95 e-Letters

published between 2014 and 2017

  • Pure Autonomic Failure: the importance of waiting to confirm the diagnosis

    Dear Editor,
    Thomas Brown provided a helpful recent review of pure autonomic failure (PAF) in this journal1. However, there is one very important point which I would wish to add. The diagnosis of this rare condition should be made with extreme caution in the early years of presentation. The first case in the review had a diagnosis of PAF made just two years after symptoms began. In fact, other commoner causes of primary autonomic failure may declare themselves many years after the symptoms first begin, and I have seen a number of such cases during my time at the autonomic unit in Queen Square. A rough rule of thumb used to be that at least five years should pass before making a definitive diagnosis of PAF2. More recent evidence suggests that even longer may be required. A third of cases already with symptoms suggestive of PAF for an average of 5 years were found to have an alternative diagnosis with subsequent follow up for a further four years3. Alternative diagnoses included other synucleinopathies such as Parkinson’s disease, dementia with Lewy bodies or multiple system atrophy.
    For many years attempts have been made to find reliable ways to distinguish PAF from other causes of primary autonomic failure. PAF is seen classically as a post-ganglionic disorder whilst multiple system atrophy is seen as a pre-ganglionic form of primary autonomic failure. Utilising this pathophysiological difference, a variety of differentiating tests have been attempted....

    Show More
  • SUDEP: The thing we fear more than death

    We came across the pages of the article of Nashef and Leach recently released to the scientific community through the Practice Neurology with a great enthusiasm.1 The authors have not only had courage, but have had special ability in touching on such an important matter in a scientific and subtle manner simultaneously. As we know, along the past twenty years there has been exponential growth in the number of articles published on SUDEP with a triple increase in this scientific production in the last ten years.2 Therefore a great scientific breakthrough in SUDEP has been established with regard to epidemiological aspects, specific risk factors, mechanisms involved and possible preventive measures (if they really exist) 3. And yet, it is very well defined that the main risk factor for SUDEP is the presence and number of generalized tonic-clonic seizures (GTCS)3 and the best way to avoid a tragic event is to control these GTCS. In parallel, there is also an imperative issue in relation to all these aspects: how, when, where, why and what patients should we address on SUDEP? Despite the great effort of epileptologists and elegant studies already published, the discussion on SUDEP with patients is still a matter of debate among experts.4 In order to enable this debate, we will have to somehow let the conservative side and consider the current numbers that demonstrate that 1 in every 1000 young adults and 1 in every 4500 children with epilepsy may suddenly die. 5 And more, accor...

    Show More
  • ‘Organic’ and ‘non-organic’

    Stone and Carson(1) say:
    ‘Non-organic refers to things unrelated to living matter,… This is a word that cannot be used to describe conditions suffered by living human beings.’ They refer ‘to the OED sense: “something characterised by structural or other pathological change in an organ or organs”. They acknowledge it was useful ‘in the 19th century when neurologists first saw the pathology of multiple sclerosis or motor neurone disease. But what about genetic generalised epilepsy or migraine—pathological, but not necessarily structural, or at least no more so than, for example, unipolar depression where there is quite substantial evidence for structural changes. ’(2)

    They are right to point out the difficulties in separating ‘functional and non-organic’ disorders from those with structural or ultrastructural abnormalities but they should not be ‘blackballed from the club of legitimate conditions because of the difficulty in establishing their veracity.’

    Most of us understand that epilepsy, migraine and spasmodic torticollis, for example, have underlying structural or other subtle abnormalities; the fact that modern techniques don't always disclose the organic basis does not mean they are psychogenic, do not exist, or are illegitimate.

    Functional was originally the result of altered function (rather than structure) of an organ, system, etc.; but its meaning confusingly changed to a psychological cause as opposed to a physical one. The term...

    Show More
  • Authors response to Dr Hughes' comments

    We thank Dr Hughes for his thoughtful letter and his previous extensive comments as part of the reviewing process.

    The main issue Dr Hughes has raised is the possibility of another local structural lesion. He asks that “Some cross checking with digital examination of the floor of the mouth, palpation of the tongue, or the results of attempts by a clinician to correct the position of the tongue may be all that is required to confirm or refute the working diagnosis.

    We had already addressed this in our paper “The ENT and oral maxillofacial surgeons had noted a small post-traumatic neuroma/granuloma at the left retromolar space but with no other oral structural abnormality “. And expanded on this in response to the query in Dr Hughes’s first review, “No structural lesion to the tongue muscle or its attachments was observed on clinical examination by an experienced ENT surgeon or on independent examination by an oral maxillofacial surgeon”.

    We feel our explanation as outlined in our paper remains the most plausible.

  • Correspondence and referee’s reports on Carr AS, et al. Wisdom tooth extraction causing lingual nerve and styloglossus muscle damage. Pract Neurol 2017;17:218–221.

    Dear Editors,
    Carr AS, et al. Pract Neurol 2017;17:218–221. doi:10.1136/practneurol-2016-001491
    In the interests of enhancing our understanding of bulbar function from the perspective of the jobbing clinical neurologist, I am writing to you regarding a publication in this month’s edition of Practical Neurology (Carling et al.). As it stands I do not think the case report does justice to the scholarship of the seven authors.
    I enjoyed formally refereeing the paper on two separate occasions (enclosed), and offered a third comment (“I think the patient has a lingual nerve palsy and a tethered tongue on the left, both occurring as a complication of surgery”) by email, in the hope that it could appear in a little box as “reviewer’s comments”. I now think that I would have provided a better service to PN if I had sent this single sentence to you instead of the first review.
    In the report, the authors champion the careful approach required in patients with very focal abnormalities involving the lower cranial nerves and the muscles they innervate. They describe in pictures and very useful tables—copies of which are already on my office wall—the detailed knowledge that is required to avoid misattribution in the crowded and inaccessible house of the structures involved in bulbar function.
    The patient concerned had a third molar removed and postoperatively had a lingual nerve palsy. This complication is anticipated, and is included in the consent pro...

    Show More
  • Re:Psychogenic non-epileptic seizures in early Huntington's disease - a different experience
    Filipe B Rodrigues

    Dear Editor,

    We thank Prof Barker for sharing his thoughts and experience[1] on our report of psychogenic non-epileptic seizures in Huntington's disease[2]. We agree that neuropsychiatric manifestations are extremely common in HD and most cases of unusual behaviours or movements would not fall into the category of functional disorders. Moreover we certainly do not wish to give the impression that non- epileptic...

    Show More
  • The palmomental reflex: Reply to Schott and Rossor
    Andrew J Lees

    Dear Editor,

    The only thing that I can disagree with in Jonathan Schott and Martin Rossor's article on the palmomental reflex in the December issue of Practical Neurology is their plea that I should stop scraping. I have found that the palmomental reflex can be made more specific for detecting brain damage by applying the orange stick or car key to the hypothenar rather than the thenar eminence. A positive palmomen...

    Show More
  • Psychogenic non-epileptic seizures in early Huntington's disease - a different experience
    Roger A Barker

    Dear Editor,

    We were interested to read the recent case study presented by Rodrigues and Wild in the current edition of Practical Neurology where they discussed "Psychogenic non-epileptic seizures in early Huntington's disease"[1]. The authors gave an in-depth account of the clinical profile of a 51-year-old male with early Huntington's disease (HD) who presented with repeated episodes of repetitive, purposeless, c...

    Show More
  • The Babinski sign: Key Facts
    Mark Baker

    Dear Editor,

    In his recent Neuromythology article1 on the Babinski sign, Professor Kiernan repeats the oft quoted recommendation regarding the best method for testing the plantar response, which is to:

    'Run a car key (figure 1; or as some have suggested, a most expensive motor car key...) along the lateral border of the sole.'

    It will not surprise many readers of Practical Neurology to learn th...

    Show More
  • The biography of functional disorders
    Jonathon P Tomlinson

    This sensitive review is very welcome, as in our experience, most patients with functional disorders are told by specialists what they do not have. I would be much more inclined to refer patients if this kind of approach was more common. It is one reason why relatively few of the patients we see in primary care are referred for specialist attention. Specialists are seeing only the tip of the primary care functional iceb...

    Show More