Factors influencing mortality in ALS are clearly multifactorial.  The ability to determine these factors and subsequently modify them could be of therapeutic significance in ALS.  The large EPIC cohort recently established that a higher body mass index may be neuroprotective, i.e. reduce mortality in ALS, which was evident in both males and females.  This association could be explained by hypermetabolism.  Namely ALS patients  and SOD-1 mice seem to exhibit a hyper-metabolic state.  Perhaps the link to sport may also have a similar etiology?  Is this a chance association only?  What do you think??

Gallo V et al. Prediagnostic body fat and risk of death from amyotrophic lateral sclerosis: The EPIC cohort. Neurology. 2013;80:829-838.

Cortical atrophy, as assessed by novel volumetric MRI techniques, have revealed inconsistent results in ALS.  Specifically, while cortical thinning, predominantly within the motor/frontal lobe regions, have been reported by some, absence of atrophy has been reported by others.  Mioshi and colleagues elegantly demonstrate a potential explanation for such discordant MRI findings.  Namely, ALS patients with cognitive dysfunction exhibit abnormalities within the motor and somatosensory areas  while pure ALS patients have normal MRI findings.

Could this be of diagnostic or event therapeutic significance in ALS?  Perhaps!!

Mioshi E, Lillo P, Yew B, Hsieh S, Savage S, Hodges JR, Kiernan MC, Hornberger M. Cortical atrophy in ALS is critically associated with neuropsychiatric and cognitive changes. Neurology. 2013 19;80:1117-23.

This is your Captain speaking: “Go easy on the drugs and alcohol and enjoy your flight”

Planes and an ailing brain….

The most common reason to be referred to neurology was seizure, followed by stroke.

Dr Susanna Park from the Institute of Neurology, UK critically considers Stroke and the First Week

The focus of the March issue of JNNP is stroke, highlighting a range of research assessing risk factors, prognosis, treatment and management.

In this timely issue, Kauranen and colleagues¹ present a study which emphasises the importance of cognitive function in the early stages after a stroke as a predictor of long term outcomes. In the study, 140 patients who experienced a first ever ischaemic stroke underwent a neurocognitive evaluation within the first week post-stroke. Before their stroke, all of the patients were engaged in full time employment. Six months later, the main predictor for a return to work was the number of cognitive deficits that were present in the first week.

Importantly, each additional cognitive deficit present in the first week doubled the likelihood of a patient being unable to return to employment at follow-up. This study stresses the role of cognition as an important predictor of long term outcomes post-stroke², with previous studies indicating that cognitive status is also predictive of long-term functional status³ and quality of life⁴.

In the same issue of JNNP, El Hachioui and colleagues⁵ identified that the degree of aphasia one year following a stroke could be predicted within the first week by a combination of factors including phonology scores and stroke severity indices. These studies highlight the first week following stroke as a critical window not only for prognosis but also for rehabilitation and recovery.  Not only are early assessments of cognition and language invaluable for predicting long-term prognosis for stroke patients, but early interventions may be able to intervene in this time period to boost functional recovery and rehabilitative outcomes long-term.

Recovery post-stroke.
Wallaby great Michael Lynagh and his neurologist Dr Rob Henderson – discuss Noddy’s miraculous recovery post-stroke

References

1. Kauranen T, Turunen K, Laari S, Mustanoja S, Baumann P, Poutiainen E (2013) The severity of cognitive deficits predicts return to work after a first ever ischaemic stroke. JNNP 84: 316-321.

2. Arauz A. (2013) Return to work after stroke: the role of cognitive deficits. JNNP 84: 240.

3. Wagle J, Farner L, Flekkᴓy K, Bruun Wyller T, Sandvik L, Fure B, Stensrᴓd B, Engedal K (2011). Early post-stroke cognition in stroke rehabilitation patients predicts functional outcome at 13 months.  Dement Geriatr Cogn Disord 31: 379-387.

4. Nys GM, van  Zandvoort MJ, van der Worp HB, de Haan EH, de Kort  PL, Jansen BP, Kappelle LJ. (2006). Early cognitive impairment predicts long-term depressive symptoms and quality of life after stroke. J Neurol Sci 247: 149-56

5. El-Hachioui H, Lingsma HF, van de Sandt-Koenderman MWME, Dippel DWJ, Koudstaal PJ, Visch-Brink EG. (2013) Long-term prognosis of aphasia after stroke.  JNNP 84: 310-315.

Ismail and colleagues report on a high concurrence rate of multiple sclerosis (MS) and ALS.  Interestingly, the C9orf72 expansion was identified in 80% of the ALS-MS patients.  Given that the inflammatory system appears to be deregulated in patients expressing the c9orf72 expansion, with down-regulation of the neuroprotective CXCL10 chemokine and dysfunction of NF-kB activity,   the findings in the present study imply a role for inflammation in ALS.  The implications being that modulation of these neuroinflammatory pathways may prove therapeutically useful in ALS, especially given that c0orf72 expansions may be identified in up to20% of sporadic ALS patients.

Ismail et al.  Concurrence of multiple sclerosis and amyotrophic lateral sclerosis in patients with hexanucleotide repeat expansions of C9ORF72. J Neurol Neurosurg Psychiatry 2013;84:79-87 doi:10.1136/jnnp-2012-303326.

Omar and colleagues identified abnormalities in processing and identification of common flavors in patients with frontotemporal lobe syndromes.  This loss of flavor processing was associated with atrophy of the left entorhinal cortex, hippocampus, parahippocampal gyrus and temporal pole.  From a clinical perspective, these findings provide insights into abnormal eating behavior patterns in FTD and ultimately will enable a better management of such patients.

Omar et al.  Flavour identification in frontotemporal lobar degeneration. J Neurol Neurosurg Psychiatry J Neurol Neurosurg Psychiatry 2013;84:88-93 doi:10.1136/jnnp-2012-303853

It may seem strange, but asking patients with Parkinson’s disease to rate how much their symptoms have improved doesn’t seem to match up to clinical improvements, at least as assessed by using the conventional symptoms scores used by clinical researchers.

Doctors and researchers use different symptom scores to help measure the symptoms of people with Parkinson’s disease. Examples are the Unified Parkinson’s Disease Rating Scale (UPDRS), the Mini-Mental State Examination (MMSE), and the 39-item Parkinson’s Disease Questionnaire (PDQ-39).

These tools are useful as they enable neurologists to assess how much a person is affected by the symptoms of Parkinson’s. These scores may also be useful to weigh up if a treatment works, by measuring the patient’s symptom scores before and after treatment. The scores can be important for working out if a person has Parkinson’s disease, or a different type of movement disorder.

People with Parkinson’s will usually improve after taking dopamine agonists. In addition to symptom scores, neurologists will often ask patients how they feel after taking treatment – without any real objective appraisal.

In recent research published in JNNP – a study of 133 patients with Parkinson’s disease measured the improvement in people’s symptoms immediately after starting treatment with dopamine agonists, then again after an average of six and then 12 months of treatment.

Researchers used symptom scores and tests, as well as asking patients to rate how much their symptoms had improved. They then compared the test results with people’s own ratings, to see how they matched up.

Of interest, most patients showed improvements in their measured scores although there was little relationship between these scores and how patients rated their own symptoms. The best match was between patient scores and the results of the UPRDS. When the researchers compared people’s own scores with this scale, they found around 33 in 100 people who showed no improvement in their UPDRS score at the first follow-up rated their improvement as moderate or better. Around 29 in 100 of those whose UPDRS score showed a good response said they had either no improvement or only a slight improvement.

What do these findings mean for the clinician?

Clearly, neurologists and their patients eed to come up with better ways to establish treatment responses.

Any suggestions?

Further reading: Davidson MA, McGhee DJM, Counsell CE. Comparison of patient rated treatment response with measured improvement in Parkinson’s disease. J Neurol Neurosurg Psychiatry 2012;83:1001–1005. http://jnnp.bmj.com/content/83/10/1001.full

 Medical publishing metrics and financial yields

In “Chapters from My Autobiography” published in 1906, Mark Twain noted that “figures often beguile me, particularly when I have the arranging of them myself; in which case the remark attributed to Disraeli would often apply with justice and force: ‘There are three kinds of lies: lies, damned lies, and statistics”.

Apparently no mention of the phrase could be found in the Disreali archives, but regardless of attribution, the phrase found resonance at the time and thereafter, and seems particularly relevant in the ever changing world of medical publications and the calculation of their metrics.

To understand the basis of publication metrics and value more generally, perhaps it would be best to start by considering why clinicians seek to publish their clinical research in the first place. Is it to convey advancement, to participate in medical discovery, to improve patient outcomes, to generate personal funding, publicity, acclaim, or to enhance their curriculum vitae? While considering these motives, what then are their metrics of success? Separately, irrespective of the desires of an author, from a publishing perspective, medical journals remain a profitable business, associated with significant monetary awards. Indeed, publication of a single manuscript can generate reprint sales of GB£1.5million and beyond.

Where then lies the future role of a specialty journal such as JNNP? In the past there was significant branding across the specialty journals, and each appeared to developed a separate niche. Readers would adopt their favourite journals, some for a good fireside read, others passing for or contributing to bathtime entertainment (‘What neurologists read’; Charles Warlow, personal communication). With significant developments across electronic media, and planned changes in free and open access for all medical research, such individualised approaches may begin to have less meaning, as the boundaries become blurred. One only needs to observe the changes afoot across the newspaper media landscape to understand the new rules of engagement (or non-engagement), the impact of new media and the effects of cannabalism. Similarly, medical students, allied health staff and doctors, whilst previously having turned their attention to medline and equivalent to direct their research needs, may now be more engaged with Facebook and Twitter.

When considering the role of JNNP in medical publishing and education more generally, the journal is now approaching its centenary.

Age has not wearied the journal, and it continues to metamorphose with the times.

In addition to being at the forefront of current neuroscience practice, the journal’s reputation built up from launch in 1920, is reflected through achievement of the longest journal citation half-life across the clinical neurosciences. That is to say that the original JNNP manuscripts continue to be heavily cited, many in the thousands, some now with more than 10,000 citations.

With the perpetual evolution of medical publishing, JNNP continues to be excited about the future. The journal strives to identify key new developments and potential future discoveries in the constantly changing world of neuroscience.

Traversing the realm of clinical neuroscience, we hope to uncover the citation classics of the future, while at the same time, catering to a more general audience with teaching and wider instruction in mind.

Despite the challenges of an ever-changing medical publishing landscape, with the need to adapt and remain fresh (including the launch of our new cover image with this issue), we look forward to sharing the trail blazers with you over the coming year!

Introduction

A web app has been developed that can test the eyesight of people with conditions that cause vision loss – particularly stroke.

Hemianopia is a condition that causes a loss of vision in either the right or left sides of both eyes. It is a common side effect of stroke. Less commonly, it occurs after an injury to the brain or a brain tumour.

People with hemianopia can often make adaptations and changes to their lifestyle to cope with the loss of vision. The first step is to identify exactly how their hemianopia has affected their vision. Doctors usually do this by testing a person’s eyes using what’s known as a visual field test. This tests the portion of the person’s surroundings that they can see at any one time.

Researchers have now developed an app to test the visual field of people with Hemianopia – as published in JNNP (Patient Choice; Koaiva and colleagues, 2012):

http://jnnp.bmj.com/content/83/12/1222.full.pdf

The app, called ‘Read-Right’, is part of a free web-based resource for people with hemianopia. To find out if it can be used to help people with hemianopia, researchers used it to test the vision of people with the condition. They also tested their visual field with the conventional test doctors use, called the Humphrey automatic visual field analyser. They then compared how well the tests performed.

What does the new study say?

The Read-Right app was an accurate way of testing people’s visual field.

Read-Right was particularly good for finding parts of people’s vision that hadn’t been affected by hemianopia. It rarely falsely identified people as having problems with their field of vision when they didn’t.

What does this study mean?

If you have concerns about your vision after a stroke, like Wallaby great, Michael Lynagh (Fig 1 above; http://www.dailymail.co.uk/sport/rugbyunion/article-2138304/Michael-Lynagh-mend-stroke.html), or a brain injury, then this app may prove useful to monitor your recovery.

Fig 2: Dr Rob Henderson and Michael Lynagh – dodging the cannonball….

Listen to the poscast with the author:

http://jnnp.bmj.com/content/suppl/2012/12/05/jnnp-2012-302270.DC3

Reference:

Koiava N, Ong Y-H, Brown MM, et al. A ‘web app’ for diagnosing hemianopia. J Neurol Neurosurg Psychiatry 2012;83:1222–4. http://jnnp.bmj.com/content/83/12/1222.full

Dr Susanna Park from the Institute of Neurology, London has created a novel picture of 2012

It is the time of year when reflection on the year that was and anticipation to the year ahead starts to build. The countdown is on to 2013… However it’s hard to capture a year in a statistic, or in a sentence. Far easier to try to visually depict the year and word clouds provide a simple and accessible metric to evaluate content. In this word cloud, all abstracts from JNNP original research papers and reviews from 2012 are summarized with the top 150 most commonly appearing words shown with their size representing the frequency of use. Clearly, 2012 was the year of the patient… What do you think will be in 2013?