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Archive for June, 2014

Traumatic brain injury: a case of too much oxygen?

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.

Seizures and malignant MCS strokes: A real problem!

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
  • Cerebrovascular disease
  • Research paper

Seizures after decompressive hemicraniectomy for ischaemic stroke

  1. C J Creutzfeldt,
  2. D L Tirschwell,
  3. L J Kim,
  4. G B Schubert,
  5. W T Longstreth Jr,
  6. K J Becker

DEAR BLOGGERS, I WILL BE AWAY FOR THE NEXT 2 WEEKS.  WILL CATCH UP AGAIN ON JULY 6TH

 

BULA FROM FIJI:)))

 

Surgery in Normal Pressure Hydrocephalus: Dont wait to shunt!

19 Jun, 14 | by Steve Vucic, Web Editor

Normal pressure hydrocephalus (NPH), a misnomer as the intracranial pressure is increased, may be a debilitating disorder presenting with cognitive decline, gait abnormalities and urinary incontinence.  Surgery may arrest and reverse the neurological deficits.  The consequences of postponing shunting surgery remain unknown, although the eager neurologist may be reassured by the neurosurgeons that there may be time.  In this issue of JNNP, Andren and colleagues dispel this myth and suggest that early intervention may be best and should be performed soon after the diagnosis.  Importantly, all three deficits seemed to be impacted.

 

MUST READ http://jnnp.bmj.com/content/85/7/806.abstract

Neurol Neurosurg Psychiatry 2014;85:806-810 doi:10.1136/jnnp-2013-306117
  • Neurosurgery
  • Research paper

Natural course of idiopathic normal pressure hydrocephalus

  1. Kerstin Andrén,
  2. Carsten Wikkelsø,
  3. Magnus Tisell,
  4. Per Hellström

SHUNTING SHOULD BE PERFORMED ASAP?  What do you think?

From website https://www.google.com.au/search?q=normal+pressure+hydrocephalus&source=lnms&tbm=isch&sa=X&ei=36eiU46ZDcSRkQXbiIDYCQ&ved=0CAYQ_AUoAQ&biw=1680&bih=890#facrc=_&imgdii=_&imgrc=Dxu_fwNRwrhwjM%253A%3BqyTkidWdUVnKaM%3Bhttp%253A%252F%252Fgardenrain.files.wordpress.com%252F2009%252F05%252Fnormal-pressure-hydrocephalus1.jpg%3Bhttp%253A%252F%252Fgardenrain.wordpress.com%252F2009%252F05%252F01%252Fnormal-pressure-hydrocephalus-nph%252F%3B420%3B365

Dystrophin levels in Beckers MD: A cliff effect?

16 Jun, 14 | by Steve Vucic, Web Editor

Becker muscular dystrophy (BMD) is an inherited muscle disorder caused by deficiency of dystrophin. The relationship between disease severity and dystrophin levels needs further assessment. In this issue of JNNP, a Dutch group reported an association between dystrophin level s and disease severity in BD. Specifically, dystrophin levels were not a major determinant of disease severity unless dystrophin levels < 10% in muscles. Interestingly the study suggested that disease severity is more dependent on mutation site. So, where to from here, and what should be our treatment strategies?

Read more http://jnnp.bmj.com/content/85/7/747.abstract

 

Neurol Neurosurg Psychiatry 2014;85:747-753 doi:10.1136/jnnp-2013-306350
  • Neuromuscular
  • Research paper

Dystrophin levels and clinical severity in Becker muscular dystrophy patients

Navigating the sea of rare disease: a roadmap for leukodystrophies

16 Jun, 14 | by Arun Krishnan, Web Editor

Many of you will have seen the movie, Lorenzo’s Oil, with Nick Nolte and Susan Sarandon. It tells of the moving story of a young boy with a serious neurological illness. Despite the considerable difficulties with both diagnosing and treating him, his parents, Augusta and Michael Odone, remain resolute in trying to help their son, Lorenzo. In the end, the movie leaves us with the possibility that there may be a treatment for his condition, a form of leukodystrophy- a group of conditions in which neurological symptoms occur due to metabolic damage to the myelin sheath that insulates nerve fibres.

Lorenzo’s Oil was released in 1992 and despite the 20 years that have followed, there remains no definitive cure for this group of disorders. However on a positive note, there have been huge strides in our ability to characterise and diagnose this group of disorders. The major reason for this is the giant leaps that medical science has made in clinical and molecular genetics. However, as with all rare diseases, the pace of change is so rapid that once in a while you need experts to provide you with a roadmap of how to proceed when you think you are dealing with one of these rare conditions. Regardless of whether you end up dealing with a ‘curable’ condition or not, just being able to provide a clear and definite diagnosis goes a long way to easing the many years or even decades of anxiety and distress that patients and their families experience when a diagnosis is not easily forthcoming.

In this issue of JNNP, Ahmed and colleagues have provided an outstanding review that is useful for any clinical neurologist http://jnnp.bmj.com/content/85/7/770.full) . They have succeeded in providing a clear step-by-step overview of how a neurologist should proceed when a patient presents with potential signs of a leukodystrophy. The article discusses clinical symptoms, MRI characteristics and other useful tests that can help achieve a diagnosis.

This is well-written and easy to follow, with case histories of patients included. A must read for clinical neurologists anywhere.

Treatment of dystonic tremor: Time to get educated.

12 Jun, 14 | by Steve Vucic, Web Editor

Dystonic tremor, such as writer’s cramp, may be a debilitating condition often missed by non-neurology physicians, and even neurologists. Importantly, dystonic tremors may be misdiagnosed leading to unnecessary investigations and delay in effective therapies. In this issue of JNNP, Langs group from Toronto write a superb review on this subject. The review focuses on therapies for dystonic tremor, encompassing pharmaceutical (oral drugs), toxin and surgical approaches. The review highlights important therapies, emphasising the role of botulinum toxin, and provides an nice algorithm for treatment (see below).

 

 

Figure 3

Separating the treatable from the untreatable: a neurologist’s dilemma

11 Jun, 14 | by Arun Krishnan, Web Editor

My last blog (27th May) was all doom and gloom about having to break bad news to patients. It is not always thus in Neurology. Sometimes the converse happens and you are able to give a patient some unexpectedly good news. I saw a patient recently who had significant muscle weakness and wasting and who was thought to have motor neuron disease (MND). She had been unbelievable stoical in the face of such disastrous news and had resolved to spend her final days contributing to research, managing her affairs for her still very young family and to ensuring that she and her husband went on a cruise around the South Pacific before her death.

However, there was something in the back of my mind that was niggling way. Could it be that she did not have motor neuron disease? She seemed to be a little too stable for too long. She had a number of blood tests and other investigations to look for additional possibilities and all had been negative. Now, we must bear in mind that by the time a neurologists reaches this stage, you are holding out very little hope of finding anything meaningful and the diseases that you are trying to exclude are so fine print that their very mention would draw gasps from one’s colleagues.

In this patient’s case, a repeat set of blood tests disclosed one abnormality that had not turned up the first time round, namely the presence of antibodies to glycosphingolipids, immunologically important compounds that are present on the nerve membrane. The presence of these antibodies does not mean that you do not have motor neuron disease but it does cast some doubt on the diagnosis. In this patient’s case, it was enough to convince me to commence the patient on infusions of intravenous immunoglobulin,a treatment for a condition known as multifocal motor neuropathy with conduction block (MMNCB), a condition that can mimic MND very closely. The patient had more nerve conduction studies, but these did not disclose the typical features of MMNCB. Nevertheless, I said to her that this was the last throw of the dice. She was happy to proceed and we commenced the infusions. After one month, no change. After two months, nothing at all. After 6, perhaps a little if you were trying to be optimistic. After 12 months…OK, there was something going on here. She was responding. Now, 18 months down the track it is clear that she never had MND and that her real diagnosis was MMNCB. There were no faults in the diagnostic process by any of the clinicians that she had seen but it was simply a case of her condition refusing to budge, until the nerve had been thoroughly blasted with IVIG. It also illustrates how important it is to remember that all tests have their false positives and false negatives.

MMNCB can be a very tough diagnosis to make. In this issue of JNNP, there is an interesting paper by Nobile-Orazio and colleagues that outlines a new method of serum assay that may yield a higher detection rate of these antibodies and thereby facilitate a diagnosis of MMNCB http://jnnp.bmj.com/content/85/7/754.abstract. This is an important paper, particularly given that MMNCB is a treatable condition.

So how did our patient react to the change in diagnosis? Well, some patients get very overwhelmed and there have been cases where people have sold their home and spent all their money, thinking that they have a terminal illness, only to be told that they need to finance another 20-30 years of life.

In our patient’s case, no such dramas, just unadulterated joy.

Reduction in blood pressure: Does the J curve still talk??

5 Jun, 14 | by Steve Vucic, Web Editor

A ‘J-curve’ association between blood pressure (BP) and stroke has been previously reported, whereby highest and the lowest BPs were associated with increased risks of recurrent stroke among patients with recent ischaemic stroke.  Clearly, such a finding has management implications.  In an upcoming issue of JNNP, the PROGRESS Collaborative Group dismiss this myth.  Namely, large reductions of BP (> 20 mmHg) WERE NOT associated with an increased risk of any types of stroke, with the annual incidence hovering between 2-3%.

 

So I guess, the answer may well be to treat hypertension aggressively, but not to the point of dizziness.

 

Read more:  http://jnnp.bmj.com/content/early/2014/05/14/jnnp-2014-307856.full

 

http://jnnp.bmj.com/content/early/2014/05/14/jnnp-2014-307856/F1.large.jpg

 

Degree of blood pressure reduction and recurrent stroke: the PROGRESS trial

  1. Hisatomi Arima1,
  2. Craig Anderson1,
  3. Teruo Omae2,
  4. Mark Woodward1,
  5. Stephen MacMahon1,3,
  6. Giuseppe Mancia4,
  7. Marie-Germaine Bousser5,
  8. Christophe Tzourio6,7,
  9. Stephen Harrap8,
  10. Lisheng Liu9,
  11. Bruce Neal1,
  12. John Chalmers1,
  13. for the PROGRESS Collaborative Group

J Neurol Neurosurg Psychiatry doi:10.1136/jnnp-2014-307856

Ultrasound of therapeutic utility in essential tremor?

3 Jun, 14 | by Steve Vucic, Web Editor

Essential tremor (ET) may be a debilitating neurological condition, interfering with activities of daily living.  Therapy for ET may be ineffective and remains relatively limited.  In an upcoming issue of JNNP (online first) an innovative methods for treating ET was reported, namely magnetic resonance-guided focused ultrasound (MRgFUS).  This “surgical technique” aims to induce lesion in the thalamus (thalmotomy) as a means of treating ET.  Although surgical thalmotomy and DBA of thalamus are well established, these techniques are invasive.  Importantly MRgFUS appear safe, is  less-invasive and appears to be effective, although more works is needed on patient selection.

 

 

Raad more at:  http://jnnp.bmj.com/content/early/2014/05/29/jnnp-2014-307642.abstract

 

Unilateral magnetic resonance guided focused ultrasound thalamotomy for essential tremor: practices and clinicoradiological outcomes

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Latest from JNNP