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Archive for May, 2014

Parkinsons disease: managment includes more than just treating the motor symptoms

30 May, 14 | by Steve Vucic, Web Editor

Parkinson’s disease is a slowly progressive neurodegenerative disorder of the exptrapyramidal system. Clinically, PD is regraded as a predominantly motor disorder, and the current therapies are aimed at managing this aspect of PD.  However, the PD population is elderly and so often, a holistic approach, some may call it a “geriatric approach”, is not heeded by the managing team resulting in morbidity and prolonged hospitalization.  In an upcoming issue of JNNP, Lubomski and colleagues address the issue of co-morbidities in PD in a large Australian cohort.   Specifically, PD patients were  more likely to suffer serious health problems, including delirium, adverse drug reactions, syncope, falls and fractures when compared to non-PD patients.  Clearly, the health system needs to be resources to address such issues, and it may be time for neurologist to rest away much needed resources from the geriatric discipline.

 

 

Read more:  http://jnnp.bmj.com/content/early/2014/05/29/jnnp-2014-307822.abstract

 

Hospitalisation and comorbidities in Parkinson’s disease: a large Australian retrospective study

Breaking bad (news): doctors and the conversations they don’t want to have.

27 May, 14 | by Arun Krishnan, Web Editor

I am writing this midway through a Tuesday, meaning that I am about 30% through my working week. How is it going, you ask? Well, it’s all relative isn’t yet? In the last 24 hours, I have had to break some really terrible news to two young patients, both in their mid 30’s, one of whom clearly now has motor neuron disease and the other who has Huntington’s Disease. To say they did not expect this news would be a huge understatement. Neither had a family history, even the patient with Huntington’s Disease, yet both have now received awful diagnoses given that both conditions are incurable and fatal. What do I feel, having delivered this news….well, more on that later. These diagnoses were reached after a long period of investigation and follow-up. Neither had the classical phenotype associated with the disease and both were initially diagnosed as having treatable conditions. Problem was that neither responded to treatment, hence the re-evaluation, hence the prolonged agony (for the patients, not for me I hasten to add).
For anyone who has ever seen a neurologist and who has wondered what makes them tick, here is the answer: neurologists are intellectual junkies. We like the thrill of the chase, we love that feeling of nailing down that once elusive diagnosis. The more rare the disease, the greater the intellectual satisfaction. Eponymous syndromes, new diseases that have not yet been delineated, rare genetic disorders that the medical literature forgot….all fair game.  At the end of that diagnosis however, there is a person and while many patients are relieved that a diagnosis is finally reached, all will carry daily physical, cognitive and functional reminders of the intellectual adventure that I, as the neurologist, decided to undertake.
So, how did my consultations with these patients turn out? Well, both patients were accompanied by family and no one, not even the patient or his parent, spouse or child shed even a single tear. There were questions about prognosis, there were questions about magical cures, there were questions about whether I was sure of what I was saying. The answers were: fatal, no and yes.
When it came to my role in this process, there was only one question left? Was I empathetic? The answer I am afraid will never be known but I would not be surprised if I was slightly deficient in that department. I mean, I did all the things they tell you in medical school: don’t deliver news across the table, pull your chair closer to the patient’s, maintain eye contact, be sincere, no prolonged introduction, short, to the point…give time for the news to sink in and stay on message. Be honest.
A recent article from a Canadian centre has assessed the level of empathy demonstrated by young doctors in the delivery of news regarding the diagnosis of motor neuron disease (1).  The major finding was that there are issues with the way in which doctors are taught to deliver bad news. The study reflects previous work that has demonstrated dissatisfaction with how doctors deliver bad news to patients (2).  Is the problem with medical education or is it doctors who are desensitized to these awful scenarios?

1. Schellenberg KL, Schofield SJ, Fang S, Johnston WS. Amyotroph Lateral Scler Frontotemporal Degener. 2014 Mar;15(1-2):47-54. doi: 10.3109/21678421.2013.843711. Epub 2013 Nov 18. Breaking bad news in amyotrophic lateral sclerosis: the need for medical education.
2. McCluskey L, Casarett D, Siderowf A. Amyotroph Lateral Scler Other Motor Neuron Disord. 2004 Sep;5(3):131-5. Breaking the news: a survey of ALS patients and their caregivers.

Radiation induced myopathy: Beware of it?

26 May, 14 | by Steve Vucic, Web Editor

Radiation induced tissue damage is an important but under recognized issue in neurology.  Radiation therapy usually induces central nervous system pathology manifesting as “tumor” like lesions.

Less recognized are disorders of the peripheral nervous system, which radiation therapy could also injure.     In an upcoming issue of JNNP, Ghosh and Milone report an incidence of ~10% of radiation induced myopathy in a cohort of cancer patients.  Interestingly the most common clinical manifestation was head drop, reflecting the area of radiation (neck and upper torso).  EMG disclosed myopathic changes in axial muscles (which could be hard to interpret) while myopathy was evident in 50% of muscles on pathology.  Of concern, RIM may be delayed as long as 15 years, and consequently a through history is required in all patients presenting with head drop to glean a history of radiation therapy.  Clearly, radiation protocols need to be improved to prevent this debilitation side-effect.

 

Read more at:  http://jnnp.bmj.com/content/early/2014/05/16/jnnp-2013-307447.abstract

 

 

J Neurol Neurosurg Psychiatry doi:10.1136/jnnp-2013-307447
  • Neuromuscular
  • Research paper

Clinical and laboratory findings of 21 patients with radiation-induced myopathy

 

Alemtuzumab: The new effective and safe frontier in active RRMS?

23 May, 14 | by Steve Vucic, Web Editor

Alemtuzumab is a recently licensed monoclonal antibody directed against the CD52 antigen on lymphocytes.  The efficacy of alemtuzumab has been established in phase II and III studies for RRMS, with spectacular reduction in relapse rates, although the disability reduction was better in the Phase II studies, where a reversal of disability was noted.  Clearly, alemutuzumab is quiet effective, with beneficial effects most evident in patients with active disease.  The fact that the drug may need to be administered once or twice in a lifetime has appeal and it has the potential to further alter the MS landscape.

In an upcoming issue of JNNP (Online first), Professor Coles’ group reports on the longer-term experience of the efficacy and safety of alemtuzumab in active RRMS.  As with trials, there were impressive reductions in disability accumulation and relapse rates, with majority of active RRMS patients stabilizing.  Furthermore,  alemutuzumab appeared safe, with autoimmune thyroid disease the major side-effect.

 

Read more on this back to the future medication at: http://jnnp.bmj.com/content/early/2014/05/21/jnnp-2014-307721.abstract

 

Neurol Neurosurg Psychiatry doi:10.1136/jnnp-2014-307721
  • Multiple sclerosis
  • Research paper

Alemtuzumab treatment of multiple sclerosis: long-term safety and efficacy

Neuroimaging in dementias: An important diagnostic aid!

21 May, 14 | by Steve Vucic, Web Editor

Althgally the diagnosis of dementias remains clinically based, neuroimaging techniques have been increasingly utilised as diagnostic aids.  While “older” or more conventional techniques have been implmenented to exclude “mimc” disorders, newer techniques have been implemented to identify patters of atrophy or metabolic abnormalites that could aid in the type of dementia.  In this issue of JNNP, an elegant review highlighting the currently utilised techniques identifying patterns of atrophy, T1 and T2 lesions etc, puts neuroimaging in specific focus.  Helpful alogartithms have been proposed to guide the reader.  A potential role for “metabolic” imaging (SPECT and PET) is also discussed.

You can read more at http://jnnp.bmj.com/content/85/6/692.full

 

An algorithmic approach to structural imaging in dementia

Editor's Choice

  1. Lorna Harper1,
  2. Frederik Barkhof2,
  3. Philip Scheltens3,
  4. Jonathan M Schott1,
  5. Nick C Fox1

Managing epilepsy: a challenge for the neurologist

16 May, 14 | by Arun Krishnan, Web Editor

Managing epilepsy is hard work. The detailed history of events, trying to work out if each seizure is the same type of event or if there are many different semiologies… not easy, not easy at all. Then there is the issue of anticonvulsants and monitoring not only their effectiveness, but also their potential adverse effects. We know about teratogenicity and we are learning more and more about bone health and fertility. However, of all the aspects of epilepsy management, it is the effects of the condition on a patient’s independence that often takes up a large part of the consultation. Only the other day, I spent a substantial period of two successive consultations in two different patients, simply listening while two young patients with epilepsy proceeded to tell me how unfair life was, or more specifically how unfair it was that they were no longer able to drive. One patient wondered if a second opinion from an epileptologist may result in them driving sooner. No, it won’t.

Then, there is the perennial question: “how long do I have to take these tablets for?”. For that question, Bonnett and colleagues http://jnnp.bmj.com/content/85/6/603.full have provided an important set of data. They have analysed data from the Standard and New Antiepileptic Drug Study (SANAD arm B) and have provided models that may help predict the chance of attaining seizure remission in a 12-month period. Variables that may be predictive include a family history of epilepsy, the number of seizures, seizure type and the treatment used. For a condition that has such a significant and serious impact on quality of life, this study provides important and clinically relevant answers that can be used in the day-to-day management and counselling of patients with epilepsy.

Single nerve conduction study in GBS: is it sufficient?

15 May, 14 | by Steve Vucic, Web Editor

The diagnosis of GBS is a clinically based.  Nerve conduction studies are utilized to confirm the type of GBS, i.e. demyelinating versus axonal.  Serial nerve conduction studies have been proposed as a means of confirming whether an”apparently” axonal form of GBS represent distal demyelination.  Such confirmation has prognostic implications for the patients.  In addition, previous studies have suggested a limited utility of NCS in early GBS (within 10-14 days of symptom onset).  In a retrospective study, Nicolas’ group suggested that single NCS may be sufficient to establish a diagnosis of GBS, even in the early stages.  These results are interesting and somewhat contradictory  to the existing literature.

 

Read more at:  http://jnnp.bmj.com/content/early/2014/05/09/jnnp-2014-307815.abstract

 MY VIEW:  BEST TO REPEAT STUDY IN 4-6 WEEKS.  What do you think???

Research paper: Electrophysiological diagnosis of Guillain–Barré syndrome subtype: could a single study suffice?

  • Yusuf A Rajabally,
  • Marie-Christine Durand,
  • James Mitchell,
  • David Orlikowski,
  • Guillaume Nicolas

J Neurol Neurosurg Psychiatry Published Online First: 9 May 2014 doi:10.1136/jnnp-2014-307815

 

Alcohol and seziures: A bad mix!!

13 May, 14 | by Steve Vucic, Web Editor

Alcohol fulled violence and head trauma has potentially devastating medical & social impacts.  The development of new-onset seizures post head trauma is well recognized, although it has been poorly researched.  In the June issue of JNNP, a Finnish group tackle this vexing issue. In a large database study, the authors report on the risk factors for new-onset seizures following head-related violence.  Importantly, new-onset seizures appeared to be common, occurring in 5.7% of the cohort.  Alcohol-related index injury , moderate-severe brain injury and premorbid psychiatric disease all predisposed to new-onset seizures.  Given these findings, social and medical strategies aimed at curbing alcohol related violence/head trauma are clearly warranted and are important.

 

READ MORE AT:  http://jnnp.bmj.com/content/85/6/598.abstract

 

  • Research paper

Predictors of new-onset seizures: a 10-year follow-up of head trauma subjects with and without traumatic brain injury

Intracerebral haemmorhage (ICH): Grave prognosis indeed

11 May, 14 | by Steve Vucic, Web Editor

Strokes are invariably a clinical biomarker of “unwellness” of the central nervous system leading potentially life long disability and shorter survivial.  While there is good prognostic data peratining to ischeamic strokes, that of ICH remains unknown.  As a neurology trainee it was implied, largely from consensus, that ICH strokes exhibit a better prognosis. In this issue of JNNP, Poon and colleagues performed a meta analysis on the very important topic of prognosis following an ICH.  Importantly, this meta-analysis reports on the grave prognosis of these types of strokes with a 46% 1 year and 29% 5 year survivial.  In addition, risk factors for adverse prognosis following ICH were also reported.  Clearly we, as neruologist, need to develop better management strategies for this devstating type of stroke.

 

 

Read more:  http://jnnp.bmj.com/content/85/6/660.abstract

 

Autologous haematopoietic stem cell transplantation in CIDP: Taking a hammer to the walnut?

8 May, 14 | by Steve Vucic, Web Editor

Chronic inflammatory demyelinating neuropathy (CIDP) remains a difficult disorder to treat, particularly when patients fail to respond to one of the first line therapies.  In addition, complications relating steroid and impressive treatments remain high    and disability for this disorder is invariably a difficult management problem.  In the June issue of JNNP, the utility of autologous stem cell transplantation was investigated.  In a small study Press and colleagues report on clinical improvement in CIDP patients 2-6 months after therapy.  However, a substantial proportion relapsed requiring further treatment and the complications were potentially serious, including pancreatitis and hemorrhagic cystitis.

The question of therapeutic utility remains, particularly selecting the appropriate patient.  Further studies are clearly needed, but none-the-less this is a must read article for the neuromuscular physicians.

 

http://jnnp.bmj.com/content/85/6/618.abstract

 

Latest from JNNP

Latest from JNNP