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Recent eLetters

Displaying 1-10 letters out of 88 published

  1. The Babinski sign: Key Facts

    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 that the author of this particular recommendation was the late Professor Henry Miller and moreover that he was proscriptive with regard to the precise type of ignition key. In his introduction to the book 'Remembering Henry'1, reflecting on the remarkable life and career of one of the undoubted pioneers of modern British neurology (and prolific music critic), the late Lord Walton of Detchant wrote:

    'Books could be (and probably will be) filled with quotations from his tongue and from his fertile pen, some few of which were hallowed for posterity as 'Henry Millerisms' in a popular medical journal. Who can forget such comments as: 'The best instrument for obtaining the plantar response is the ignition key of a Bentley'; or, 'Hemiplegic multiple sclerosis is a rarity and is to be diagnosed only by me'?'

    Henry George Miller (1913-1976) became Professor of Neurology at Newcastle University in 1964. This was the second chair in neurology to be established in the UK (Roger Gilliat having been appointed as professor of clinical neurology at the Institute of Neurology, Queen Square in 1962). He is best known for his work on multiple sclerosis (publishing the first ever therapeutic trial in 19532) and accident neurosis. Henry was successively Dean of Medicine (1966-1968) and Vice Chancellor (1968-76) of Newcastle University, served as Secretary-General-Treasurer of the World Federation of Neurology and Chairman of the medical panel of the MS Society, and held visiting chairs in Australia, Canada and the USA.

    References

    1. Remembering Henry. LOCK S, WINDLE H (Editors). BMJ. The Devonshire Press. 1977.

    2. Acute disseminated encephalomyelitis and acute disseminated sclerosis; results of treatment with ACTH. MILLER HG, GIBBONS JL. BMJ. 1953;2(4850):1345-1348

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  2. The biography of functional disorders

    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 iceberg. Referral rates vary widely depending variously on levels of experience, resilience, tolerance of uncertainty and access to peer or specialist support.

    One advantage GPs have over our specialist colleagues is that we get to know our patients over many years - which is how long it may take to discover the biography behind their functional symptoms. A second advantage we have is a knowledge of their family and social context within which they experience their symptoms enabling us to take a systemic approach. We have more in common with Oliver Sacks- who rediscovered the importance of narratives divulged slowly, and used to visit his patients at home like his father, a London GP, than we do with our younger neurological colleagues.

    It's worth remembering that a clinical history is not narrative and biography is not psychology. Stone warns against framing functional neurological symptoms in psychological terms, which I would agree with - mental illness, if it co-exists ought to be seen as a co-morbidity rather than a primary cause. But Stone's approach is still biomedical and lacks the systemic approach that time, continuity and contextual factors illuminate. I recently invited older GPs to talk to trainees about their favourite patients. Many described patients who were struck with their functional symptoms for years, decades even - in a state of narrative chaos, before something changed and they found some kind of peace with themselves and were much better able to live with their symptoms. In narrative medicine this is a shift from chaos to quest, where making sense takes over from searching for a cure. There are many ways of making sense of functional symptoms and my main concern about Stone's approach is that usually it's most honest to admit we just don't (yet) know.

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  3. Romberg's test has stood up to the test of time and should remain standing

    Title: Romberg's test has stood up to the test of time and should remain standing (Neuromythology: Letter to the Editor)

    I was Interested to read Professor Martin Turner's article entitled Romberg's test no longer stands up. In the article Professor Turner describes the test as "the process of standing unsupported with the eyes closed and feet together for 30 s" and asserts that "the positive result is the patient falling to the floor" which he gives as one of his reasons for abandoning the test. He further states that the test is "neither highly sensitive nor specific for its original purported purpose". Furthermore, along with the authors of previous reviews of the test which he quotes,1,2 he denies Romberg the credit of having linked the positivity of the test to a dorsal column lesion. I note that Professor Turner does not reference the 2nd Edition Romberg's Manual, the original source description3 of the test in and his description of the test or sign does not corresponded with the original. Romberg3 describes his sign twice in the Manual. In the second description, at the top of page 396 of Volume 2, Romberg states "If he [the patient with Tabes Dorsalis] is ordered to close his eyes while in the erect posture, he at once commences to totter and swing from side to side; the insecurity of his gait also exhibits itself more in the dark". In the earlier description in Volume 1, on pages 226-227, he says "I have observed that anaesthesia of the muscles alone without loss of tactile power, invariably accompanies tabes dorsalis. A simple experiment suffices to determine the fact. If the patient is told to shut his eyes while in the erect posture, he immediately begins to move from side to side, and the oscillations soon attain such a pitch that unless supported he falls to the ground." Note firstly that there are arguably two parts to Romberg's test. The first part, requires no time interval, on the contrary, a positive Romberg's test is demonstrated by an increased sway that begins "at once" or "immediately" after the eyes are closed. Furthermore, according to his second description of the sign, a fall to the floor is not required and only the now never-undertaken second part of the test, requiring darkness, is potentially dangerous. Romberg's claim for specificity and sensitivity is that "it invariably accompanies tabes dorsalis" and "it is a symptom which I have not observed in other paralyses, nor in uncomplicated amuarosis; since then [since he first said that he described the sign 10 years previously], I have found it in a considerable number of patients [with Tabes Dorsalis] .... and in no case have I found it wanting." Romberg also states that "some patients mention the circumstance [increased unsteadiness in the dark, while standing without support] without being asked about it". It is also interesting that a little later on in the same description, on page 397 of Volume 2, Romberg says that as it progresses, "the necessity of employing the eyes becomes more and more urgent; if he [the patient with Tabes Dorsalis] closes his eyes, even while sitting, his body begins to sway to and fro" and he "is no longer able to recognize the position of his own limbs and cannot tell whether the right leg is crossed over the left, or the reverse". Since this description of the disease was prior to the discovery of the causative spirochaete, it quite possibly that Romberg (as well as other 19th century Neurologists) may have included other causes dorsal column damage and large fibre neuropathy / neuronopathy and among his cases. Romberg3, correlating the clinical features with the pathology, mentions on the same page as his sign, that post-mortem findings "almost without exception show the existence of partial atrophy of the spinal cord" and that "the contents of the cords of the cauda equina have often been found to have disappeared to such an extent that nothing but the empty neurilemmatous sheaths seemed to remain". He further noted that "it is of especial interest to observe that the posterior, sensory roots are occasionally alone affected in conjunction with the posterior columns of the spinal cord, the anterior motor columns and nerves retaining their normal structure". To my mind, that counts as linking the positivity of the test to a dorsal column lesion and indeed to a lesion of the sensory roots and / or nerves which Professor Turner, incidentally, did not mention as a cause of a positive Romberg's sign. Sir William Gowers4 in the 1888 American Edition of his textbook cited Romberg and his test as did the legendary Jean-Martin Charcot5 in his Tuesday Lectures. It is interesting that Gowers4, referring to Romberg's test states that "the effect of closure of the eyes is greatest when sensation in the soles of the feet is defective, but does not depend on this loss; it may be marked when sensation on the soles of the feet is perfect". Although the current method of joint position testing in the Hallux, referred to by Professor Turner, seems unlikely to have been employed at the time of Gowers, it is certainly my experience that dorsal column function or peripheral nerve proprioceptive sensory function can be sufficiently impaired to cause "positive Rombergism" when concurrent testing of joint position sense in the hallux on both sides is apparently within normal limits. By way of explanation of this personal observation, I would suggest that proprioception is likely to be a more complex predominantly unconscious function of the nervous system, rather than the simple transmission of the position of the distal phalanx of the hallux to consciousness. Therefore, I disagree with Professor Turner's assertion that simply testing joint position test in the Hallux is a substitute for Romberg's test. Before dismissing a test proposed by the one of the greatest Neurologists of the 19th century who was author of the first systematic textbook of Neurology ever written and who made numerous other seminal contributions to our subject, I think we should be a little more circumspect. Wilson6 in his classic 1940 textbook also considered Romberg's sign useful and it is considered an important part of the neurological examination in most modern textbooks of Neurology. In my view, Romberg's Test is a very useful part of the neurological examination, because it so beautifully illustrates and confirms to the clinician and student alike, the degree to which loss of proprioception contributes to ataxia and impaired balance in any particular case (whichever of the limited number of underlying pathophysiologies is responsible). A positive Romberg's test therefore helps the clinician to attribute ataxia and / or impaired balance to pathology in the dorsal column of the spinal cord, or in the peripheral large nerve fibres destined for the dorsal columns, rather than to pathology in the cerebellum. It is not dangerous and has stood the test of time. I believe Romberg's test should remain part of the routine neurological examination taught to all medical students and used by all Neurologists. I believe that Professor Turner should stand down on this issue and that Professor Romberg and his test remain upstanding. References 1. Lanska DJ, Goetz CG Romberg's sign. Development, adoption, and adaptation in the 19th century Neurology 2000;55:1201-6. doi:10.1212/WNL.55.8.1201

    2. Pearce JM Romberg and His Sign. Eur Neurol 2005;53:210-13 doi:10.1159/000086732

    3. Romberg MH A Manual of The Nervous Diseases of Man 2nd Edition 1851 Volumes 1&2 226- 227,396-397. Translated and edited by Sieveking EH, London: Sydenham Society Reprint: Alabama: Gryphon Editions Ltd 1983:

    4. Gowers WR A Manual of Diseases of the Nervous System. American Edition P.289 Philadelphia: P.Blakiston, Son & Co 1888 Reprint: Alabama: Gryphon Editions Ltd 1983:

    5. Charcot JM Charcot the Clinician. The Tuesday Lectures Excerpted and translated case presentations by Goetz CG P143 Raven Press New York 1897

    6. Kinnier Wilson SA Neurology Volume 1 P.494 London: Edward Arnold & Co 1947

    "Provenance and peer review. Not commissioned, Externally peer reviewed. This paper was reviewed by Martin Tuner, Oxford, UK."

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  4. OPTIC NEURITIS AND VISUAL ELECTROPHYSIOLOGY

    Sir, I read with much interest the review by Weerasinghe and Lueck on the diagnosis of optic neuritis. The authors provide an extensive and detailed analysis on the differential diagnoses and the management of the disease. The importance of retinal electrophysiology is also acknowledged when differentiating retinal disorders that can mimic optic neuritis. Surprisingly, however, the diagnostic role of pattern-reversal Visual Evoked Potentials (VEPs) is not discussed, not even mentioned. VEPs can assess visual pathway function at a millisecond time scale and no other visual function measure shares this temporal sensitivity. More than 40 years ago Martin Halliday demonstrated a delay in the VEPs after an attack of optic neuritis. Since then, a huge number of studies have provided converging evidence about the importance of VEPs recording in optic neuritis and Multiple Sclerosis (MS). Doubtless, the clinical use of VEPs has changed over time and the advent of magnetic resonance imaging (MRI) have reduced the use of VEPs in clinical practice. However, even in the MRI era, an abnormal VEP in unaffected eyes of patients with MS provides evidence for clinically silent demyelinating lesions in the optic pathway. This, in turn, is extremely useful in identifying dissemination in space and, therefore, in establishing the diagnosis of MS. In their review, the authors also fail to mention another important electrophysiological test, i.e. the Pattern Electroretinogram (PERG). The PERG is generated by the activity of the retinal ganglion cells. Taken together, PERG and VEPs are useful to identify the site(s) of dysfunction along the retinocortical pathway. In a typical case of optic neuritis PERG and VEP recordings might not be necessary. However, these techniques can still retain many roles: first, to show the magnitude of conduction block of the optic nerve fibres in the acute stage of the disease. Secondly, to demonstrate the optic nerve conduction delay due to demyelination. Third, to follow remyelination, which is seldom correlated to an improvement in visual acuity, through the shortening of VEP latency. Finally, to define the eventual retrograde degeneration of ganglion cells, which is reflected by an amplitude reduction of the PERG N95 peak. Moreover, when diagnosing atypical optic neuritis, these neurophysiologic techniques should be implemented as a part of a comprehensive assessment. Of note, the visual pathway can now be more precisely evaluated in detail by means of new neurophysiological tests (multifocal VEPs and ERG). These techniques provides higher sensitivity and specificity in detecting abnormalities in visual function in optic neuritis and MS

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  5. Reply from Pennington et al.

    We welcome debate around this emerging condition. We all recognise a situation when as clinicians we have had a strong feeling that a patient has a functional complaint from early on in the consultation. However, the differential diagnosis for Functional Cognitive Disorder is neurodegenerative dementia which itself affects behaviour and personality and could, therefore, influence many of the cues clinicians pick up on when "divining" a functional diagnosis. Anecdotally, we have seen patients in our clinic diagnosed with a functional disorder at other centres whom we have subsequently discovered to have definite neurodegenerative disease. As an example, we were referred a patient thought to have a psychiatric disorder and functional left leg movements. After a detailed assessment including CSF biomarkers and SPECT scanning, we suspected organic disease. His positive C9orf72 genetics have recently demonstrated he suffers from a genetic form of Frontotemporal dementia and we believe that this altered his behaviour such that he manifested a functional syndrome at the start. Preceding medical events such as general anaesthesia or chemotherapy can be triggers for health anxiety or FCD, but could also be a source of cognitive symptoms. General anaesthesia (particularly if performed for cardiac indications) is a risk factor for hypoxic or ischaemic brain damage (which if focal can present with isolated cognitive deficits). Chemotherapy agents can cause neurological complications (e.g. methotrexate encephalopathy); their use also implies a history of malignancy with consequent risk of tumour recurrence and direct or indirect neurological complications. Establishing the aetiology of cognitive complaints is the key challenge in the memory or general neurology clinic. Large-scale research studies suggest there is a continuum between Subjective Cognitive Impairment (where individuals report cognitive symptoms but perform normally on testing), MCI and dementia2. Given treatments may be effective only in the earliest stages, identifying prodromal dementia will in the future be increasingly important. Distinguishing incipient dementia at the Mild Cognitive Impairment (MCI) phase from Functional Cognitive Disorder (FCD) can be difficult, but is of vital importance to ensure patients receive appropriate prognostic information including reassurance where necessary and are entered into clinical trials where appropriate. This distinction becomes particularly tricky in high-functioning individuals, and those with co-morbid psychiatric conditions. The clinical history is very useful, and conversational analysis has been successfully used to differentiate dementia from FCD1. However we would counsel against solely using the clinical history to distinguish FCD from incipient dementia at the MCI phase. We know that anxiety, depression and abnormal behaviour may occur in early dementia all of which might confound use of a clinical history to differentiate incipient dementia from FCD. Neuropsychology assessments are very useful in this context- they can reveal patterns of deficits that are not consistent with organic pathology, deficits out of keeping with FCD, or highlight significant psychiatric symptoms warranting referral to psychiatry or psychology services. Neurologists are often not trained in identifying psychiatric disorders, therefore a structured neuropsychology assessment including questionnaires looking for symptoms of depression or anxiety can often be very helpful. The authors would regard neuroimaging as an essential part of the assessment, as recommended by NICE3. Even a patient with a 'classic' FCD history may be harbouring structural brain pathology. To give a clinical example, a 56 year old man presented to our general neurology clinic with intermittent word finding difficulties during conversations and when typing. He had a history of anxiety disorder, and his language appeared normal during the consultation and on formal assessment. Neurological examination was normal, and a functional cause was suspected. However on MR imaging a large glioblastoma multiforme was found. Not performing neuroimaging risks missing individuals with a strategic infarct, malignancy or other structural pathology. Whilst such cases are not common, missing such a diagnosis would have very serious consequences for the patient. The authors agree that investigations such as SPECT, CSF biomarkers and autoantibodies should be used selectively, but these can be helpful in difficult cases. We plan to explore further the additive clinical value of each of these tests. Discovering the aetiology of cognitive symptoms often requires us to operate at the limits of current knowledge about how the brain guides behaviour. We do not believe we are at the point where we can always offer certainty to a patient. However, we find it possible to reassure patients effectively even when we represent this uncertainty to them. If we suspect functional cognitive disorder, we introduce the concept at the first consultation and offer reassurance saying we will do further tests and follow up to rule out rarities. We find this is actually quite effective as a means of reassurance, perhaps more so than being entirely dogmatic that there cannot be organic pathology as most patients will know it is more or less impossible to prove a negative in neurology. Overall we feel that whilst we might strongly suspect FCD clinically at the first consultation, it is unwise in almost all cases to make a firm diagnosis of FCD without appropriate investigations. Even after diagnosis and initial management of FCD, a period of clinical follow up can be informative. It is only such follow up that has allowed us to begin to understand critical components of FCD and to summarise clinical features of the condition.

    References 1. Elsey, C., Drew, P., Jones, D., Blackburn, D., Wakefield, S., Harkness, K., Venneri, A. & Reuber, M. Towards diagnostic conversational profiles of patients presenting with dementia or functional memory disorders to memory clinics. Patient Educ. Couns. pii: S0738, (2015). 2. Garcia-ptacek, S., Cavallin, L., Kramberger, M. G., Winblad, B., Jelic, V. & Eriksdotter, M. Subjective Cognitive Impairment Subjects in Our Clinical Practice. 419-430 (2014). doi:10.1159/000366270 3. NICE. Dementia diagnosis and assessment. (2015).

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  6. Re: Pennington et al. Functional cognitive disorder: what is it and what to do about it?

    Pennington and colleagues review of Functional Cognitive Disorder (FCD) [1] is very welcome to guide practice in a complex condition that is presenting increasingly to general and cognitive neurology clinics. However, in our view Pennington's presumption towards specialist assessment, investigation and surveillance is unnecessary and misses an opportunity to provide patients with a prompt diagnosis, explanation and reassurance and immediate treatment for the FCD and/or significant relevant co-morbidities e.g. depression, chronic fatigue syndrome (CFS). In particular, we cannot agree with Pennington's comment that "Functional cognitive disorder should be diagnosed only after excluding other causes of cognitive decline as far as possible". We believe that clinicians should aim to make a positive "rule in" diagnosis of FCD, exactly as proposed for other functional disorders [2] and that this can be done in the context of the general neurology clinic. Pennington et al rightly point out features in the history that suggest FCD but in our view they underestimate their significant diagnostic value. Additional positive features for FCD in our experience are a high number of presenting symptoms, the patient's emotional response to the symptoms being more distressing than the actual consequences of any cognitive lapses, co-morbid mood disorders, chronic pain or fatigue and recent relevant life events including physical illnesses such as mild traumatic brain injury, anesthesia or chemotherapy exposure. Brief cognitive instruments (such as MoCA, ACE) are of limited value in this context and have an opportunity cost in a 30 minute general neurology consultation. Instead we suggest listening for particular features in the patient's spontaneous speech that point towards FCD. A richly detailed history in which multiple examples of cognitive lapses are described, and in which events are precisely located in time, the patient's ability to reference an earlier part of the consultation [4], and normal expressive language (e.g. the absence of word finding pauses) are all evidence of normal cognitive function. These can be fed back to the patient to help explain the diagnosis, in the same way that a positive Hoover's sign can be explained to a patient with functional lower limb weakness. We argue that most patients with suspected FCD do not need investigations such as brain imaging or neuropsychology. These are much more likely to produce false positives with attendant iatrogenic harm than to shed any light on the diagnosis, particularly in younger patients where the pre- test probability of dementia is extremely low. The authors write that "Cerebral atrophy that is out of keeping for the patient's age ... should prompt further investigations". We would regard lower than expected brain volume as a non-specific finding in a patient with typical FCD symptoms. The temptation to perform further tests to clarify non-specific findings from a previous test demonstrates the perils of embarking on low yield investigations in the first place. Tests for anti-VGKC and NMDA receptor antibodies should be reserved for patients presenting with the clinical syndromes associated with these antibodies, which are readily differentiated from FCD on the basis of the clinical history. We believe that routine neuropsychological assessment for the diagnosis of FCD is clinically unnecessary, not cost-effective and potentially misleading. For example, a neuropsychology report stating that "early neurodegeneration cannot be ruled out" in a case where the clinician strongly suspects FCD may reduce patients' confidence in the diagnosis. We would virtually never consider amyloid positron emission tomography or cerebrospinal fluid biomarkers appropriate for patients with suspected FCD (although they may have a role in rare cases in which a diagnosis of Alzheimer's disease needs to be changed to FCD and this is not initially accepted by patients, family and other healthcare professionals[5]). These tests are abnormal only in Alzheimer's disease so cannot "rule out" dementia in any case. We welcome Pennington's comments about the need to develop treatments for FCD. One of us (JAC) often recommends two books to patients that beautifully illustrate the idiosyncrasies of normal memory; Forgetting by Daaisma and Moonwalking with Einstein by Joshua Foer. For patients with mild FCD, normalising their symptoms e.g. by explaining how we (the neurologist) make many of the same mistakes can be helpful. An emerging nosology can help practitioners select an individualised explanatory model and prioritise treatment i.e. (1) FCD in isolation, with or without dementia-related health anxiety, (2) FCD in the context of a psychiatric co-morbidity (most typically depression) and (3) FCD as a feature of another functional disorder e.g. CFS, fibromyalgia (adapted from [6]). The stability of functional neurological diagnoses is well demonstrated and the same likely applies to FCD, as Pennington and colleagues point out. We therefore disagree with the suggestion that it is necessary to follow patients up to ensure that their cognitive function remains stable. Follow-up might of course be offered for other reasons e.g. where the clinician needs more time to explain the diagnosis. In summary, while welcoming Pennington and colleagues important contribution to this emerging area of neurological practice, we urge general neurologists to have confidence in making a positive diagnosis of FCD, particularly in younger patients. We believe that unnecessary specialist referral, investigation and surveillance of patients with FCD risks delaying treatment and potentially incorrect diagnoses of pre- dementia (e.g. MCI) or dementia itself.

    References 1. Pennington C, Newson M, Hayre A, et al. Functional cognitive disorder: what is it and what to do about it? Pract Neurol 2015;15:436-444 2. Stone J. Functional neurological disorders: the neurological assessment as treatment. Neurophysiol Clin 2014;44:363-373 3. Stone J, Reuber M, Carson A. Functional symptoms in neurology: mimics and chameleons. Pract Neurol 2013;13:104-113 4. Jones D, Drew P, Elsey C, et al. Conversational assessment in memory clinic encounters: interactional profiling for differentiating dementia from functional memory disorders. Aging Ment Health 2015; DOI:10.1080/13607863.2015.1021753 5. Coebergh JA, Wren DR, Mumford CJ. 'Undiagnosing' neurological disease: how to do it, and when not to. Pract Neurol 2014;14:436-439 6. Stone J, Pal S, Blackburn D. Functional (Psychogenic) Cognitive Disorders: A Perspective from the Neurology Clinic. J Alzheimers Dis 2015;48 Suppl 1:S5-S17

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  7. A case of functional cognitive disorder with acute onset?

    While reading Pennington et al's (2015) article on Functional Cognitive Disorder, I was struck by the similarity between their descriptions and a patient seen in clinic (patient X), with the exception that X's symptoms appeared over the course of a few days with no identifiable precipitating event. Extensive physical tests and brain imaging investigations were carried out and no organic disease was found yet symptoms were reportedly severe and having a major impact on X's functioning. Here I discuss X's case (with identifiable information changed in order to respect patient confidentiality) and propose that there may be a sub-type of functional cognitive disorder with an acute onset.

    Patient X is a 48 year old house-wife and local preacher. She presented to clinic with her husband after being referred by her GP due to the development of severe confusion and cognitive impairment developing from a previously high level of functioning just a few days earlier. Symptoms began to appear on the Sunday evening and peaked on the Tuesday, with no change between then and the clinic assessment (Thursday). X reported completely 'losing track of time' and reported severe memory loss which her husband had first noticed. During the assessment, she claimed inability to answer any of our questions herself and repeatedly looked to her husband for answers. When asked why she could not answer the questions, she said that she 'could not remember' and described problems with attention, concentration and executive function. This appeared inconsistent with her performance in daily life as she had managed to maintain her previous social roles, although we had no objective baseline for comparison.

    X had no previous psychiatric history and no significant medical history. She was not taking any regular medications except for the oral contraceptive pill. She reported a normal upbringing. The only identifiable recent stressor was that X had recently started a new university course, part of which involved looking at different personality types, which she said had 'confused' and 'worried' her.

    It was first thought that she may have organic disease as her symptoms came on suddenly, however she returned to psychiatry clinic following extensive tests as no physical cause could be found. Could this be a case of functional cognitive disorder with acute onset? Further assessment and follow-up of patient X and others like her could unravel a subgroup of patients who fall into this category.

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  8. Re:Comment on Death in pregnancy: a call for neurological action (JP Leach. Practical Neurology 2015)

    The outlined model of cooperative and integrative services for pregnant women with neurological conditions is warmly welcomed (and not a little envied!). While such services are provided the English centres mentioned, it is sad that such high quality provision remains patchy and incomplete. This nettle-grasping should no longer be quiet, but should be loudly heralded and made the norm. The recently updated SIGN guidelines recommend a joint approach from all corners, and disparate organisations such as the ILAE, the ABN, the Medical Royal Colleges, and the RCOG need to come together to make such synergy routine. Only then can we ensure that complications of epilepsy during pregnancy and the first post-partum year follow other obstetric complications in decreasing incidence.

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  9. Comment on Death in pregnancy: a call for neurological action (JP Leach. Practical Neurology 2015)

    Dear Sirs, We welcome Dr Leach's comments and a call for action from Neurologists, regarding service provision for women of childbearing age with neurological disorders1. We would also like to highlight that quietly, a number of us are grasping the nettle. Risk is inherent in clinical practice. Managing risk effectively and pro- actively in preference to reactively minimises the likelihood of a poor outcome. This can be achieved by identifying those at risk and adapting practices to avoid that risk. This has not often been more concisely put than by Cicero who is quoted as stating that "To make a mistake is only human; to persist in a mistake is idiotic". We agree that at the very least, missing an opportunity to avoid a maternal death is idiotic, and certainly tragic. A joint approach is needed and at St George's University Hospitals NHS Foundation Trust, Consultants in Obstetrics, Obstetric anaesthetics, Neurology, Neurosurgery and Neuroradiology have together with excellent support from Maternal Medicine Midwifery and Neurology Clinical Nurse Specialists, been working collaboratively for some time. Our Epilepsy Group run a regular clinic together with Maternal Medicine Midwives for all women with Epilepsy delivering at St George's, and there is a monthly joint Neurology and Obstetric clinic in maternal medicine where women with complex (and sometimes more simple) neurological disorders can be advised regarding pregnancy, delivery and breastfeeding. Careful individualized plans for Neurological (including surgical and vascular), Obstetric, Anaesthetic and midwifery led care are made for these women. We know from prospective data collection (risk also comes from not knowing what you are doing) that unplanned episodes and adverse outcomes have been avoided. We are also aware that similar clinical services are available at other Hospitals including University College London Hospitals, at Queen Charlotte's and Chelsea Hospital, part of Imperial College Healthcare NHS Trust, and has long been available at Birmingham Women's NHS foundation Trust. A call from the NHS England Strategic Clinical Networks was recently made for a Neurologist advisor to join the London Maternity morbidity and mortality expert panel. This is an opportunity to act and work together that the clinicians at St George's will not be missing. Yours sincerely, Dr Dominic Paviour, Consultant Neurologist Dr Ingrid Watt Coote, Consultant in Obstetrics and Maternal Medicine Dr Hannah Cock, Consultant Neurologist and Epileptologist, Ms Amanda Reeve, Clinical Nurse Specialist, Atkinson Morley Epilepsy Group Trudy Williams, Specialist Midwife in Maternal Medicine St George's University Hospital NHS Foundation Trust, London

    References: 1. Death in pregnancy: a call for neurological action John Paul Leach, Pract Neurol 2015;15:244-245 doi:10.1136/practneurol-2015 -001097

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  10. Are the new ABN guidlines for MS of any use in clinical practice?

    As a jobbing MS neurologist in Southampton I am really not sure how these guidelines are going to assist in my clinical practice.

    Perhaps first and foremost, if I was to follow these guidelines I would find myself frequently in breach of NHS commissioning criteria with perhaps severe implications for both myself and my hospital trust given the cost of these therapies. As such I feel the authors should have given a little more attention to this issue, highlighting where guidance is against what we are actually allowed to do.

    My experience of multiple sclerosis patients is that they do not simply fall into two broad categories of active vs more active and therefore I find the division of the drugs into two broad categories equally unhelpful and unnecessary. Perhaps the most obvious example is that fingolimod has considerably more potency than some of the other category one drugs and by these guidelines own definitions fingolimod could easily be classed as a category two treatment and therefore be a good option for more active patients, as it is already under the NHS commissioning criteria.

    The guidelines recommend using alemtuzumab or natalizumab in patients with more active disease as defined by one relapse in the previous year on interferon with MRI criteria. In clinical practice treatment decisions in such patients are heavily influenced by factors such as disease duration, treatment duration, relapse severity, prior relapse frequency on and off treatment, and gadolinium enhancement on MRI (with number of T2 lesions not being particularly useful). These guidelines could and should have been more progressive in discussing a correct approach for different patient types. Among other considerations that should have been discussed include treating young women with highly active multiple sclerosis for whom alemtuzuamb now provides the possibility of potent treatment and pregnancy at the same time, and JC virus negative patients for whom natalizumab offers a virtually risk free high potency therapy assuming that JCV status is regularly monitored whilst on treatment.

    Finally in non-relapsing established progressive disease there is absolutely no evidence to support immunomodulation and given the costs of therapy I really think this guidance could have taken a bolder approach in making firm recommendations about cessation of treatment to support clinicians in their discussions with such patients.

    Overall I feel these guidelines offer a fairly simplistic and non- commital approach, that as UK neurologists we are often unable to follow due to commissioning restrictions, and with the wide range of therapies now available a more progressive, customised approach that addresses a wide range of patient characteristics is what is required.

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