What do neurology information seekers really want?

A few months ago, Neurochecklists set out to discover how people go about searching for neurology information. We therefore carried out an online survey of neurology information users. We asked 10 critical questions about the who, what, wherewhy, and how of neurology information quest.

https://pixabay.com/en/questions-who-what-how-why-where-1328465/

 

We asked these question specifically to guide a major Neurochecklists upgrade. This knowledge is, after all, critical for a website which has set out to be the best source of clear, concise, and comprehensive neurology information. But we needed help to know what really matters to people when they go foraging for neurology. What do they really want, and how do they go about satisfying their need?

Search Key. GotCredit on Flickr. https://www.flickr.com/photos/gotcredit/32943606783/

 

The response we got was heart-warming; about 190 people answered our online questions. Below are the questions along with the insights we gained from the answers.

Q&A. opensource.com on Flickr. https://www.flickr.com/photos/opensourceway/5556249000

 

Who searches for neurology information?

More than 50% of our responders were consultant neurologists, and about 15% were medical consultantsNeurology trainees constituted about 7%. The range of users is however quite broad, including nurses, surgeons, medical students, and patients! See the breakdown in the pie chart below:

Insight: There are diverse neurology information seekers!

How often do we forage for neurology information?

Neurology information is in high demand, with >50% of responders seeking information at least once a day, and >80% at least once a week. Below is the breakdown:

Insight: There is a huge craving for neurology information!

Where do we go when we need neurology information?

Online websites are by far the most popular source of quick neurology information, accounting for >50% of responses. This is followed by journals which account for just over 25% of responses. Very few responders access textbooks, handbooks, downloadable apps or online videos. Below is the breakdown:

Insight: Neurology source information is now mainly online

Where are we when we most crave neurology information?

In a question which allowed multiple answers, the clinic was by far the most common setting for looking up neurology information. We however also have a strong urge for neurology on the ward, and at home! Below is the breakdown:

Insight: The need for neurology information has no boundaries

Why do we access neurology information?

The most frequent reasons responders access neurology information were to answer clinical questions and for personal study. Other reasons were to aid discussions with patients, and to look for relevant references.

Insight: the checklist approach is the best solution

What devices do we use to access neurology information?

In another multiple answer question, responders most often use their phones to access online neurology information. Laptops and desktops are also favoured, but tablets much less so.

Insight: neurology information must be device-compatible

What features do we most favour in an online neurology database?

We asked what features responders most desire in an online neurology database, and the front-runners here are accuracy and currency of information, followed by conciseness, adequacy, ease of navigation, and link to references.

 

Insight: Neurochecklists is on the right track

***

We wish to extend our thanks to everybody who took part in the survey, including the many who attempted it after the closing date! We have taken all the responses on board, and we have been working night and day to provide an enhanced Neurochecklists. Watch out for our next blog post to find out the changes we will be launching soon. Neurology seekers, watch this space!

By AnsonloboOwn work, CC BY-SA 4.0, Link

8 things we now know about the toxicity of gadolinium to the brain

When it comes to imaging the nervous system, nothing but an MRI will do for the fastidious neurologist. CT has its uses, such as in detecting acute intracranial bleeding, but it lacks the sophistication to detect or differentiate between less glaring abnormalities. It also comes with a hefty radiation dose. MRI on the other hand, relying on powerful magnetic fields, is a ‘cleaner’ technology.

SLEIC 6. Penn State on Flickr. https://www.flickr.com/photos/pennstatelive/4946556307

MRI scans on their own are however often insufficient to sate the craving of the neurologist for precision. A plain MRI scan, for example, will not tell if a multiple sclerosis lesion is old or new, and it may fail to detect subtle but significant lesions such as low grade brain tumours or lymphoma. Many lesions on routine MRI scan are also ill-defined and non-specific, and could pass for abscesses, vasculitis, inflammation or just small vessel disease (wear and tear) changes.

The Brain. I has it. Deradrian on Flickr. https://www.flickr.com/photos/mgdtgd/3507973704

To silence the niggling doubts, the neurologist often requests an MRI scan with contrast. The idea is to use a dye to separate the wheat from the chaff, the active lesions from the silent ones. This works because sinister lesions have a bad and dangerous habit of disrupting the blood brain barrier. All such insurgencies across the hallowed BBB is sacrilege, a sign that something serious is afoot, (or is it underfoot?). Contrast dyes, on the other hand, are adept at detecting these breaches, traversing them, and staining the sinister lesion in the process. This stain appears on the MRI scan as contrast enhancement. MRI with contrast is therefore invaluable, and a positive study is a call to arms.

By © Nevit Dilmen, CC BY-SA 3.0, Link

Without any doubt, gadolinium is the favoured dye for contrast MRI scans. Gadolinium (Gd) is a lanthanide rare earth metal and it is one of the heavier elements of the periodic table with atomic number 64. It is named after the thrice-knighted Finnish chemist Johan Gadolin, who also discovered the first rare earth metal, yttrium.

Periodic table model. Canada Science and technology Museum on Flickr. https://www.flickr.com/photos/cstmweb/4888243867

We know a lot about some of the risks of injecting gadolinium into the body, such as its tendency to accumulate in people with kidney impairment (who cannot excrete it efficiently). We also know that it may cross the placenta to damage the developing baby. These are however hazards with simple and straight-forward solutions: avoid gadolinium in pregnancy, and don’t use it in people with poor renal function.

By Hi-Res Images ofChemical Elements – http://images-of-elements.com/gadolinium.php, CC BY 3.0, Link

Much more challenging is the problem of gadolinium deposition in the brain of people with normal renal function. This is concerning because it is unpredictable, and because it has the potential to compromise brain structure and function. This blog has previously asked the question, “Is gadolinium toxic?“. The question remains unanswered, and regulatory agencies are still studying the data to provide guidance to doctors. Patient groups on the other hand have been up in arms, as one would expect, impatiently waiting for answers. What then is the state of play with gadolinium? Should neurologists and their patients really be worried? Below are 8 things we now know about gadolinium and its potential brain toxicity.

By Peo at the Danish language Wikipedia, CC BY-SA 3.0, Link

•••

1. Gadolinium deposition is related to its insolubility at physiological pH

The toxic potential of gadolinium is thought to be the result of its insolubility at physiological pH. Furthermore, gadolinium competes against calcium, an element fundamental to cellular existence. This competition is obviously detrimental to the body.

064 Gadolinium-Periodic Table of Elements. Science Activism on Flickr. https://www.flickr.com/photos/137789813@N06/22951789105

2. The less stable gadolinium agents are the most toxic

There are two forms of gadolinium based contrast agents (GBCAs): the less stable linear GBCAs, and the more stable macrocyclic GBCAs. The linear GBCAs are more toxic, of which Gadodiamide (Omniscan) stands out. Other linear agents are gadobenate dimeglumine (MultiHance), gadopentetate dimeglumine (Magnevist), gadoversetamide (OptiMARK), gadoxetate (Eovist), and gadofosveset (Ablavar). The macrocyclic GBCAs, even though safer, are not entirely blameless. They include gadobuterol (Gadavist), gadoterate meglumine (Dotarem), and gadoteridol (ProHance). Therefore, choose your ‘gad’ wisely.

By زرشکOwn work, CC BY-SA 3.0, Link

 

3. Gadolinium deposits in favoured sites in the brain

It is now established that gadolinium deposits in three main brain areas. The most favoured site is the dentate nucleus of the cerebellum. Other popular regions are the globus pallidus and the pulvinar. This deposition is, paradoxically, visible on plain T1-weighted MRI scans where it shows as high signal intensity.

By Polygon data were generated by Database Center for Life Science(DBCLS)[2]. – Polygon data are from BodyParts3D[1], CC BY-SA 2.1 jp, Link

4. The risk of deposition depends on the number of injections

The risk of gadolinium deposition in the brain is higher with multiple administrations. Stated another way, and to stretch this paragraph out a bit longer, the more frequently contrast injections are given, the higher the chances gadolinium will stick to the brain. The possible risk threshold is 4 injections of gadolinium. The fewer the better…obviously!

Number-04. StefanSzczelkun on Flickr. https://www.flickr.com/photos/stefan-szczelkun/3931901057

5. Gadolinium also deposits outside the brain

The favoured site of gadolinium deposition outside the brain is the kidney, where it causes nephrogenic systemic fibrosis, a scleroderma-like disorder. This however occurs mostly in people with renal impairment. Gadolinium also deposits in other organs outside the brain including bone, skin, and liver. (Strictly speaking, this item has nothing to do with the brain, but it helped to tot up the number to 8 in the title of this blog post, avoiding the use of the more sinister se7en).

By JudgefloroOwn work, CC BY-SA 4.0, Link

 6. Harm from gadolinium brain deposition has not been established

Whilst we know for sure that gadolinium deposits in the nervous system, harm from deposition has not been definitively established. There are, however, reports that gadolinium deposition may produce muscle and eye symptoms, and chronic pain. There are also reports of cognitive impairment manifesting as reduced verbal fluency.

Words words words. Chris Blakeley on Flickr. https://www.flickr.com/photos/csb13/4276731632

7. Precautions may reduce the risk of gadolinium brain deposition

The current recommendation is not to withhold the appropriate use of gadolinium, but to observe simple precautions. Sensibly, use GBCAs only when absolutely necessary. Also consider preferentially using macrocyclic GBCAs and evaluate the necessity for giving repeated GBCA administrations.

 

By IntropinOwn work, CC BY-SA 3.0, Link

 

8. There are emerging ways to avoid gadolinium toxicity

The safest use of gadolinium is not to use it at all. There are some developments in the pipeline to achieve this, although probably not in the very near future. Such developments include manganese based contrast agents such as Mn-PyC3A. A less definitive option is to mitigate the effects of gadolinium by using chelating agents; two such potential agents are nanoparticles and 3,4,3-LI(1,2-HOPO).

 •••

Why not get the snapshot view of gadolinium toxicity in the neurochecklist:

Gadolinium-based contrast agent (GBCA) toxicity

…and leave a comment!

•••

MRI scan. NIH Image Gallery on Flikr. https://www.flickr.com/photos/nihgov/30805879596

What is so distinctive about anti-MUSK myasthenia gravis?

Myasthenia gravis (MG) is an iconic neurological disorder. It is classical in its presentation, weakness setting in with exertion and improving with rest. This fatigability is demonstrable in the laboratory when repetitive nerve stimulation (RNS) of the muscles results in a progressively decremental response. Clinically, myasthenia gravis is often a benign disorder which restricts itself to the muscles of the eyes: this ocular MG manifests just with droopy eyelids (ptosis) and double vision (diplopia). At the extreme however is generalised MG, a severe and life-threatening condition that justifies its grave appellation

By Posey & Spiller – Posey & Spiller: Fatigue (Ptosis) in a patient with MG (ed. 1904), Public Domain, Link

Myasthenia gravis depletes the energy reserve of muscles, something which is entirely dependent on acetylcholine (ACh), a chemical released at nerve endings. After release, ACh traverses the neuromuscular junction (NMJ) to attach itself to the acetylcholine receptor (AChR), which is comfortably nestled on the surface of the muscle. This binding of chemical to receptor is a significant event, setting sparks flying, and muscles contracting. In myasthenia gravis, this fundamental process is rudely disrupted by the onslaught of acetylcholine receptor antibodies. These aggressive AChR antibodies, produced by the thymus gland in the chest, vent their rage by competitively binding to the receptor, leaving acetylcholine high and dry. Eventually, the rampaging antibodies destroy the receptor in an act of unjustified savagery.

Drosophilia Neuron. NICHD on Flickr. https://www.flickr.com/photos/nichd/29596368551/

In tackling myasthenia gravis, it is no wonder that neurologists first have to hunt down the ferocious AChR antibodies. They whisk off an aliquot of serum to a specialist laboratory, but waste no time in planning a counteroffensive, confident that the test will return as positive. The strategy is to boost the level of acetylcholine in the NMJ, tilting the balance in favour of ACh against the antibodies. The tactic is to zealously despatch a prescription for a drug that will block acetylcholine esterase inhibitor, the enzyme which breaks down acetylcholine. The neurologist then closely observes the often dramatic response, one of the most gratifying in clinical medicine; one minute as weak as a kitten, the next minute as strong as an ox. MG is therefore one disorder which debunks the wicked jibe that neurologists know so much…but do so little to make their patients better!

Drosophilia Neuromuscular Junction. NICHD on Flickr. https://www.flickr.com/photos/nichd/34754479075

Unfortunately for the neurologist, every now and then, the AChR antibody test result comes back as negative. In the past, the dumbfounded and befuddled, but nevertheless undaunted neurologist, will march on, battling a diagnosis of antibody-negative MG. Nowadays however, this not a comfortable diagnosis to make because AChR antibody is no longer the only game in town. We now know that there are many other antibodies that are jostling for commanding positions in the anti-myasthenia coalition. These include anti LRP4, cotarctin, titin, agrin, netrin 1, VGKC, and ryanodine. However, the clear frontrunner in this melee is anti-MUSK antibody, responsible for 30-50% of MG in which there are no AChR antibodies.

By PyMol, CC0, Link

Anti MUSK syndrome has many distinguishing features that set it apart from the run-of-the-mill myasthenia gravis. Below are five distinctive markers of anti-MUSK syndrome:

  1. Subjects with anti-MUSK syndrome are typically middle-aged women in their 3rd or 4th decades. This is younger than the usual age of people with AChR MG. Indeed neurologists now recognise typical myasthenia as a disease of older people.
  2. People with anti-MUSK syndrome present with acute and prominent involvement of head and neck muscles. This results in marked swallowing and breathing difficulties. They are therefore at a higher risk of myasthenia crisis.
  3. Single fiber electromyogram (sfEMG), a specific and reliable neurophysiological test of MG, is often normal in anti-MUSK syndrome. This is partly because the limb muscles are usually spared in anti MUSK syndrome.
  4. People with anti-MUSK myasthenia often do not benefit from, nor do they tolerate, the  acetylcholinesterase inhibitors which are used to treat MG. Indeed, these drugs may worsen anti-MUSK syndrome.
  5. Thymectomy, removal of the thymus gland, is not beneficial in people with anti-MUSK syndrome, unlike its usefulness in AChR MG.
Thymus gland 2. RachelHermosillo on Flickr. https://www.flickr.com/photos/rachelhermosillo/5388860587

All this is just the tip of the evolving myasthenia gravis iceberg. You may explore more of myasthenia in our previous blog posts:

How is innovative neurology research energising myasthenia?

What is the startling research unsettling the treatment of myasthenia gravis?

What is the relationship of pregnancy to myasthenia gravis?

Is Zika virus infection a risk factor for myasthenia gravis?

What does the EMG show in LRP4 myasthenia gravis?

What’s evolving at the cutting-edge of autoimmune neurology?

What are the most iconic neurological disorders?

***

You may also explore anti-MUSK, and all the other myasthenia gravis subtypes, in neurochecklists. Go on…you know you want to know more!

Antibody lights. Isabelle on Flickr. https://www.flickr.com/photos/diamondgeyser/456900567/

 

What is the value of temporal artery biopsy in the diagnosis of GCA?

Giant cell arteritis (GCA) is a nasty inflammatory disorder that affects the large arteries. Because it characteristically involves the temporal artery, this form of vasculitis is also referred to as temporal arteritis. It usually affects people over the age of 50 years and manifests with sudden onset headache, scalp pain, and a thick, tender temporal artery. GCA is often accompanied by polymyalgia rheumatica (PMR) , a painful condition of the joints and muscles. The active systemic inflammation in GCA is often detected by the erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) tests. These distinguishing features constitute most of the diagnostic criteria for GCA.

By National Institute of Standards and Technology – https://www.flickr.com/photos/usnistgov/5828207621/, Public Domain, Link

Most people with GCA however do not have all the ‘classical’ features of the disease. A high index of suspicion is therefore required to sniff out the duplicitous miscreant. It is particularly imperative to make the diagnosis as early as possible to prevent the dreaded complications of GCA, sudden blindness and stroke. The treatment of GCA, implemented according to established treatment guidelines, involves several months of oral steroids, drugs which cause immune suppression and a host of other side effects. It is therefore essential that the diagnosis of GCA is made correctly to avoid putting the patient on a long, risky, and unnecessary treatment.

By Henry Vandyke CarterHenry Gray (1918) Anatomy of the Human Body (See “Book” section below)Bartleby.com: Gray’s Anatomy, Plate 508, Public Domain, Link

The conventional method of establishing the definitive diagnosis of GCA is by performing a temporal artery biopsy. This involves taking a short segment of the temporal artery as it traverses the temple. This procedure however only confirms the diagnosis of GCA in 1335% of people with the condition. One reason the biopsy has such a poor sensitivity is that it is often performed after treatment has already commenced. Another reason the biopsy is often normal is that the inflammation in GCA occurs in patches, sparing large segments of the artery. Don’t even think about it-taking a longer biopsy segment does not increase the yield of temporal artery biopsy. Put another way, “specimen length is not associated with diagnostic yield of temporal artery biopsy.

By NephronOwn work, CC BY-SA 3.0, Link
What then is the value of the temporal artery biopsy in the diagnosis of GCA? This is the question posed by Bowling et al in their incisive paper titled Temporal artery biopsy in the diagnosis of giant cell arteritis: does the end justify the means? They reviewed 129 temporal artery biopsies and found that the clinical diagnosis of GCA was confirmed in only 13% of cases. Furthermore, the outcome of the biopsy rarely ever influenced the treatment; 87% of those with a normal biopsy result still continued their treatment. The miffed authors therefore rhetorically, and indignantly, asked: “can we justify invasive surgery to all patients on histological grounds when the results may not alter management?” 
Ipswich, Waterfront, Ipswich Campus, The Big Question Mark Sculpture. Martin Pettitt on Flikr. https://www.flickr.com/photos/mdpettitt/8671901426

This is an entirely reasonable question especially because there are other more accurate and less invasive ways of establishing the diagnosis of GCA. These include:

But the answer to the authors’ rhetorical question is anyones guess. It is a sad tradition of medicine that studies such as these take ages to change practice. Indeed I predict the the temporal artery biopsy will sidestep this minor hurdle and simply continue its long and agonising reign. Despair!
By No machine-readable author provided. Spekta assumed (based on copyright claims). – No machine-readable source provided. Own work assumed (based on copyright claims)., Public Domain, Link
You can at least read more on GCA in my previous blog post titled Advances in the management of giant cell arteritis. You may also explore these comprehensive neurochecklistsGiant cell arteritis (GCA): clinical featuresand Giant cell arteritis (GCA): diagnosis and management.

 

FDG. TRIUMF Lab on Flikr. https://www.flickr.com/photos/triumflab/8232448893

 

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

 

The 13 most dreadful neurological disorders…and the groups standing up to them

Neurology embodies some of the most dreadful diseases known to man. Every neurological disorder is disheartening, each characterised by unique frustrations for patients and their families. It is difficult to quantify the distress and misery these afflictions impose on their victims, and even harder to appreciate the despair and anguish they evoke in those who care for them.

Brain Art. Ars Electronica on Flikr. https://www.flickr.com/photos/arselectronica/7773544158

It is clearly hard to compare the impact of different neurological diseases. Some neurological disorders however stand out because of the consternation their names evoke, and the terror that follows in their wake. These diseases come with unimaginable physical and psychological burdens, and crushing demands on human and material resources. They impose either a debilitating morbidity, or a hasty mortality.

Neural pathways in the brain. NICHD on Flikr. https://www.flickr.com/photos/nichd/16672073333

The nervous system ailments in the list below pose exacting therapeutic challenges, resistant as they are to all attempts at treatment or cure. This list sets out to emphasise the urgency for neuroscience to find a remedy for each of them, but it does not intend to belittle the horror of the disorders omitted from it. The choice of the number 13 is, sadly, self-evident. Here then are the top 13 most dreadful neurological disorders…all with gold links to the associations helping to defeat them.

Working Brain. Gontzal García del Caño on Flikr. https://www.flickr.com/photos/euskalanato/2052487054

Ataxia

Ataxia, in lay terms, is incoordination. This typically manifests as an unsteady gait and clumsiness. Ataxia converts all activities of daily living into burdensome chores. Whilst many types of ataxia are preventable or reversible, primary ataxias are progressive and carry a dismal outlook. In this category are Spinocerebellar ataxia (SCA)Friedreich’s ataxia, and Ataxia telangiectasia. You may read more about ataxia in these previous blog posts:

The 43 spinocerebellar ataxias: the complete checklists

Old drugs, new roles?

Will Riluzole really be good for cerebellar ataxia?

Brain tumours

Brain cancers hardly need any description. They are either primary, arising from the brain cells, or metastatic, spreading to the brain from other organs. Some primary brain cancers, such as meningiomas and pituitary tumours, are, relatively, treatable. Many others are unfortunately ominously malignant. The most dreadful in this category is surely the spine-chilling glioblastoma multiforme. You may check out these previous blog posts for more on these tumuors: 

Calming the rage of brain tumours: hope for a dreaded cancer

Maggots, viruses and lasers: some innovations for brain tumours 

Are steroids detrimental to survival in brain tumours?

Peripheral neuropathy

Peripheral neuropathy is ubiquitous in the neurology clinic. Neuropathy may result from reversible situations such as overindulgence in alcohol, uncontrolled diabetes, or Vitamin B12 deficiency. Neuropathy is often just a minor inconvenience when it manifests with sensory symptoms such as tingling and numbness. It may however be debilitating when it presents as limb paralysis, or complicated by major skeletal deformities. At the severe end of the spectrum of neuropathy are the hereditary forms such as Charcot Marie Tooth disease (CMT) and Familial amyloid polyneuropathy. Read more in these blog posts:

The 52 variants of CMT… and their practical checklists 

What’s looming at the frontline of peripheral neuropathy?

Will a pill really hold the cure for CMT?

Creutzfeldt Jakob disease (CJD)

CJD is the most iconic of the prion diseases. These disorders are as horrendous as they are enigmatic, defying categorisation as either infections or neurodegenerative diseases. More puzzling is their ability to be either hereditary and acquired. CJD exists in the classic or variant form, but both share a relentlessly rapid course, and a uniformly fatal end. You may read more in these previous blog posts titled:

Final day of ANA 2015- Prions center stage

What are the links between Prion diseases and Parkinsonian disorders?

Dementia

Dementia is the scourge of longevity. Its name strikes terror because it insidiously colonises the cells that make us who we are. The most prominent dementia is Alzheimer’s disease, but it has equally dreadful companions such as Frontotemporal dementia (FTD) and Dementia with Lewy bodies (DLB). Read more on dementia in these blog posts:

How bright is the future for Alzheimer’s disease?

Alzheimer’s disease: a few curious things 

Alzheimers disease and its promising links with diabetes

Dystonia

Dystonia marks its presence by distressing movements and painful postures. At its most benign, dystonia is only a twitch of the eyelid (blepharospasm) or a flicker of one side of the face (hemifacial spasm). At the extreme end, it produces continuous twisting and swirling motions, often defying all treatments. The causes of dystonia are legion, but the primary dystonias stand out by their hereditary transmission and marked severity. Read more on dystonia in these blog posts:

Why does dystonia fascinate and challenge neurology?

Making sense of the dystonias: the practical checklists

Huntington’s disease (HD)

Huntington’s disease is an iconic eponymous neurological disorder which is marked by the vicious triumvirate of chorea, dementia, and a positive family history. It is an awful condition, often driving its victims to suicide. It is a so-called trinucleotide repeat expansion disorder, implying that successive generations manifest the disease at an earlier age, and in more severe forms (genetic anticipation). You may read more on HD in the previous blog post titled:

What are the prospects of stamping out Huntington’s disease? 

Motor neurone disease (MND) 

Also known as Amyotrophic lateral sclerosis (ALS), MND is simply devastating. Recognising no anatomical boundaries, it ravages the central and peripheral nervous systems equally. MND creeps up on the neurones and causes early muscle twitching (fasciculations) and cramps. It then gradually devours the nerves resulting in muscle wasting, loss of speech, ineffectual breathing, and impaired swallowing. Our previous blog posts on MND are:

Is neurology research finally breaking the resolve of MND?

The emerging links between depression and MND

What is the relationship of MND and cancer?

Does diabetes protect from MND?

MND and funeral directors-really?

Multiple sclerosis (MS)

Multiple sclerosis is a very common disease, and gets more common the further away you get from the equator. It is the subject of intense research because of the devastation it foists on predominantly young people. Many drugs now ameliorate, and even seem to halt the progression of, relapsing remitting MS (RRMS). This is however not the case with primary progressive MS (PPMS) which, until the introduction of ocrelizumab, defied all treatments. There are many contenders vying for the cause of MS, but the reason nerves in the central nervous system inexplicably lose their myelin sheaths remains elusive. You may read more on MS in these blog posts:

The emerging progress from the world of MS

What are the remarkable drugs which have transformed the treatment of MS?

Is low vitamin D a cause of multiple sclerosis?

Muscular dystrophy 

Muscular dystrophy is an umbrella term that covers a diverse range of inherited muscle diseases. The most devastating, on account of its early onset and unrelenting progression, is Duchenne muscular dystrophy (DMD). Adult neurologists will be more familiar with late onset muscular dystrophies such as Myotonic dystrophy and Facioscapulohumeral muscular dystrophy (FSHD). Read more on muscular dystrophy in these previous blog posts:

How is neurology stamping out the anguish of Duchenne?

The A–Z of limb girdle muscular dystrophy (LGMD)

Rabies

Rabies, a rhabdovirus, is a zoonosis-it is transmitted to man by a wide range of animals such as dogs, bats, racoons, and skunks. It is the quintessential deadly neurological disease, popularised by the Steven King book and film, Cujo. Rabies manifests either as the encephalitic (furious) or the paralytic (dumb) forms. It wreaks havoc by causing irritability, hydrophobia (fear of water),  excessive sweating, altered consciousness, and inevitably death. Whilst there are vaccines to protect against rabies, a cure has eluded neuroscientists. This blog is yet to do justice to rabies but it is, at least, listed in the post titled What are the most iconic neurological disorders? But you could better by checking neurochecklists for details of the clinical features and management of rabies.

Spinal cord injury

Nothing is quite as heart-wrenching as the sudden loss of body function that results from spinal cord trauma. This often causes paralysis of both legs (paraplegia), or all four limbs (quadriplegia). This life-changing disorder is often accompanied by loss of control over bowel and bladder functions, and complications such as bed sores and painful spasms. You may read about the heroic efforts to treat spinal cord injury in the blog posts titled:

6 innovations in the treatment of spinal cord injury

Head transplant, anyone?

Tetanus

Tetanus is an eminently preventable disease, now almost wiped out in developed countries by simple immunisation. It however continues its pillage and plunder in the developing world. It strikes young and old alike, often invading the body through innocuous wounds. Tetanus is caused by tetanospasmin and tetanolysin, the deadly toxins of the bacterium Clostridium tetani. The disease is classified as generalised, localised, cephalic, or neonatal tetanus. It is characterised by painful spasms which manifest as lockjaw (trismus), facial contortions (risus sardonicus), trunkal rigidity (opisthotonus), and vocal cord spasms (laryngospasm). The disease is awfully distressing and, when advanced, untreatable. It is a stain on the world that this avoidable disorder continuous to threaten a large number of its inhabitants. Check neurochecklists for more on the pathology, clinical features, and management of tetanus.

 

Light brain. Mario D’Amore on Flikr. https://www.flickr.com/photos/kidpixo/3470448888

As for all lists, this will surely be subject to debate, or perhaps some healthy controversy. Please leave a comment.

The emerging links between depression and MND

At first, it seemed like a single drop, but it is quickly turning into a trickle. The first inkling was a study of >1,700 people with motor neurone disease (MND) which was published in the journal Neurology titled Depression in amyotrophic lateral sclerosis. The authors found that depression is a very frequent diagnosis shortly before people are diagnosed with MND.

Von Vincent van Gogh - The Yorck Project: 10.000 Meisterwerke der Malerei. DVD-ROM, 2002. ISBN 3936122202. Distributed by DIRECTMEDIA Publishing GmbH., Gemeinfrei, Link
Von Vincent van Gogh – The Yorck Project: 10.000 Meisterwerke der Malerei. DVD-ROM, 2002. ISBN 3936122202. Distributed by DIRECTMEDIA Publishing GmbH., Gemeinfrei, Link

Surely a coincidence, I thought. A rogue finding, or even an understandable response to illness. My excuses were however debunked by another paper published soon after in the Annals of Neurology. Titled Psychiatric disorders prior to amyotrophic lateral sclerosis, the study found that depression may precede the diagnosis of MND by more than 5 years. The authors also report a high frequency of other psychiatric conditions preceding the diagnosis of MND, such as anxiety and psychosis

Depression. Nils Werner on Flikr. https://www.flickr.com/photos/130721398@N06/25363062843
Depression. Nils Werner on Flikr. https://www.flickr.com/photos/130721398@N06/25363062843

 

And just off the press is this report from Nature Communications titled Genetic correlation between amyotrophic lateral sclerosis and schizophrenia. What do we make of this? Is this just the tip of the iceberg? Surely more studies are needed before any firm conclusions. Perhaps this may lead to some early biomarker that enables neurologists to stop the process of progression to full blown MND. Perhaps.

https://pixabay.com/en/sky-clouds-rays-of-sunshine-414199/
https://pixabay.com/en/sky-clouds-rays-of-sunshine-414199/

_________________________________________________________________________

neurochecklists-image