Autoimmunedisorders are probably the most proliferative field of neurology. It seems like there is a blazing headline every week announcing a new antibody disease. Many of these antibodies are esoteric, but some shake the foundations of medical practice. Anti-MOGantibody is one of those which requires you to stop and pay attention, and it has significantly affected neurological practice in a very big way.
Perhaps the most important thing about anti-MOG antibody disease is that, like the chameleon, it presents in many guises. For the neurologist therefore, the first thing is to recognise these varied manifestations. Here then is a quick list of the 9 manifestations of anti MOG antibody disorder.
The Neurology Lounge is always on the lookout for catchy neurology article titles to adorn its shelves. My previous blog post in this quest was The art of spinning catchy titles.
Since then, there have been quite a few brilliant article titles that have caught my fancy. We must acknowledge the wordsmiths who craftily and meticulously think up these magical headlines; they put in a lot of thought to conjure up the right words to use. The look into their crystal balls to predict the best way to play around with the meanings. With a bit of lexical alchemy, they miraculously come up with the titles that make us do a double-take, but do so with a smile. Below are 9 such catchy titles.
This title reflects the science suggesting that Parkinson’s disease originates from the gut. This editorial restates the proposition that α-synuclein starts accumulating in the intestines before migrating, up the vagus nerve, ‘in a prion-like fashion’, to the brain.
Patent foramen ovale (PFO) is a hole in the heart which connects the upper two heart chambers, or atria. It normally closes after birth, but in some people it persists to cause some grief to cardiologists and neurologists. Whether a PFO causes migraine or not is a long standing contentious issue in Neurology. The authors of this study found no link between migraine and (PFO). The title is brilliant, but the tone of finality is probably premature; I guess this debate is far from over.
And still on migraine is this headline grabber. A bit on the basic science spectrum, I quote from the abstract to give you a flavour: ‘This review focuses on recent structural and functional neuroimaging studies that investigated the role of subcortical and cortical structures in modulating nociceptive input in migraine, which outlined the presence of an imbalance between inhibitory and excitatory modulation of pain processing in the disease‘. I would rather stick with the punchy headline myself.
This is a clear play on the defining feature of neuromyelitis optica (NMO), a long segment of inflammation in the spinal cord. This is what neurologists call longitudinally extensive transverse myelitis (LETM). This is an excellent editorial, worthy of the headline. It emphasises the point that NMO really has no defining features, not even the presence of the ‘defining’ antibody, anti-aquaporin 4- just ask anti-MOG NMO about this
How do you prevent a harmful preventative practice?. By a paper with a title that is pure genius of course. The authors of this paper highlight the persisting, anti-guideline, practice of using prophylactic antiepileptic drugs (AEDs) in people who have had intracerebral haemorrhage (ICH). The paper rhetorically asks if this has ‘become a habit too difficult to break?’ Not going by this catchy headline!
Parasomnias are diseases that occur during or related to sleep. This headline is for an editorial on a new parasomnia called anti IgLON5 antibody disorder. This is the subject of my previous blog post titled IgLON5: a new antibody disorder for neurologists. The headline writer here is clearly a fan of John Milton. I however struggled to make the connection between the excellent headline and the subject of the paper. I however presume it relates to the ‘loss of sleep paralysis‘ that accompanies many sleep disorders, including the quintessential parasomnia- REM sleep behaviour disorder (RBD). Excellent title anyway.
With a slightly wicked wit, this headline focuses on the slow walking speed of people with hereditary spastic paraplegia (HSP), contrasting this with the increasing research output on the disease. A bit dated I admit, but the paper refers to work which identified the genetic basis of SPG3, one of the commoner HSPs. A lesson in headline writing from the archives you may say.
The headline is brilliant, but the content goes way over my head. It is an editorial on a basic science paper. For the curious and the nerdy, I quote an extract: ‘during synapse elimination in the developing neuromuscular junction, branch-specific microtubule destabilization results in arrested axonal transport and induces axon branch loss. This process is mediated in part by the neurodegeneration-associated, microtubule-severing protein spastin‘. Enough I hear you say. OK, just stick with the headline.
Do you have any catchy titles-please drop a comment.
What is it about neurological inflammatory disorders that makes them so rebellious? Why do they defy convention and disregard their defining features. I discussed a similar phenomenon in my previous blog post titled Why is neuromyelitis optica (NMO) endlessly surprising neurology? NMO refused to play by the rules and was punished by having it’s named changed to NMOSD. Perhaps it’s time for CLIPPERS to suffer the same fate….starting with a shorter acronym perhaps?
Neurology is a broad specialty covering a staggering variety of diseases. Some neurological disorders are vanishingly rare, but many are household names, or at least vaguely familiar to most people. These are the diseases which define neurology. Here, in alphabetical order, is my list of the top 60 iconic neurological diseases, with links to previous blog posts where available.
The Neurology Lounge has a way to go to address all these diseases, but they are all fully covered in neurochecklists. In a future post, I will look at the rare end of the neurological spectrum and list the 75 strangest and most exotic neurological disorders.
Neuromyelitis optica (NMO) may be seen as the rarer and more mysterious cousin of multiple sclerosis (MS). It is characterised by a long segment of inflammation in the spinal cord, and this occurs almost simultaneously with inflammation of the optic nerves. Unlike MS, there is usually no involvement of the brain. NMO is also known as Devic disease, after the French neurologist Eugène Devic.
NMO has had a very chequered history, refusing to be tied down, and defying all attempts at pigeon-holing. It has thrown up surprises over the decades, from its humble beginnings as a possible variant of multiple sclerosis, to its current complex status as an independent entity. It marks its territory by sprinkling anti aquaporin 4, its presumed causative antibody. We however now know that NMO doesn’t respect any of its defining features, even the presence of aquaporin 4. The International consensus diagnostic criteria for neuromyelitis optica spectrum disordersconfirms this.The experts struggled to pin it down … couldn’t…gave in… and took the easy way out: they developed awider construct to accommodate it all, calling this neuromyelitis spectrum disorders (NMOSD).
Why is NMO such an enigma? Because neurologists are never satisfied with the superficial. We like digging deeper, unearthing the hidden. And the longer neurologist study NMO, the more unusual the syndrome turns out to be. No wonder NMO now also encompasses patients with cerebral, diencephalic, and brainstem lesions. How more intriguing can it get? Here are 6 surprisingreports about NMO.
1. Spinal movement disorders
Spinal movement disorders are not run-of-the-mill in neurology. Myoclonus is probably the closest we get to see. A recent report in Movement Disorders (where else) enlightens us that spinal movement disorders in NMOare not infrequent (pardon the double negative, but it’s so convenient sometimes). The authors classify these disorders into five: tonic spasms, focal dystonia; spinal myoclonus,spontaneous clonus, and tremors.The paper cautions that NMO may present first with spinal movement disorders, and these are often ‘overlooked, mislabeled, or under-treated’.
2. Impaired sense of smell
Just when you thought only neurodegenerative diseases present with an impaired sense of smell, an article turns up in Journal of Neurology titled Olfactory dysfunction in neuromyelitis optica spectrum disorders. The authors of the paper found that slightly more than half of the 49 subjects with NMO had olfactory dysfunction. Bring out the UPSIT.
The hallmark of NMO is the longitudinally extensive transverse myelitis (LETM), inflammation of the spinal cord at least 3 vertebral segments long. There are however many other diseases that present with LETM-see this review article in Nature Reviews Neurology which listsdiseases that may manifest with LETM.Spoiler alert-it’s not open access! A recent piece in Neurology further extended the list (pardon the puns) with a case of MELAS presenting with LETM. MELAS is a mitochondrial disease that is more notorious for being a stroke mimic. Not to be outdone, JAMA Neurology had a case of nitrous oxide myelopathy with LETM. Last word however to Neurology, LETM may be seen in CLIPPERS.
5. Genetic pointer to relapse after treatment
It is no news that the monoclonal antibody, Rituximab, is an effective treatment for NMO. What is news is the report of a genetic marker of poor responsiveness to treatment with Rituximab. Researchers publishing in JAMA Neurology report that the fragment c gamma receptor 3A (FCGR3A) polymorphism increases the risk of relapseon treatment. Why on earth did they check for that specific polymorphism? I didn’t have access to the full article to find out…if you have the answer please let us know.
6. Escalation of treatment improves outcome
Sadly the outcome of NMO is not as good as one would hope. Relapses are common after remission, and these are not always amenable to treatment. A recent article however raised the spirits by showing that recalcitrant relapses may respond to escalation of the treatment level. The authors carried out a large scale trial published in Annals of Neurology titled Neuromyelitis optica: Evaluation of 871 attacks and 1,153 treatment courses. With escalation of treatment, raising the bar a notch higher, remission is achieved in many cases. There is therefore no place for pulling any punches when it comes to NMO.
The phenotype of neuromyelitis optica will no doubt evolve further. Please leave a comment on any unusual sightings.