23 Jul, 14 | by Steve Vucic, Web Editor
The era of the 20th century, while associated with tremendous progress, has also heralded the era of chronic diseases that were not prevalent in our ancestors. Stress has been portrayed, at least in the lay media and amongst patients, as an important factor in this. Stress is associated with definite physiological changes. In this issue of JNNP, Professor Andrew Lees proposes a link between chronic emotional stress and biological abnormalities that may contribute to development of neurodegenerative diseases such as PD. Importantly novel avenues for research are highlighted, that could yet unlock the mystery of PD.
Read more at: http://jnnp.bmj.com/content/85/8/878.full
The importance of relaxing
22 Jul, 14 | by Arun Krishnan, Web Editor
Of all the medical specialties it may be argued that neurology, more than any other, provides the most exacting test of one’s clinical skill. OK, I am a little biased but that does not mean I’m wrong. I mean, the echocardiogram got rid of the cardiology examination and the respiratory and gastro exams were only ever making up the numbers. And honestly, when your endoscope is your best friend, who cares about anything else. As for rheumatology, well all their patients end up on steroids and methotrexate regardless of the diagnosis so it doesn’t really count and besides when those drugs doesn’t work, you know that the neurology referral can never be far away.
Bravado aside, it is true that until recently, there was very little for a neurologist to go by in terms of making a diagnosis aside from his/her clinical acumen. Even now, neurology clinical meetings are dominated by heated discussion that focuses on ensuring meticulous documentation of clinical abnormalities as a prelude to further investigation and treatment. Even for experienced neurologists, this process is at times difficult. Of all the challenges that are thrown at a neurologist, one of its greatest tests is separating out weakness that is ‘neurological’ from that which is ‘functional’. In other words, working out whether there is a clear neurological diagnosis at hand or whether the symptoms are based in other social and psychological factors, possibly coupled with underlying biological vulnerability.
A few years ago, Stone and colleagues from Edinburgh provided an outstanding review article in JNNP http://jnnp.bmj.com/content/76/suppl_1/i13.extract that served as a comprehensive overview of the presenting features, pathophysiological mechanisms and possible treatment strategies for patients with functional disorders. Table 2 in that review showed the many different ways in which clinicians communicate this diagnosis to patients. The authors provided the novel parameter of ‘number needed to offend’ to demonstrate how poorly some of these communication methods were received by patients. At the top of the list was ‘symptoms all in the mind’, followed by ‘hysterical weakness’ and then ‘psychosomatic weakness’. The paper also provided a range of more effective ways of establishing rapport and helping patients overcome their deficits.
In the most recent issue of JNNP, McCormack and colleagues have taken management of these conditions a step further http://jnnp.bmj.com/content/85/8/895.abstract . They have undertaken a retrospective review of the management of motor conversion disorders in an inpatient neuropsychiatry unit. In contrast to the long-term impairment that we often see in this condition, the authors demonstrated that patients treated in their unit experienced marked improvements in functioning. The authors correctly mention that further prospective studies would be useful in providing a reliable evidence base for the management of these conditions.
21 Jul, 14 | by Steve Vucic, Web Editor
Guillain-Barre syndrome (GBS) is an autoimmune neuropathy with a heterogeneous etiology affecting both the myelin and the axon. GBS is probably the most frequent cause of a rapidly progressive neuropathy in the Western world. Although the demyelinating variant is more frequent in Western forms of GBS in Asia, the axonal form (termed AMAN) is more frequent. The review by Bae and colleagues in this issue of JNNP is an authoritative review on the topic providing novel clinical, physiological, pathological and pathophysiological insights.
Must read at http://jnnp.bmj.com/content/85/8/907.abstract
Guillain–Barré syndrome in Asia
18 Jul, 14 | by Steve Vucic, Web Editor
CIDP is an autoimmune disorder of the peripheral nerves. As its name implies, the disorder is chronic and long-term treatment (therapeutic dependance) may be frequently observed. The factors predicting such treatment dependence need to be elucidated in order to achieve a more appropriate patient management. In this issue of the journal, Rabin and colleagues identify the “treatment-dependent” and ” treatment withdrawal” groups establishing important clinical differences between the groups. Of relevance, therapy with IVIg seems to be associated with the former group while the latter groups was more response to steroids, suggesting differences in the underlying immunopathogenic processes.
Read more at: http://jnnp.bmj.com/content/85/8/901.abstract
Chronic inflammatory demyelinating polyradiculoneuropathy: search for factors associated with treatment dependence or successful withdrawal
- Magalie Rabin1,
- Gurkam Mutlu2,
- Tanya Stojkovic1,3,
- Thierry Maisonobe1,4,5,
- Timothée Lenglet1,4,
- Emmanuel Fournier1,3,4,
- Pierre Bouche1,
- Jean-Marc Léger3,
- Karine Viala1,4
16 Jul, 14 | by Steve Vucic, Web Editor
While pregnancy is associated with a reduced risk of relapses, caution is required in the post-partum period. In this issue of JNNP, Portaccio and colleagues elegantly demonstrate an increased risk of relapse in the post-partum period. Greater disease activity before and during pregnancy were reported as predictors of an increased chance of a relapse in the post-partum period. Therapy with DMDs in the immediate post-partum period seemed to reduced risk of future relapse.
Read more at http://jnnp.bmj.com/content/85/8/845.abstract
Neurol Neurosurg Psychiatry 2014;85:845-850 doi:10.1136/jnnp-2013-306054
Postpartum relapses increase the risk of disability progression in multiple sclerosis: the role of disease modifying drugs
- Emilio Portaccio1,
- Angelo Ghezzi2,
- Bahia Hakiki1,
- Andrea Sturchio1,
- Vittorio Martinelli3,
- Lucia Moiola3,
- Francesco Patti4,
- Gian Luigi Mancardi5,
- Claudio Solaro6,
- Maria Rosaria Tola7,
- Carlo Pozzilli8,
- Laura De Giglio8,
- Rocco Totaro9,
- Alessandra Lugaresi10,
- Giovanna De Luca10,
- Damiano Paolicelli11,
- Maria Giovanna Marrosu12,
- Giancarlo Comi3,
- Maria Trojano11,
- Maria Pia Amato1,
- for the MS Study Group of the Italian Neurological Society
14 Jul, 14 | by Arun Krishnan, Web Editor
Multiple sclerosis (MS) for most people engenders troublesome images of young people with disability, in wheelchairs, unable to care for themselves. The mere mention of the term elicits these fears. Surprisingly, the potential MS-mimic conditions tend to get a less fearful response. In my own practice, I have noticed that telling patients that their symptoms could be due to rheumatoid arthritis, Sjogrens Syndrome or systemic lupus erythematosus (SLE) does not have the same impact and yet we know that neurological involvement in those disorders can be disastrous, often much worse than MS.
So, why is it so? I guess MS is common and many people will know of someone or will have seen images of someone with MS, most of which will depict some degree of disability. Yes, MS is disabling but it is also now increasingly treatable, with numerous agents on the market and even more in clinical trials. The sobering aspect to most of this is that they all generally do the same thing-reduce clinical relapses and hopefully make the MRI scan look a little prettier. What does this do for long term disability? Well, we don’t really know. What we do know is that these drugs act by reducing inflammation but many years down the track, inflammation will stop and neurodegeneration will set in. We also now have increasing evidence that neurodegeneration-the process of gradual loss of brain cell populations-may start very early in the disease. The race is on to find a drug that can act on neurodegeneration, thereby stopping the very process that causes long-term physical disability.
In this issue of JNNP, there is a very interesting paper that provides a ray of hope in that regard. Fillipi and colleagues ( http://jnnp.bmj.com/content/85/8/851.abstract )have demonstrated that a new oral medication for MS, laquinimod, is associated with a reduction in the loss of brain volume that can accompany MS. This drug has been previously shown to reduce disability progression and this study was aimed at working out how it exactly did this. This is an important study as it is now widely accepted that loss of brain volume may be a marker of neurodegeneration and this measurement is now increasingly being used to assess the effectiveness of newer MS drugs.
What does this mean for patients? To answer that question, long-term follow-up will be needed focussing on patient-orientated parameters, particularly measures of physical function. In the meantime, this study provides hope that we may be entering an exciting new phase in the quest for effective treatments for MS.
10 Jul, 14 | by Steve Vucic, Web Editor
Frontotemporal dementia (FTD) is a devastating neurodegenerative disorder predominantly affecting the frontal and temporal regions of the brain, although the pathology may be more general. In the absence of a diagnostic test, the diagnosis of FTD remains clinically and pathologically based. Recently, novel clinical and pathological criteria have been developed to encompass the heterogeneous nature of FTD. In this issue of JNNP, Chare and colleagues report on the validity of these new criteria in a large international cohort of FTD/dementia. In this must read manuscript, the pathological validation of criteria was established and clinical features such as disinhibition, food preferences and naming, though to be diagnostically useful, did not differentiate different forms of FTD.
Read more at: http://jnnp.bmj.com/content/85/8/865.full
New criteria for frontotemporal dementia syndromes: clinical and pathological diagnostic implications
- Leone Chare1,2,
- John R Hodges1,2,3,
- Cristian E Leyton1,3,
- Ciara McGinley4,
- Rachel H Tan1,
- Jillian J Kril4,5,
- Glenda M Halliday1,2
1 Jul, 14 | by Arun Krishnan, Web Editor
I have often wondered about the fate of those patients that I see once or twice and then who seem to vanish into the wilderness. I have occasionally seen some of these patients at a shopping centre or walking down the street, and I must admit to a sense of relief that they are ambulant, functioning and not appearing terribly disabled. However, these patients are in the minority. In most cases, you either see a patient at regular intervals or you just never see them again.
So, what happens to these patients? Well, given how busy clinical practice can become, doctors don’t have the time and possibly the energy to think about all those patients who got away. May be they are fine, perhaps they are not and decided to look elsewhere for a solution to their problem. In any case, you don’t really know if you got it right or if you got it wrong. Personally, I am sure that I make many mistakes and that sometimes these are inconsequential and therefore overlooked by patients, while at other times perhaps they do result in some distress for the patient. The issue for me is feedback from my colleagues. I would love to know how and why I got something wrong as that is the only way to improve. But from my personal experience I know that it is difficult to tell a colleague that he/she got the diagnosis wrong, unless they have specifically requested your opinion regarding a patient. So, without knowing what happened you really can’t work on getting better at what you do.
In this JNNP podcast, we hear from Professor Martin Samuels (1). He is a highly distinguished neurologist from Harvard. He talks to Professor Huw Morris on clinical decision making, the ‘benefits’ of making mistakes and apologising to patients when you get it wrong.
- https://soundcloud.com/bmjpodcasts/how-neurologists-think-what-my-errors-taught-me?utm_source=feedburner&utm_medium=feed&utm_campaign=Feed%3A+pn%2Fpodcasts+%28Latest+from+PN+podcasts%29&g=w_jnnp_podcasts_sidetab) .
24 Jun, 14 | by Arun Krishnan, Web Editor
The term itself is scary enough-“traumatic brain injury” (TBI). What exactly does it mean? Well, it is possibly the commonest cause of death and disability worldwide. It refers to neurological symptoms that occur as a consequence of sudden trauma to the brain. It is most commonly due to a direct blow to the head, but some authorities also include other accidents in this definition, such as episodes of acute acceleration/deceleration which lead to brain injury, such as being thrown from a moving object. The symptoms vary significantly from one individual to another. A lot of sufferers who survive the acute injury develop chronic symptoms that may last a lifetime and for which there is no definite cure. These symptoms include changes in cognition, impaired mobility, problems with concentration and changes in personality and mood. These impairments affect not only the patient, but may have significant impact on friends and family. What is even more distressing for patients, is that these deficits cannot be directly measured and there is no simple test that tells the patient how severe the damage is or what the prognosis is likely to be. While some cases of TBI are mild and resolve, other patients have very serious brain injury which may affect long-term function and, in acute cases, possibly even survival.
In these severe cases, there has been considerable research on how these patients should be managed in the setting of an intensive care unit. In this issue of JNNP, there is a very important paper by Rincon and colleagues that looks at the role of oxygenation in determining the outcome of TBI http://jnnp.bmj.com/content/85/7/799.full . As noted in the accompanying Editorial http://jnnp.bmj.com/content/85/7/711.full , one of the principles of intensive care management is the provision of adequate oxygenation for the patient. The study by Rincon et al however explores an alternative possibility in TBI: what if we are doing damage by giving too much oxygen? Their results provide evidence that high levels of oxygen were associated with a worse outcome in patients with severe TBI. Why is this case? They suggest that providing high levels of oxygen may actually result in a reduction in oxygen delivery to the brain due to changes in cardiac output and that these reductions may impair the metabolic activities of neurons.
This is a large multi-centre study with interesting conclusions. Importantly from a clinical perspective, the study has implications for how we manage patients with severe TBI.
22 Jun, 14 | by Steve Vucic, Web Editor
The risk of seizures after structural brain lesions, such as strokes, would be expected to be high, although this has not been studied in detail. Clearly, such issues would have great bearing on patient management. In this issue of JNNP, the risk of seizures in patients post malignant MCA territory strokes that underwent decompressive hemicraniectomy was reportedly high, impacting on patient recovery and management. None-the-less, all patients would have chosen surgery irrespective of seizure risk. These issues should be discussed with families of patients who are in this predicament, putting a brighter picture on outlook.
Read more at : http://jnnp.bmj.com/content/85/7/721.abstract
J Neurol Neurosurg Psychiatry 2014;85:721-725 doi:10.1136/jnnp-2013-305678
Seizures after decompressive hemicraniectomy for ischaemic stroke