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Archive for January, 2014

The New Wonder Drug

30 Jan, 14 | by Emma Foster

I’m currently working as a junior doctor in a subacute hospital, on the Neurology Ward. Our in-patients come in for symptom management, and sometimes end of life care. Conditions include motor neuron disease, Huntington’s disease, multiple sclerosis, and the Parkinson Plus ‘three ugly sisters’ (although I’ve only seen MSA & PSP in my time here).


What a terrible group of diseases. There’s nothing like working on this ward to make you want to find a cure, or at least a way of slowing down the often rapid progression of these debilitating life-sentences. Given the lack of cures on offer, quite a few patients have gone out to alternative health providers, and are taking a large range of herbal supplements, crystals, homeopathy, magnetic therapy…. the list is endless. Understandably, they will do everything in their power to change the course of the relentless disease.


I admitted a patient yesterday, with MND. Confined to a wheelchair, with non-invasive ventilator support for up to 18 hours a day, he asked whether I had “a pill to make me stronger”. I said that I didn’t. “How about steroids?” At first I thought he meant ‘roids, the sort body-builders take. “No, not those” – he clarified – steroids, like his wife took for her fibromyalgia. She was weak, then took steroids and became better. I explained the different pathologies and that although we knew steroids helped fibromyalgia, we hadn’t yet found something that effective for MND. “How about stem cell therapy? The Government is trying to put a stop to that, aren’t they?” I acknowledged that stem cell therapy was a field of ongoing research, with potential, but as yet it was not the standard of care. I hadn’t heard that the Government was trying to put a stop to it; privately I thought they were busy enough stopping The Boat People, Gay Marriage, and Sharks Off The Coast of Western Australia, and probably didn’t have enough time to stop stem cell therapy too. “Well”, my patient finally said, “I take these from a Chinese medicine doctor”. ‘These’ turned out to be six large bottles full of very large pills, of uncertain vintage, uncertain ingredients, prescribed at frequent intervals. “They helped for a bit, but then started to not work as well, which he said could happen, so he’s now doubled the dose. Do you think they work?”


Reading the excellent article by Dr Colin J Mumford (JNNP 2014)1, titled ‘When patient opinion and clinical science are implacably opposed: the view form an MS specialist’, highlights that this challenge is faced the world-over by neurologists (and all doctors) when treating people with devastating diseases. Given a realistic (often bleak) outlook from us, people seek out sources they’re comfortable with: YouTube, tabloids, and current affair programs of varying credibility. Anecdotes about ‘my father’s-golf-partner’s-step-son’s-friend’ and ‘a-friend-of-my-aunt-who-lives-overseas’ speaks at a level understood by our patients far better than do our elite journal publications. Access to these journals, both in terms of knowing how to get a copy, and then understanding the content, is difficult without some sort of scientific or medical background.


I feel we have a duty to spend time with patients, ask about other treatment options they’ve explored, and where possible, discuss why our brand of medicine does not routinely offer these. If there’s scope, maybe even discuss why we set up trials the way we do, why we trust the results of multicentre double blinded RCTs more than anecdotes, and why we receive even our own ‘novel’ treatments with a degree of scepticism.


Perhaps go even further, and as Mumford urges, that we address these issues in a much larger forum; that we “embrace opportunities to exploit radio, TV and the internet whenever possible”. Have you got an opportunity to set up information sessions for groups of your patients? Perhaps there’s a support network with regularly meetings that you can attend to discuss new and upcoming treatments? Or maybe you can do it online, via FaceBook groups or other social networking pages designed for patients? Write in to the local current affairs program that has espoused the virtues of the latest wonder drug – give the ‘other’ view.


1 – ‘When patient opinion and clinical science are implacably opposed: the view from an MS specialist’, Mumford, CJ. J Neurol Neurosurg Psychiatry 2014;85:125-126 doi:10.1136/jnnp-2012-303857

Feeling hot hot hot!

17 Jan, 14 | by Emma Foster

Happy new year!

I’m typing this blog on the living room floor, under the air con.  The south-east of Australia is in the middle of a heat wave; my home town had four consecutive days with temperatures over 40 degrees Celsius (that’s over 104 degrees Fahrenheit for those playing in other parts of the world).

Bit of a celebration tonight though; the cool change has come through – currently 30 degrees outside (86F).


While admitting person after person for heat-related problems, I wondered what sort of neurological issues could arise in cases of extreme heat.


Source:   Cyclist helping a local avoid heat stroke.



Here is a hot selection of articles from the archives of JNNP.
First, a beautifully written introduction to heat stroke, from Lorenzo et al:

“The most severe condition among the group of heat stress disorders is heat stroke (HS). It may be divided into two forms: classic & exertional. The former is due to environmental heat exposure and characterised by hyperthermia (above 40C) and neurological dysfunction (headache, delirium, convulsions and coma). Exertional HS affects subjects performing strenuous exercise in heat environmental conditions and is characterised by more severe symptoms than the classic form. Residual neurological defects have been reported in up to 20% of exertional HS survivors”. 1

This article continues on to describe the case of a 45 year old female who suffered exertional HS after hiking for several hours during the middle of a very hot day. She presented to hospital with abrupt loss of consciousness, and after being cooled, she gradually came to over several hours. She had motor coordination impairment and headache; the former recovered by time of discharge, but the headache continued on.

18 months later she was seen in an outpatient headache clinic, with ongoing daily headaches, not relieved with various NSAIDs, steroids or tramadol. After some adjustment of medications, the headache was successfully treated with amitriptyline and gabapentin, and in time these were successfully tapered off. The authors conclude that the case represents new daily persistent headache, possibly induced by heat stroke.



Delving back to almost 30 years ago, I uncovered this piece: ‘Spinal cord lesions in heat stroke’2, from Spanish authors Delgado et al. This is a case study of a 66 year old man who died 15 days after suffering heat stroke. The gentleman was an in-patient in a psychiatric unit, and had endured a heat wave with temperatures up to 38 degrees, with no air conditioning. He came to medical attention after three hours of remittent right-sided somatomotor partial seizures with secondary generalisation, that resolved with IV clonazepam. Initial neuro exam was normal except for moderate drowsiness & mental confusion. CT scan & CSF were normal, and EEG recording was diffusely slowed (5 Hz).   12 hours after admission, he developed quadreplegia, needed intubation & ventilation, but had preservation of cranial nerves & conscious state. Throughout the duration of his admission he was not seen to sweat, requiring ongoing physical cooling.

He remained just so for 14 days, when he suddenly died.

Pathology results revealed severe lesions in the anterior and intermedio-lateral horns of the spinal cord. As this patient’s only apparent insult was hyperthermia, it is suggested that the motor neurons & vegetative neurons of the spinal cord are specially sensitive to hyperthermia. A very interesting read, including figures of histology slides.
‘Downbeat nystagmus from heat stroke’3 is a letter to the editor documenting the case of a previously well 22 year old man who collapsed during a run. On presentation, he had a core body temperature of 39 degrees and was incoherent and combative. Several days into admission he complained of blurred vision, and was found to have downbeat nystagmus, associated with a midline cerebellar syndrome. MRI with DWI was normal. LP was normal. Thiamine and magnesium did not improve the nystagmus. The patient was lost to follow up, however, the authors felt it may be that the vermal cerebellum and vestibulocerebellum may be particularly susceptible to thermal injury.


So, for those fellow Aussie heat-wave sufferers, keep cool, invest in air-conditioning, and avoid strenuous activity in the heat of the day.

Go to Journal Club in your lunch break instead.


1) Lorenzo, C. Ambrosini, A. Coppola, G. et al. Heat stress disorders and headache: a case of new daily persistent headache secondary to heat stroke. JNNP. 2008; 79: 610-611.

2) Delgado, G. Tunon, T. Gallego, J. a Villanueva, J. Spinal cord lesions in heat stroke. JNNP. 1985; 48: 1065-1067.

3) Van Stavern, G. Biousse, V. Newman, N. Downbeat nystagmus from heat stroke. JNNP. 2000; 69: 403-404.

Below the surface

7 Jan, 14 | by Professor Matthew Kiernan, Editor of JNNP


Dr Susanna Park reviews the recent JNNP hits:


Condensed in this word cloud is the year 2013……as seen in the Journal of Neurology, Neurosurgery & Psychiatry.


From the abstracts of original research papers and reviews from the Journal in 2013, the 150 most commonly used words are depicted graphically, with the most frequently appearing words larger in size.

The single most common word for 2013 is again ‘patients’– with the most commonly studied patient groups being stroke, Parkinson’s disease, multiple sclerosis, amyotrophic lateral sclerosis and frontotemporal dementia. However, looking deeper, a substantial number of rare disorders were also included in the Journal in 2013 – including multiple system atrophy1, Bickerstaff brainstem encephalitis2, logopenic progressive aphasia3, and mitochondrial disorders4.

Across this broad clinical spectrum, a common feature was the focus on cognition and cognitive impairment – assessed in at least 15 different patients groups and in healthy ageing. Articles in the Journal covered aspects of cognition from the predictive power of initial cognitive deficits in stroke recovery and return to work5 to the cognitive dysfunction of impaired deception and lie production in Parkinson’s disease and essential tremor6 and the role of the Mediterranean diet in improving cognition in older adults7.

What do you think are the hot topics of 2013 and how will these develop in 2014?


1. Bjornsdottir A et al. (2013) Incidence and prevalence of multiple system atrophy: a nationwide study in Iceland. JNNP 84(2): 136-140.

2. Shahrizaila N & Yuki N. (2013) Bickerstaff brainstem encephalitis and Fisher syndrome: anti-GQ1b antibody syndrome. JNNP 84 (5): 576-583.

3. Whitwell JL et al. (2013). Elevated occipital β-amyloid deposition is associated with widespread cognitive impairment in logopenic progressive aphasia. JNNP 84(12): 1357-1364.

4. Pitceathly RD et al. (2013). Distal myopathy with cachexia: an unrecognised phenotype caused by dominantly-inherited mitochondrial polymerase γ mutations. JNNP 84 (1):107-110.

5. Kauranen T et al. (2013). The severity of cognitive deficits predicts return to work after a first-ever ischaemic stroke. JNNP 84 (3):316-21.

6. Mameli F et al. (2013). Lies tell the truth about cognitive dysfunction in essential tremor: an experimental deception study with the guilty knowledge task. JNNP 84(9): 1008-1013.

7. Martinez-Lapiscina EH et al. (2013). Mediterranean diet improves cognition: the PREDIMED-NAVARRA randomised trial. JNNP 84(12):1318-25.

Latest from JNNP

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