure clinicians that cannabis is OK…sort of. The paper reports the findings of a double-blind study of 225 people with Lennox-Gastaut syndrome (LGS), the severe epilepsy disorder which manifests with dangerous drop attacks. The study subjects were randomised to receive one of two two doses of cannabidiol, and their response was compared to a matched control group who were treated with placebo. The good news is that both doses of cannabidiol produced “greater reductions in the frequency of drop seizures than placebo”. Dispel the fear…and embrace the reefer!

Cannabis sativa (marijuana plants). James St. John on Flickr. https://www.flickr.com/photos/jsjgeology/19815465305https://www.flickr.com/photos/jsjgeology/19815465305

This paper has nothing to do with why you can’t get that musical note out of your head, as revealed by the full title which is “Can’t get it off my brain: meta-analysis of neuroimaging studies on perseverative cognition”. But do not panic, at least not yet: perseverative cognition, it turns out, is just a fancy term for worrying or rumination. Published in the journal Psychiatry Research: Neuroimaging in 2020, the paper is a meta-analysis of 43 imaging studies of pathological worriers, those of us who have “intrusive, uncontrollable, repetitive thoughts”. To cut to the chase, and to avoid going round in circles, the centre of our rumination is a part of the brain called the anterior cingulate cortex (ACC). This structure, the authors said, is “critical for the pathological expression of rumination and worry”. You can now panic: you have the ACC to add to your list of things to worry about!

ARTZYBASHEFF, Boris. Anxiety-Frustration-Repressed Hostility, 1947. Halloween HJB https://www.flickr.com/photos/halloweenhjb/49801864797

Visual snow: are we beginning to see the light?

Visual snow syndrome is a relatively new thing for neurologists who are very much in the dark about it. From being a manifestation of migraine, it has climbed up the slippery disease classification ladder and is now a full-fledged syndrome of its own. It is therefore time for neurologists to see the light of visual snow, and what better way to show it than by such a catchy title. The paper, published in the journal Neurology in 2019, calls attention to this enigmatic phenomenon by providing a detailed review of the syndrome. But is the light bright enough for the neurologist?

https://upload.wikimedia.org/wikipedia/commons/f/f1/Visual_snow3.gif

An unexpected twist

Things that go twist in neurology are almost always dystonic, and this paper is no exception. Published in the journal Neurology Clinical Practice in 2019, the full title of the paper is “Sternocleidomastoid muscle hypertrophy in cervical dystonia: an unexpected twist”. It is a rather convoluted tale of a patient with Parkinson’s disease (PD) who developed cervical dystonia 10 days after he was treated with quetiapine. However, his sternocleidomastoid muscle was massively enlarged or hypertrophied, an impossible thing to happen within 10 days. You are beginning to get the twist. With their curiosity piqued, and their Sherlock Holmes hat on, the authors discovered that the patient has a previous history of dystonia, and the hypertrophy is really not as new as it first appeared. One surely for Agatha Christie fans, a tale with many unexpected twists. But the catchy title belies an important lesson- Occam’s razor doesn’t always cut sharply.

By Wuerdelos – Own work, CC BY-SA 4.0, Link

Multiple sclerosis enters a grey area

The idea that multiple sclerosis (MS), the quintessential white matter brain disease, is also a grey matter disease, is a bit of a shock to the traditional neurologist. The grave implication is that MS, the ultimate neuroinflammatory disease, is also a neurodegenerative disorder. This paradigm shift has all to do with the much better imaging tools available which show the grey matter changes in MS. And this editorial, published in the journal Nature in 2019, sums up the situation aptly, and catchily. The paper on which the editorial is based discusses how T cells interact with β-synuclein to cause the grey matter pathology in Lewis rats (rodents susceptible to experimental inflammatory diseases). Catchy editorials like this are doing a good job of taking us into the grey zone, and enabling us to see MS as much more than just relapses and progression.

By Mgconcrete – Own work, CC BY 3.0, Link

Don’t just stand there: do something!

If this headline doesn’t stop you in your tracks, then nothing ever will. The full title of this editorial is “Don’t just stand there: do something! The case for peri-ictal intervention”, and it comes from the journal Epilepsy Currents published in 2019. The paper it comments on, published in the journal Neurology in 2019, investigated the risk factors and best treatment for post-ictal hypoxaemia, that is low oxygen levels after generalised convulsions. It is not clear that the paper adds any new insights apart from stating the rather obvious fact that generalised convulsions lead to low blood oxygen levels. They however point out that the earlier oxygen is administered, the better the outcome. Not exactly groundbreaking research, but it resulted in an excellent catchy editorial.

Lightning over Maidstone, Kent. Sprogz on Flickr. https://www.flickr.com/photos/sprogz/14502687917

Heartbreakers

This editorial, full title being “Heartbreakers-cardiac stress after uncomplicated generalized convulsive seizures”, is from the journal Epilepsy Currents published in 2019. And it is all about the risk of potentially fatal cardiac complications of epilepsy. The author was commenting on a paper published in the journal Epilepsia in 2019, which investigated the biomarkers of cardiac stress after a generalised convulsion. In essence, they were on the hunt for any red flags of fatal arrythmias, and of Takotsubo cardiomyopathy. And (you may insert drum roll here) the leading heartbreaker turned out to be… high‐sensitive troponin T (hsTNT). A catchy title to remind us that the heart is at risk in epilepsy.

Mr. Bowie. L’Orso Sul Monociclo on Flickr. https://www.flickr.com/photos/reallyterriblephotographer/5675092938

***

Catchy titles help make the point. And there are more in the pipeline; the next in the series will look at catchy titles from the neurology archives. Watch this space, as they say.

July 13, 2020 Ibrahim Imam GENERAL

How effective is stem cell transplantation for NMO?

Autologous nonmyeloablative hematopoietic stem cell transplantation for neuromyelitis optica. Burt RK, Balabanov R, Han X, et al. Neurology 2019; 93:e1732-e1741. Abstract OBJECTIVE: To determine if autologous nonmyeloablative hematopoietic stem cell transplantation (HSCT) could be a salvage therapy for neuromyelitis optica spectrum disorder (NMOSD). METHODS: Thirteen patients were enrolled in a prospective open-label cohort study (11 […]

via How effective is stem cell transplantation for NMO? — Neurochecklists Blog

July 12, 2020 Ibrahim Imam GENERAL

Remembering

Remembering Author: Donald G McKay Synopsis This book is about Patient HM, probably the most important patient in the history of neuroscience because of what his brain revealed about how memory works. Known since his death by his real name, Henry Molaison, he developed profound amnesia following brain surgery to treat uncontrolled epilepsy, and the […]

via Remembering — The Doctors Bookshelf

July 4, 2020 Ibrahim Imam GENERAL

Permanent Present Tense

Permanent Present Tense Author: Suzanne Corkin Synopsis Henry Molaison, known throughout his life as Patient H.M, is the fateful neuroscience patient whose misfortune completely redefined our understanding of the inner workings of the brain. This book explores the amnesia Henry developed following unprecedented brain surgery for severe epilepsy, a unique disability that made him ‘the gold […]

via Permanent Present Tense — The Doctors Bookshelf

July 3, 2020 Ibrahim Imam GENERAL

Video: getting a handle on neurology

Neurochecklists is a comprehensive and practical neurology database. It is an easy to use handy and searchable reference tool. This short video shows how neurochecklists gives you a handle on neurology.

via Video: getting a handle on neurology — Neurochecklists Blog

***

Amyotrophic lateral sclerosis (ALS)

Aphantasia

Chronic inflammatory demyelinating polyneuropathy (CIDP)

Corticobasal degeneration (CBD)

Epilepsy

Essential tremor

Frontotemporal dementia (FTD)

Inclusion body myositis (IBM)

Meningitis

Migraine

Multiple sclerosis (MS)

Multiple system atrophy (MSA)

Myasthenia gravis (MG)

Myotonic dystrophy

Neurofibromatosis

Narcolepsy

Poliomyelitis

Progressive supranuclear palsy (PSP)

Restless legs syndrome (RLS)

Stiff person syndrome (SPS)

Synesthesia

Stroke

Tabes dorsalis

Trigeminal neuralgia

Tuberous sclerosis

***

***

Let us then celebrate the pioneers…

Eponymous and anonymous alike

Share this:

Like this:

Share this:

Like this:

Share this:

Like this:

Share this:

Like this:

Share this:

Like this:

***

***

Share this:

Like this:

Share this:

Like this:

Share this:

Like this:

Share this:

Like this:

Share this:

Like this: