Practical Neurology current issue

Highlights from this issue

Smith, P., Fuller, G. — 2012-03-26T05:16:06-07:00

When faced with a difficult problem what do you do: you have looked at the evidence and still you are unsure? Do you perhaps wonder what Dr So-and-so or Professor Whatsit would do? One of the privileges of editing Practical Neurology is that we can ask So-and-so and Whatsit to tell us just what they would do and why. In this edition, we have indulged ourselves richly.

Chris Allen lets us listen in on his teaching on page 97. We anticipate that most readers will find his teaching tips interesting and useful. We asked Simon Kerrigan and Robin Grant to explore the difficult dilemmas and decisions involved in managing patients presenting with low-grade gliomas on page 72. John Paul Leach tells us on page 103 how he tackles the important issue of discussing sudden unexpected death in epilepsy (SUDEP), in the context of...

Low-grade brain tumours and seizures

Kerrigan, S., Grant, R. — 2012-03-26T05:16:06-07:00

Introduction

Up to 80% of people with low-grade brain tumours (WHO Grade I and II) experience seizures at some time during their illness, compared with up to 60% of people with high-grade tumours (WHO Grade III and IV).1 For most patients with low-grade tumours, seizures are the presenting complaint and in approximately half of cases the seizures are refractory to medical treatment. We have used two cases to illustrate some dilemmas and difficulties arising from the management of low-grade brain tumours presenting with seizures.

Case 1

Patient 1 suffered a generalised tonic-clonic seizure while driving. He attended the emergency room and had an unenhanced CT brain scan (figure 1). This showed a low density lesion in the right posterior frontal lobe. The lesion appeared to spare the grey matter but with a suspicion of vasogenic oedema and an underlying mass lesion; thus,...

Improving the diagnostic accuracy in parkinsonism: a three-pronged approach

2012-03-26T05:16:06-07:00

Separating Parkinson's disease from the various causes of atypical parkinsonism (AP) is a common and clinically relevant challenge in clinical practice. Distinguishing between the different causes of AP is even more difficult. Here the authors discuss a systematic, clinically based and three-pronged approach that can assist clinicians in establishing the correct diagnosis in the consulting room. The three consecutive steps include: (1) to verify that the clinical syndrome truly represents parkinsonism (hypokinetic–rigid syndrome); (2) to search systematically for ‘red flags’ (alarm signs that may signal the presence of AP); and (3) to integrate these two steps, as a basis for a narrow differential diagnosis and a guide for further ancillary tests.

Seizures after head injury

Smith, P. — 2012-03-26T05:16:06-07:00

This remarkable study1 from Minnesota describes the clear relationship between brain injury severity and the relative risk (RR) of post-traumatic epilepsy among 4500 head injured patients ascertained over 50 years (1935–1984). Many practitioners know Annegers' bottom line by heart: mild injury (loss of consciousness or post-traumatic amnesia of less than 30 minutes) RR 1.5; moderate (30 min to 24 h or skull fracture) RR 2.9; severe (>24 h or subdural haematoma or brain contusion) RR 17.0. Missile head-injured patients are separate and at greatly increased risk. Despite difficulties in translating data from a bygone era to one of detailed imaging and intensive management, this landmark work will long justify its top slot for both neurologists and neurosurgeons.

Provenance and peer review

Not commissioned; internally peer reviewed.

New diagnostic criteria for Alzheimer's disease and mild cognitive impairment for the practical neurologist

Budson, A. E., Solomon, P. R. — 2012-03-26T05:16:06-07:00

Four articles in the journal Alzheimer's and Dementia in 2011 describe new criteria for Alzheimer's disease (AD) dementia and mild cognitive impairment (MCI) due to the AD pathophysiological process (MCI due to AD), as well as the underlying rationale for them. The new criteria also include preclinical AD criteria but these are intended purely for research purposes. The new criteria emphasise that the AD pathophysiological process starts years and perhaps decades before clinical symptoms, and that biomarkers can detect amyloid β deposition and the effects of neurodegeneration in the brain. The criteria are recommendations based upon consensus meetings and will require future validation. Nonetheless, the authors believe that they are immediately helpful to the practising clinician, providing more accurate and specific guidelines for the diagnosis of AD dementia and MCI due to AD. As new diagnostic tools and treatments for AD become available, diagnoses using these criteria will enable patients with AD dementia, MCI due to AD and eventually preclinical AD to receive the best possible care.

Teaching clinical neurology

Allen, C. — 2012-03-26T05:16:06-07:00

‘No bubble is so iridescent or floats longer than that blown by the successful teacher’

‘The successful teacher is no longer on a height, pumping knowledge at high pressure into passive receptacles...he is a senior student anxious to help his juniors’

Sir William Osler (1849–1919)

‘Neurophobia’ has been a subject of interest over the years since Jozefowicz coined the term in 1994.1 Neurophobia is at least partly caused by inadequate neurology teaching, teaching that may be inadequate in quantity and/or quality.2 To improve the quantity of neurology education neurologists must get involved in the organisation of curricula in their medical school, as some have done.3 To improve the quality of their teaching neurologists need to reflect upon what makes a good teacher and/or what makes a bad teacher.4 5 We can learn something from the work of medical...

Seeing optic neuritis through myopic eyes

Sethi, P. K., Sethi, N. K. — 2012-03-26T05:16:06-07:00

Recently, while conducting inpatient neurology rounds in my hospital, a first year neurology resident in training presented the case of a 36-year-old woman. One week prior to admission, she had noticed blurring of vision and decreased visual acuity in her left eye, along with pain. "She has optic neuritis", was the instantaneous and simultaneous answer when I asked the residents for the diagnosis. "And what is the cause of her optic neuritis?" I asked. The residents looked at me as if I had two heads. "Multiple sclerosis surely, sir". My next question, whether she could have anything other than multiple sclerosis (MS), left them dumbfounded. An MRI of the brain and cervical spine was already done and a lumbar puncture was planned for later in the day. A preliminary diagnosis of clinically isolated syndrome was written in bold letters in her chart, based on normal neuroimaging results.

The ‘publicity’...

SUDEP discussions with patients and families

Leach, J. P. — 2012-03-26T05:16:06-07:00

Introduction

The Scottish legal system has published two separate verdicts emphasising the need for information on sudden unexplained death in epilepsy (SUDEP) to be discussed with patients at the earliest opportunity (box 1). This is also an increasing concern across the world as evidenced by the recent International Epilepsy Congress (a joint congress with representation from medical professionals and the voluntary sector through the International League Against Epilepsy and the International Bureau for Epilepsy) devoting two large plenary sessions to this issue, including its opening Presidential Symposium.

Box 1 What is a Fatal Accident Inquiry?

The Scottish legal system provides a framework for a fatal accident inquiry (FAI) as an investigation into death, similar to the English coroner's inquiry. An FAI is not a criminal investigation, but can be requested by anyone who feels that an inquiry into a death may yield...

Comment on 'SUDEP discussions with patients and families'

Hanna, J. — 2012-03-26T05:16:06-07:00

I would like to endorse the approach of Dr Leach that the aim of discussion of sudden unexpected death in epilepsy (SUDEP) is positively to support a person to understand and manage their seizures and lifestyle. The guidelines from the Scottish Intercollegiate Guidelines Network and the National Institute for Health and Clinical Excellence have, for over 8 years, encouraged discussion of SUDEP as essential information for people with epilepsy. The Scottish government has commissioned three public inquiries following the sudden deaths of one newly diagnosed patient and two existing patients. Both judges who presided over these inquiries found that a default position of discussion of SUDEP with patients is in keeping with the scientific evidence: that reasonable precaution might prevent some deaths in people at risk of SUDEP (whether high or low). Sheriff Duff was also clear that guidelines are tools not rules, which should give way in certain...

SNAPs, CMAPs and F-waves: nerve conduction studies for the uninitiated

Whittaker, R. G. — 2012-03-26T05:16:06-07:00

Introduction

Neurologists are absurdly fond of impenetrable jargon that only those initiated into the club can understand. The more obscure the Latin or Greek etymology the better, and preferably with plenty of syllables (dentatorubral-pallidoluysian atrophy, anyone?). In neurophysiology, we are no different; our reports are peppered with terms that only a card-carrying neurophysiologist would understand, and we are equally partial to a juicy acronym. If you are lucky, the bottom couple of lines might sum up what it all means. If you are not, then how do you sort your CMAPs from your SNAPs and your F-waves from your A-waves? At the risk of being ostracised by my colleagues, here is my attempt to unmask some of the dark secrets of nerve conduction studies (NCS) handed down through countless generations of neurophysiologists.

The scope of NCS

Broadly speaking, NCS are used to investigate diffuse processes such...

Encephalopathy in a 45-year-old woman: presented at the Advanced Clinical Neurology Course, Edinburgh 2010

Derry, C., Allen, C., Grant, R., Ironside, J., Davenport, R. — 2012-03-26T05:16:06-07:00

The history

A 45-year-old woman was brought to the emergency department by the police. She had been stopped for driving erratically and noted to be confused; an alcohol breathalyser test was negative. In the emergency department, she was disoriented in time and place, unable to give a history, but denied any symptoms.

The patient's sister had noted a behavioural change about 12 months previously when the patient began using cannabis, but otherwise she had functioned normally until 4 weeks before this presentation. Then her memory had deteriorated, her behaviour changed further and she began to have difficulties with routine tasks at work (eg, she required help to use a photocopier). She had been able to continue in her job as a lecturer, but colleagues had noticed her poor performance.

Two weeks before this admission, a neighbour found her in a confused state on the floor, having vomited and...

Neuro-Sweet's disease

Maxwell, G., Archibald, N., Turnbull, D. — 2012-03-26T05:16:06-07:00

Sweet's syndrome, or acute febrile neutrophilic dermatosis, is a multisystem, inflammatory disease characterised by tender skin lesions and neutrophilic infiltration of various organs, including the nervous system. A rare condition, neuro-Sweet's can present with a wide variety of neurological symptoms dependent on the region of the CNS affected. Here we present a case of neuro-Sweet's disease in association with Crohn's disease.

Listeria rhomboencephalitis

Abbs, A., Nandakumar, T., Bose, P., Mooraby, D. — 2012-03-26T05:16:06-07:00

A fit and healthy 48-year-old farm mechanic from Yorkshire gradually developed unsteadiness over 24 h associated with nausea, vomiting and malaise. The next day he developed a dull occipital headache and his unsteadiness worsened. His symptoms progressed over the next 48 h with slurring of speech, diplopia, rotational vertigo and intractable hiccups. He did not seek medical attention until day 4 when he woke with right-sided weakness and pareasthesia.

On arrival at the accident and emergency department, he was afebrile, mildly dysarthric and had an ataxic gait. MRI of the brain showed abnormal high T2 signal within the brainstem (figure 1). Treatment with dexamethasone was commenced, but his symptoms worsened after 48 h to include dyspnoea, cough and stridor, the last prompting laryngoscopy, which showed vocal cord paresis, and arterial blood gas analysis showed a type 1 respiratory failure.

As he continued to deteriorate, he was transferred...

Letter from Libya

Benamer, H. T. S. — 2012-03-26T05:16:06-07:00

Libya is a large Arab and North African country about three times the size of France but with a population of only 5.3 million, as the majority of the country is desert. Most of the population is young (32% under the age of 15 years) and urban, with 85% living in the main coastal cities such as Tripoli, Benghazi and Misrata.1 Libya was an Italian colony from 1911 until after the Second World War when it became a British protectorate; Libya obtained independence as a kingdom in 1951. However, in September 1969 Muammar Gaddafi established a military dictatorship that continued until he was killed on 20 October 2011.

In the 1950s Libya was considered to be one of the poorest countries in the world with high illiteracy rates and only a handful of people with university qualifications. With the discovery of oil in the 1960s, living standards...

End of the bed (end of the video) diagnosis

Dean, Z., Vincent, A., Farrugia, M. E. — 2012-03-26T05:16:06-07:00

A 23-year-old woman, who had been previously well, developed three generalised tonic-clonic seizures in 24 hours. For 5 days, she had complained of headache and fever, and her family had noted a personality change with unusual hypomanic behaviour (she cleaned the house persistently and wanted to stay out and party all night). She was admitted initially to a general medical ward, where she exhibited bizarre behaviour. She made strange noises, laughed inappropriately and performed repetitive stereotyped movements such as picking at her clothes. She had periods of agitation and at one point almost jumped out of the window. Brain imaging (CT and MRI) was normal. Cerebrospinal fluid (CSF) examination showed a raised lymphocyte count (140 lymphocytes/µL (≤5)) with normal CSF protein and glucose, and oligoclonal bands in CSF but not in serum. Blood cultures were sterile. Routine bloods and inflammatory markers, including serum C reactive protein, were normal. She...

The term 'generalised' in EEG reports: too much ambiguity?

Brigo, F. — 2012-03-26T05:16:06-07:00

EEG reporting is like trying to translate a picture into words: when not properly reflecting all the complexity of an EEG pattern, this challenging task may lead to misinterpretation, especially when the reader of the EEG report is a neurologist who is not an expert in epilepsy. One of the most common matters of debate and possible source of misunderstanding is the term ‘generalised’, which in epileptology refers to EEG discharges, seizures and epilepsies. Although this term is widely accepted in EEG reports, the use of the same word prompts debate when referring to seizures and epilepsy. Perhaps the use of the term in reporting EEGs should be reviewed.

The term ‘generalised’ has long been used, as opposed to ‘focal’ (or ‘partial’ or ‘localisation-related’), to express a dichotomous classification for both seizures and epilepsies. In 1981, the Commission on Classification and Terminology of the International League Against Epilepsy (ILAE)...

Carphology by A Fo Ben

2012-03-26T05:16:06-07:00

Atrial fibrillation and stroke

Approximately a quarter of strokes are of unknown aetiology and, even given the rapid advances in acute treatment, prevention remains better than cure. This multi-centre study enrolled 2580 patients with no history of atrial fibrillation, in whom a pacemaker or defibrillator had recently been fitted. Subjects were randomly assigned to receive (or not) continuous atrial overdrive pacing. At 3 months, subclinical arrhythmias had occurred in 10% of patients. These were associated with an increased risk of embolic events (stroke or systemic embolism; HR 2.49). After 3 years, 51 patients had experienced embolic events, including 11 with a subclinical tachyarrhythmia, although none had clinical atrial fibrillation. Sadly, pacing did not alter the risk of an embolic event. It is debateable how representative is this group of patients, with cardiac problems severe enough to require pacemakers. However, the study strongly suggests an important avenue for further...