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The aim of this study was to compare the clinical characteristics of patients with and without abnormal MR imaging admitted to a neurosurgical unit with suspected cauda equina syndrome using a retrospective study of consecutive admissions to a regional neurosurgical unit over a 10-month period. Clinical details were obtained from the case notes. A lumbar spine MR scan to investigate possible cauda equina syndrome was performed in 66 patients. There were no significant differences between those with abnormal imaging (n = 34, 52%) and those with a normal scan (n = 32, 48%) in respect of sex, clinical history or features recorded on examination. Those with normal imaging had a high frequency of weakness (n = 18, 59%), saddle numbness (n = 17, 57%), leg numbness (n = 24, 80%), urinary incontinence (n = 13, 54%) and urinary retention (n = 9, 53%). A large number of patients present to neurosurgical units with symptoms suggestive of cauda equina syndrome without any radiological evidence of structural pathology. While some may have had an alternative organic cause, we propose that these symptoms may have a "functional" origin in many patients.

Functional neurological symptoms are a common cause of disability and distress in any neurological service. Varying terminology, including hysteria, conversion disorder, dissociative, non-organic, and psychogenic, reflects the shifting nature of medical thinking, over time, regarding these presentations. They also emphasize the importance of a historical understanding when grappling with this area. This chapter traces this history, from Briquet’s monumental treatise on hysteria in 1859 to a randomized controlled trial of non-epileptic seizures in 2010. In the process, familiar figures such as Freud and Janet are encountered and the electrical treatments of Yealland, for hysteria, are re-evaluated. The chapter highlights how the application of functional neuroimaging perhaps raises more questions than it answers, at the current time, but how it has also aided the reawakening of clinical and research interest in this core area of clinical neurology.

Cognitive symptoms such as poor memory and concentration represent a common cause of morbidity among patients presenting to general practitioners and may result in referral for a neurological opinion. In many cases, these symptoms do not relate to an underlying neurological disease or dementia. In this article we present a personal perspective on the differential diagnosis of cognitive symptoms in the neurology clinic, especially as this applies to patients who seek advice about memory problems but have no neurological disease process. These overlapping categories include the following 'functional' categories: 1) cognitive symptoms as part of anxiety or depression; 2) "normal" cognitive symptoms that become the focus of attention; 3) isolated functional cognitive disorder in which symptoms are outwith 'normal' but not explained by anxiety; 4) health anxiety about dementia; 5) cognitive symptoms as part of another functional disorder; and 6) retrograde dissociative (psychogenic) amnesia. Other 'non-dementia' diagnoses to consider in addition are 1) cognitive symptoms secondary to prescribed medication or substance misuse; 2) diseases other than dementia causing cognitive disorders; 3) patients who appear to have functional cognitive symptoms but then go on to develop dementia/another neurological disease; and finally 4) exaggeration/malingering. We discuss previous attempts to classify the problem of functional cognitive symptoms, the importance of making a positive diagnosis for the patient, and the need for large cohort studies to better define and manage this large group of patients.

Background\ud \ud The evidence base for the effectiveness of psychological interventions for patients with dissociative non-epileptic seizures (DS) is currently extremely limited, although data from two small pilot randomised controlled trials (RCTs), including from our group, suggest that Cognitive Behavioural Therapy (CBT) may be effective in reducing DS occurrence and may improve aspects of psychological status and psychosocial functioning.\ud \ud Methods/Design\ud \ud The study is a multicentre, pragmatic parallel group RCT to evaluate the clinical and cost-effectiveness of specifically-tailored CBT plus standardised medical care (SMC) vs SMC alone in reducing DS frequency and improving psychological and health-related outcomes. In the initial screening phase, patients with DS will receive their diagnosis from a neurologist/epilepsy specialist. If patients are eligible and interested following the provision of study information and a booklet about DS, they will consent to provide demographic information and fortnightly data about their seizures, and agree to see a psychiatrist three months later. We aim to recruit ~500 patients to this screening stage. After a review three months later by a psychiatrist, those patients who have continued to have DS in the previous eight weeks and who meet further eligibility criteria will be told about the trial comparing CBT + SMC vs SMC alone. If they are interested in participating, they will be given a further booklet on DS and study information. A research worker will see them to obtain their informed consent to take part in the RCT. We aim to randomise 298 people (149 to each arm). In addition to a baseline assessment, data will be collected at 6 and 12 months post randomisation. Our primary outcome is monthly seizure frequency in the preceding month. Secondary outcomes include seizure severity, measures of seizure freedom and reduction, psychological distress and psychosocial functioning, quality of life, health service use, cost effectiveness and adverse events. We will include a nested qualitative study to evaluate participants’ views of the intervention and factors that acted as facilitators and barriers to participation.\ud \ud Discussion\ud \ud This study will be the first adequately powered evaluation of CBT for this patient group and offers the potential to provide an evidence base for treating this patient group.\ud \ud Trial registration\ud \ud Current Controlled Trials ISRCTN05681227\ud ClinicalTrials.gov NCT02325544

Summary Functional (psychogenic) neurological symptoms are frequently encountered in neurological practice. Cranial movement disorders—affecting the eyes, face, jaw, tongue, or palate—are an under-recognised feature of patients with functional symptoms. They can present in isolation or in the context of other functional symptoms; in particular, for functional eye movements, positive clinical signs such as convergence spasms can be triggered by the clinical examination. Although the specialty of functional neurological disorders has expanded, appreciation of cranial functional movement disorders is still insufficient. Identification of the positive features of cranial functional movement disorders such as convergence and unilateral platysmal spasm might lend diagnostic weight to a suspected functional neurological disorder. Understanding of the differential diagnosis, which is broad and includes many organic causes (eg, stroke), is essential to make an early and accurate diagnosis to prevent complications and initiate appropriate management. Increased understanding of these disorders is also crucial to drive clinical trials and studies of individually tailored therapies.

The use of electricity in medical treatment has always been technology-driven, rather than aetiology-driven; as new techniques have appeared, clinicians have quickly looked to try them in the treatment of all sorts of conditions where existing treatment options are limited. Functional disorders--as identified anachronistically in our analysis--have been key contenders for emerging electrical treatments: with Leyden jars, with galvanic and electromagnetic machines, and more recently with TMS and TENS. Parallels can be drawn with the history of electrical treatments for migraine and headache (Koehler and Boes, 2010). Regardless of the mode of delivery of electricity, stimulating a limb to produce movement has repeatedly been found to aid and assist recovery in functional motor disorders. This may also be true of non-electrical methods: we have found benefits using both therapeutic sedation and explanatory demonstration of a positive Hoover's sign as therapeutic methods of demonstrating normal movement in functionally weak limbs (Stone et al., 2014). Each surge in enthusiasm for new electrical treatments has been followed by questions about the nature of the disorder and validity of the treatment response. Physicians have tended to attribute therapeutic success initially to powerful biological or even metaphysical effects, but with time and experience these explanations have been replaced by views that the treatment works through suggestion and placebo. Discomfort with these conclusions has in the past discouraged ongoing development of electrical treatments, even if the end result for patients has been encouraging. In Edwards's Bayesian model, functional motor and sensory symptoms are hypothesized to arise when 'pathologically precise prior beliefs' mediated by attentional processes cause experience of symptoms via a hierarchy of false inferences (Edwards, 2012). It can be argued that use of TMS or peripheral stimulation to produce movement of a functionally weak limb has the specific potential to modulate pathological expectations. To reject these treatments as no more than placebo may mean missing an unusual opportunity to manipulate key elements in the mechanism of the disorder. However, changes to these 'priors' may also be dependent upon patient expectations, and as we see through history, this may only happen if the patient believes there is an actual neuromodulatory effect. This may give rise to significant ethical issues in that the treatment may well directly benefit patients but only if they are (mis)informed that there is an underlying biological rationale. We conclude that modern trials of TMS in functional disorders are part of a repeating cycle of experimentation recurring since the mid-18th century. We suspect that emerging technology, including transcranial direct current stimulation, will follow a similar pattern of experimentation, speculation and marginalization. We suggest that considering our modern efforts in a historical context could aid our ability to further expand and maintain our use of electrical therapies that have proven helpful in the past for patients with functional disorders.

Conversion disorder (previously called hysteria) describes neurological symptoms such as paralysis, movement disorder, or sudden collapses that are genuine but not related to disease. Although affected patients may appear to have a neurological disease such as multiple sclerosis or epilepsy, no structural disease process is evident, nor are symptoms deliberately feigned. Instead, brain function is disordered; hence, current preference for the term “functional neurological disorder.” Conversion symptoms exist at the interface between neurology and psychiatry and remain a common cause of disability and distress. The conundrum of hysteria was essential to the development of Freudian psychoanalytic theory, which today is only one of many competing hypotheses to explain these intriguing symptoms. Keywords: conversion disorder; Freud, Sigmund; functional symptoms; hysteria; psychogenic; somatoform; hysteria; nonepileptic attacks; psychiatry

Dissociation is a mysterious and “woolly” area for many neurologists. In this article I will try to persuade you that it describes a common and interesting group of symptoms that many of our patients have—both with and without disease. I will suggest that it should be in the differential diagnosis for anyone presenting with dizziness or blank spells. Dissociative symptoms also occur in common neurological problems such as migraine, epilepsy, and syncope as well as in people with symptoms such as functional weakness, non-epileptic attacks, and depression. They may also occur in isolation but be mistaken for disease. Being able to recognise them, explain them, and perhaps even do something about them should lead to a happier patient and a happier neurologist. Let’s look at some cases from neurology outpatients: A 34 year old woman was walking along with some friends when over a period of a minute or two she developed an intense and frightening feeling of “dizziness”. She felt hot and nauseous, had tingling on the left side of her face, and had to sit down on the path. It all resolved within 10 minutes but she was left feeling shaken by the experience. Later that week the feeling of dizziness recurred on two further occasions, just as unexpectedly, but less intensely. She found it hard to describe the dizziness but eventually said it was like “floating off or being not connected with the world somehow”. The same thing happened during a normal EEG recording. Although the family doctor had been concerned about epilepsy or a transient ischaemic attack, the diagnosis was dissociation, perhaps due to a panic attack. A 26 year old student was referred to the clinic because of “blank spells” with a query about epilepsy. They came on gradually and faded away slowly, lasting about …

In this article we offer an approach to management of functional symptoms based on our own experience and on the evidence from other specialities (because the evidence from neurology is so slim). We also tackle some of the most difficult questions in this area. What causes functional symptoms? Does treatment really work? What about malingering? We give two example cases adapted from real patients to illustrate our approach. Table 1 is not comprehensive but it summarises a large literature on the suggested causes of functional symptoms. This is a question that has been approached from many angles—biological, cognitive, psychoanalytic, psychological, social, and historical. The factors shown have been found to be more common in patients with functional symptoms than in patients with similar symptoms clearly associated with disease pathology. Tables like this can help you to make a formulation of the aetiology of the patient’s symptoms rather than just a diagnosis. An important feature of the table is the recognition of biological as well as psychological and social factors in the production and persistence of functional symptoms. View this table: Table 1 A scheme for thinking about the aetiology of functional symptoms in neurology Most of the factors in table 1 have also been associated with other types of functional somatic symptoms such as irritable bowel syndrome and chronic pain as well as with depression or anxiety. Consequently they should be regarded more as vulnerability factors for developing symptoms, than as specific explanations for why some patients develop certain symptoms such as unilateral paralysis and others have attacks that look like epilepsy. Recent functional imaging studies of patients with functional motor and sensory symptoms1 are beginning to offer biological clues (fig 1); they also challenge the idea that that such symptoms are “all in the mind”—they are in the brain too.2 Figure 1 A composite …

OBJECTIVES: To describe changes in personality after stroke and effects on carers. METHODS: A consecutive series of patients was recruited from hospital admissions with stroke. A novel questionnaire was administered to the patients' main carer at nine months after the stroke to determine their perception of the patients' pre-stroke and post-stroke personality. Personality change was identified by changes in these ratings, and associations between personality change and the following variables explored: emotional disorder in patients and carers (measured using the hospital anxiety and depression scale and a structured psychiatric interview), stroke classification (Oxford community stroke classification), residual disability (Barthel index and Nottingham extended activities of daily living scale), and lesion characteristics on computed tomography (CT). RESULTS: Carers of 35 patients with stroke took part. Reported changes in personality after stroke included: reduced patience and increased frustration (both p

Purpose The aim of this study was to examine the illness beliefs and locus of control of patients with recent onset pseudoseizures and to compare these with patients with recent onset epilepsy. Methods Twenty consecutive patients with pseudoseizures of recent onset (mean duration 5.4 months) were compared with 20 consecutive patients with recent onset epilepsy on their responses to (a) the Illness Behaviour Questionnaire (IBQ) and (b) a measure of locus of control, a dimension of the tendency to attribute events to internal or external factors. Results In comparison with patients with epilepsy, patients with recent onset pseudoseizures believed that psychological factors were less important than somatic ones were (P Conclusions Patients with pseudoseizures are less likely than those with epilepsy to see psychological factors as relevant to their symptoms, more likely to deny that they have suffered from life stress and also to have a more external locus of control. The implications for treatment are discussed.

To establish whether the DNA expansion linked to spinocerebellar ataxia type 8 (SCA 8) is associated with ataxia in Scotland; to clarify the range of associated clinical phenotypes; and to compare the findings with previous reports.DNA was screened from 1190 anonymised controls, 137 subjects who had tested negative for Huntington's disease, 176 with schizophrenia, and 173 with undiagnosed ataxia. Five unrelated ataxic patients with the SCA 8 expansion and a sixth identified subsequently had clinical and psychometric assessment; the clinical features were available in a seventh. A systematic search for other reports of SCA 8 was undertaken.Over 98% of SCA 8 CTA/CTG repeat lengths fell between 14 and 40. Repeat lengths over 91 were observed in three healthy controls (0.12%), two patients with suspected Huntington's disease (0.73%), and six ataxic subjects (1.74%; p0.0005 v healthy controls). Repeat lengths over 100 occurred in five ataxic subjects but in only one control. All seven symptomatic subjects with the SCA 8 expansion had a cerebellar syndrome; four had upper motor neurone signs; and 5/6 assessed had cognitive complaints. There was personality change in two and mood disturbance in three. In published reports, SCA 8 repeat lengths over 91 occurred in approximately 0.5% of the healthy population but were over-represented among ataxic patients (3.4%; p0.0001). The predominant clinical phenotype was cerebellar, with pyramidal signs in 50%, and neuropsychiatric features in some cases.SCA 8 expansion is a risk factor for a cerebellar syndrome, often associated with upper motor neurone and neuropsychiatric features. The expansion occurs unexpectedly often in the general population.

Background: There are numerous terms to describe seizure-like episodes not explained by disease—for example pseudoseizures, psychogenic seizures and non-epileptic attack disorder. Debates about which is best to use tend to centre around theoretical arguments and not scientific evaluation. In this study we examine the meanings of these labels for patients, which have the least potential to offend, and consequently to provide a more positive basis for further management. Methods and results: We interviewed 102 consecutive general neurology outpatients who were asked to consider a scenario that they were being given a diagnosis by a doctor after experiencing a blackout with normal tests. We investigated 10 different diagnoses for blackouts with six different connotations. Three of these connotations—‘putting it on', ‘mad' and ‘imagining symptoms'—were used to derive an overall ‘offence score'. Using this score some labels were highly offensive, e.g. ‘symptoms all in the mind' (89%) and ‘hysterical seizures' (48%). There were no significant differences between the labels ‘pseudoseizures', ‘psychogenic seizures' and ‘non-epileptic attack disorder'. ‘Stress-related seizures' and ‘functional seizures' were significantly less offensive than these three diagnoses and were equivalent to ‘tonic–clonic' and ‘grand mal'. Conclusions: Many labels for seizures unexplained by disease are potentially offensive to patients. The search for labels that accurately describe the phenomenon, can be used by patients, doctors and researchers and enhance trust and recovery is worthwhile and amenable to scientific study.

One hundred years on from the first world war, Stoyan Popkirov and colleagues review the German and British medical films that tell the same story of illness and healing

Background Studies from our group have shown that around half of patients presenting with cauda equina syndrome (CES) have normal imaging, many of whom may have evidence of a functional neurological disorder. We have also shown high rates of comorbidity of functional neurological disorders in patients with idiopathic chronic urinary retention (including Fowler9s syndrome). Aims To determine what proportion of patients with ‘scan negative’ CES and chronic dysfunctional voiding have a functional disorder by clinical consensus. To determine distress, disability and ongoing symptoms in patients with ‘scan positive’ and ‘scan negative’ CES three months after symptom onset. To determine frequency of comorbid functional disorders, distress, disability and physical function in patients with voiding dysfunction and a control urological group. Project Plans: The Back or Leg Pain with Bladder symptoms study (BLB) study is a prospective case control study of all patients admitted with possible CES to the Western General Hospital, Edinburgh. The Overview of Comorbidity in Chronic Urinary Retention (OCCUR) study is a prospective cohort study and retrospective notes review of patients with Chronic Urinary Retention (including Fowler9s syndrome) via the urodynamic clinics in Glasgow, Edinburgh and London.

Jon has had a research interest in functional disorders in neurology since 1999 starting with his PhD thesis – a case control study of patients with functional limb weakness. He has published widely in the area including systematic reviews, large cohort studies, imaging studies, exploration of new phenotypes and treatment studies. He has contributed to new diagnostic criteria for DSM-5 and ICD-11 and been involved in promoting functional disorders in training and research. He has made a website for patients at www.neurosymptoms.org which has been translated by other neurologists in to 12 other languages and receives 60 000 hits a month. He runs a weekly functional disorders clinic and as of August 2012 is a National Research Strategy (NHS Scotland) Clinical Fellow. In 2016, he edited a 51-chapter book “Functional Neurologic Disorders” with longstanding colleague Alan Carson and Mark Hallett of NIH as part of the “Handbook of Clinical Neurology” series. In September 2017, he is co-organising an international meeting on functional neurological disorders in Edinburgh – www.fnd2017.org or sign up at www.fnforum.org. Jon also co-leads the ABN special interest group on this topic (http://abnsigfd.moonfruit.com/) The idea of hysteria has been around, overtly anyway, since Hippocrates. Historians of psychiatry and medicine have obsessed over Charcot and Freud and their contributions to hysteria although arguably there were many others in the 19th century such as Briquet and Janet who had even more to offer. This talk however will examine the history of functional neurological disorders in the 20th century after Freud. This flurry of interest between 1880s and early 1920s gave way to disinterest, scepticism and concern about misdiagnosis. This was mirrored by increasing professional and geographical divisions between neurology and psychiatry after the First World War. I will explore the reason why these doldrums may have lasted such a long time. In the 1990s the advent of more detailed imaging and videotelemetry may have brought attention to functional disorders although ultimately the diagnosis has always been a positive one based on clinical signs, not normal tests. These disorders are now once again the subject of academic and clinical interest, although arguably still too much on the fringes of neurology and psychiatry. It remains commonplace for historians of Freud and psychoanalysis for example, to assume, incorrectly, that “hysteria” is a historical entity. Revisiting older material provides a rich source of ideas and data for today’s clinical researcher but also offers cautionary tales of theories and treatments that led to stagnation rather than advancement of the field. Patterns of treatment do have a habit of repeating themselves, for example, the current enthusiasm for transcranial magnetic stimulation mirrors the excitement about electrotherapy in the 19th century. For these reasons, an understanding of the history of functional disorders in neurology is arguably more important than it is for other areas of other neurological practice.

Functional disorders are one of the most common diagnoses in neurologic practice, but this is not reflected in current classification systems. The 11th revision of the World Health Organization's International Classification of Diseases (ICD-11) in 2017 offers an opportunity for these disorders to appear within both neurologic and psychiatric categories for the first time. We discuss the rationale for this proposal and highlight the potential benefits for health professionals and patients.

Background Patients with functional motor disorder (FMD) including weakness and paralysis are commonly referred to physiotherapists. There is growing evidence that physiotherapy is an effective treatment, but the existing literature has limited explanations of what physiotherapy should consist of and there are insufficient data to produce evidence-based guidelines. We aim to address this issue by presenting recommendations for physiotherapy treatment. Methods A meeting was held between physiotherapists, neurologists and neuropsychiatrists, all with extensive experience in treating FMD. A set of consensus recommendations were produced based on existing evidence and experience. Results We recommend that physiotherapy treatment is based on a biopsychosocial aetiological framework. Treatment should address illness beliefs, self-directed attention and abnormal habitual movement patterns through a process of education, movement retraining and self-management strategies within a positive and non-judgemental context. We provide specific examples of these strategies for different symptoms. Conclusions Physiotherapy has a key role in the multidisciplinary management of patients with FMD. There appear to be specific physiotherapy techniques which are useful in FMD and which are amenable to and require prospective evaluation. The processes involved in referral, treatment and discharge from physiotherapy should be considered carefully as a part of a treatment package.

OBJECTIVE: Somatic symptoms unexplained by disease are common in all medical settings. The process of identifying such patients requires a clinical assessment often supported by clinical tests. Such assessments are time-consuming and expensive. Consequently the observation that such patients tend to report a greater number of symptom has led to the use of self-rated somatic symptom counts as a simpler and cheaper diagnostic aid and proxy measure for epidemiological surveys. However, despite their increasing popularity there is little evidence to support their validity. METHODS: We tested the score on a commonly used self-rated symptom questionnaire- the Patient Health Questionnaire (PHQ 15) (plus enhanced iterations including an additional 10 items on specific neurological symptoms and an additional 5 items on mental state) for diagnostic sensitivity and specificity against a medical assessment (with 18 months follow-up) in a prospective cohort study of 3781 newly attending patients at neurology clinics in Scotland, UK. RESULTS: We found 1144/3781 new outpatients had symptoms that were unexplained by disease. The patients with symptoms unexplained by disease reported higher symptoms count scores (PHQ 15: 5.6 (95% CI 5.4 to 5.8) vs 4.2 (4.1 to 4.4) p

OPINION STATEMENT: For the treatment of functional motor disorder, we recommend a three-stage approach. Firstly, patients must be assessed and given an unambiguous diagnosis, with an explanation that helps them understand that they have a genuine disorder, with the potential for reversibility. A key ingredient is allowing the patients to describe all of their symptoms as well as their ideas about what may be wrong. The patient should clearly understand that the positive diagnosis is based on the presence of typical signs (e.g., Hoover's sign for paralysis, entrainment test for tremor) that, in and of themselves, indicate the potential for reversibility. We suggest an approach that avoids the assumption that psychological stressors in the patient's life are causing the symptoms. The symptoms themselves are often the main stressor. Insisting that there must be others often leads to a frustrated doctor and an angry patient. Rather, at this initial stage, we encourage exploration of mechanisms - e.g., triggering of symptoms by pain, injury, or dissociation - and a discussion of how symptoms manifest as "abnormal motor programs" in the nervous system.Secondly, further time spent exploring the diagnosis, treating comorbidity, and, in the context of a multidisciplinary team, experimenting with altered movements and behaviors may benefit some patients, without the need for more complex intervention.Thirdly, some patients do require more complex treatment, often with a combination of physical rehabilitation and psychological treatments. Hypnosis, sedation, and transcranial magnetic stimulation may have a role in select patients.Finally, although they have confidence in the diagnosis, many patients do not respond to treatment. Ultimately, however, patients with functional motor disorder may have much greater potential for recovery than health professionals often consider.

Objective With the identification of mutations in the conserved telomere maintenance component 1 (CTC1) gene as the cause of Coats plus (CP) disease, it has become evident that leukoencephalopathy with calcifications and cysts (LCC) is a distinct genetic entity. Patients and Methods A total of 15 patients with LCC were identified from our database of patients with intracranial calcification. The clinical and radiological features are described. Results The median age (range) at presentation was 10 months (range, 2 days-54 years). Of the 15 patients, 9 presented with epileptic seizures, 5 with motor abnormalities, and 1 with developmental delay. Motor abnormalities developed in 14 patients and cognitive problems in 13 patients. Dense calcification occurred in the basal ganglia, thalami, dentate nucleus, brain stem, deep gyri, deep white matter, and in a pericystic distribution. Diffuse leukoencephalopathy was present in all patients, and it was usually symmetrical involving periventricular, deep, and sometimes subcortical, regions. Cysts developed in the basal ganglia, thalamus, deep white matter, cerebellum, or brain stem. In unaffected areas, normal myelination was present. No patient demonstrated cerebral atrophy. Conclusion LCC shares the neuroradiological features of CP. However, LCC is a purely neurological disorder distinguished genetically by the absence of mutations in CTC1. The molecular cause(s) of LCC has (have) not yet been determined. ispartof: Neuropediatrics vol:45 issue:3 pages:175-82 ispartof: location:Germany status: published

BACKGROUND AND HISTORY It’s the middle of the clinic. Your next patient has a bulging set of case notes and struggles in to the room on two elbow crutches with a hand-written list of 15 somatic complaints. The worst symptom is progressive right leg weakness that has become so bad that any work has been impossible for six months. You have already noted some physical signs. The right leg is dragged like a sack of potatoes and when the patient climbs on the bed the leg is hauled on with both hands. On direct testing there is some ‘collapsing weakness’ even after you’ve cajoled and encouraged the patient. The reflexes are normal. How are you going to clinch the diagnosis of functional weakness? Can Hoover’s sign help you? Time to get some definitions straight. In this article we will use the term functional weakness to refer to medically unexplained weakness

Linear scleroderma "en coup de sabre" (LScs) is associated with neurological complications, the pathogenesis of which is uncertain. A 27 year old woman is reported on who developed epilepsy and focal neurological signs in association with LScs. Brain MRI demonstrated predominantly ipsilateral relapsing and remitting grey and white matter lesions. Analysis of CSF and pathology obtained at brain biopsy provides evidence of an inflammatory process which may be amenable to immunosuppressive treatment.

Background The prognosis of functional (or psychogenic) motor symptoms (weakness and movement disorder) has not been systematically reviewed. Methods We systematically reviewed PubMed for all studies of eight or more patients with functional motor symptoms reporting follow-up data longer than 6 months (excluding studies reporting specific treatments). We recorded symptom duration, physical and psychiatric comorbidity, disability, occupational functioning at follow-up and prognostic factors. Results 24 studies were included. There was heterogeneity regarding study size (number of patients (n)=10 491), follow-up duration clinical setting and data availability. Most studies (n=15) were retrospective. Reported symptom outcome was highly variable. Mean weighted follow-up duration was 7.4 years (in 13 studies where data was extractable). The mean percentage of patients same or worse at follow-up for all studies was 39%, range 10% to 90%, n=1134. Levels of physical disability and psychological comorbidity at follow-up were high. Short duration of symptoms, early diagnosis and high satisfaction with care predicted positive outcome in two studies. Gender had no effect. Delayed diagnosis and personality disorder were negatively correlated with outcome. Prognostic factors that varied between studies included age, comorbid anxiety and depression, IQ, educational status, marital status and pending litigation. Conclusions Existing follow-up studies of functional motor symptoms give us some insights regarding outcome and prognostic factors but are limited by their largely retrospective and selective nature. Overall, prognosis appears unfavourable. The severity and chronicity of functional motor symptoms argues for larger prospective studies including multiple prognostic factors at baseline in order to better understand their natural history.

Background Patients with severe functional (psychogenic) neurological symptoms such as paralysis and fixed dystonia present a therapeutic challenge, particularly if no movement is possible during physiotherapy. Sedation has been discussed as a treatment for functional neurological symptoms for 100 years but technique, use of video and outcome has not been systematically described. Methods Therapeutic sedation of patients with severe functional neurological symptoms with propofol and follow up at a neuroscience centre. Results Of eleven patients (median duration 14 months), five were cured or had major improvement with sedation. At follow up (median 30 months) four were asymptomatic, two were significantly improved and one had minor improvements. We describe a standardized anesthetic and physician technique, refined over consecutive treatments. Conclusion In carefully chosen patients, therapeutic sedation with propofol can be a useful adjunctive treatment for patients with severe functional neurological symptoms. The treatment deserves randomized evaluation.

Research on the subjective experience of dissociative (psychogenic non-epileptic) seizures (DS) is dominated by that on objective semiology.1 ,2 Patients with DS tend not to spontaneously volunteer any warning symptoms, nor feelings of resistance to seizure onset, both of which are more common in epilepsy.3 But when an ‘aura’ is sought, studies in DS have found rates of 25–60%,4–7 typically with somatic symptoms of autonomic arousal such as dizziness, depersonalisation and chest tightness along with cognitive symptoms such as fear of losing control. This cluster of symptoms may sometimes meet criteria for a panic attack or an episode of hyperventilation, but in many cases, does not. This suggests that patients with DS do have prodromal symptoms but generally don't want to talk about them. Goldstein and Mellers6 proposed that DS are a “paroxysmal, dissociative response to heightened arousal in the absence of raised general anxiety levels”. As we explored this ‘dissociative response’ in more detail with patients, we met some who were willing to admit that the attack was, at times, a welcome relief from their unbearable prodromal symptoms. This led us to discover that a number of our patients were aware of exerting conscious control over the onset of their blackouts. Between August 2010 to …

Functional neurological symptoms, or conversion disorders, have been described since the beginning of written medicine. Although originally attributed to a uterine disorder and first described as in a gynaecological text by the Middle Ages they were recognised as disorder of brain then of psyche. In the nineteenth century Charcot described them as being caused by a functional neurological lesion; one that evaded the availbel techniques of the day. A century on the technique shave become available that allow a search for these putative mechansims. In this chapter we review diagnosis and clinical presentation of functional symptoms which allows to speculate on potential mechanisms and hypothesise about potential target areas and paradigms for imaging. We then review all the imaging studies up until the end of 2012. To date the imaging conducted, both structural and functional, allow to be reasonably secure in concluding that functional neurological symptms are a genuine entity and not simply feigned. We cannot however make any claims that the underpinning mechansims of FNS have been resolved. We speculate that there are overly sensitive amygdala fear responses (i.e. abnormal response to stimuli (even objectively neutral stimuli), possibly conditioned by previous learning experiences) that drive changes in networks mediating ­perceptual experiences and/or movement plans. These changes, in the presence of abnormal self-directed attention (cf. prefrontal activations in functional imaging studies), are capable of producing movements or perceptual experiences which are not associated with a normal sense of self-agency and are therefore interpreted by patients as involuntary symptoms of an underlying disease.

Parry–Romberg syndrome (PRS) is a rare neurocutaneous disorder characterized by progressive facial hemiatrophy. In this study, 205 patients with PRS were surveyed using the Internet. Estimates of the frequency of limb involvement (19%), epilepsy (11%), and other clinical and etiologic features were obtained. There was a wide range of age at onset and considerable diagnostic overlap with scleroderma “en coup de sabre.”

Successive attempts at rebranding may be behind at least some of the proliferation of terms we have at our disposal when describing patients with what are now most often referred to as "psychogenic," "conversion," or "somatoform" symptoms. The most popular term in the movement disorder literature, "psychogenic," provides the aetiology of the disorder within the name, indicating that the symptoms are "born of the mind." Here we argue that it is logical to stop using a term that defines the disorder with regard to a poorly defined aetiology that is not supported by current evidence, and, instead, to use a broad term-functional-not as a "polite eponym" but as a term that is freer from such assumptions and does not reinforce dualistic thinking. The main argument for change is not political or even practical, but scientific.

Gerald Stern's case reminds us how neurologists, and in fact doctors in general, may have lost their way in certain aspects of their job. Each consultant, locked in their organ specialty, turned the patient upside down looking for a cause for her symptoms but left her without a diagnosis. ‘Nothing wrong with that!’ you cry, as a card-carrying ‘uncertainty tolerating’ neurologist, and we would agree in some respects. But the fact that the patient had a range of symptoms that responded dramatically to a nameless white powder (which we assume was inactive) means either her condition resolved coincidentally or perhaps more plausibly was ‘functional’. That she …

Functional (psychogenic) motor symptoms are commonly encountered in clinical neurology. Physiotherapy has face validity as a treatment for such symptoms and, anecdotally, referral of patients with functional motor symptoms (FMS) to physiotherapy services is common practice by neurologists. Here the authors sought to explore exposure to and attitudes towards patients with FMS among neurophysiotherapists.The authors used an internet survey to gather information on the knowledge and attitudes of patients with FMS among 1402 members of a UK neurophysiotherapy organisation.The response rate was 61%. Most physiotherapists saw patients with FMS, and for 25% of respondents these patients made up over 10% of their workload. Respondents were moderately interested in treating these patients (ranking them sixth out of 10 neurological conditions), but had low self-judged knowledge. Most respondents felt physiotherapy had more to offer patients with FMS, but felt poorly supported by referring neurologists and by inadequate service structures.Neurologists frequently refer patients with FMS to neurophysiotherapy services. Physiotherapists in general are interested in treating such patients and feel physiotherapy to be an appropriate treatment. However, inadequate service structures, knowledge and support from non-physiotherapy colleagues are judged to be barriers to provision of care.

Symptoms such as pain, weakness, fatigue and sensory disturbance often lack a disease explanation. Such symptoms can be a major problem for both doctor and patient, often challenging the way in which we conceptualise and practise medicine. Symptoms are the patient’s subjective experience of what is occurring in their body. By contrast, doctors are trained to find diseases such as multiple sclerosis that offer an explanation for these symptoms. When there is no disease present it becomes tempting to suggest that the symptom is not real or psychogenic. However, symptoms appear for multiple reasons, including physiological factors, psychological factors, behavioural response and cultural and social factors. For some patients disease pathology is a major factor in causing symptoms but in others it appears to be minor or absent entirely. A patient does not have to have a disease in order to have genuine symptoms.

Sporadic Creutzfeldt-Jakob disease (sCJD) and N-methylD-aspartate receptor antibody (NMDAR-Ab) encephalitis [1], share similar clinical features including prominent psychiatric symptoms, memory problems, and seizures (Table 1). Previously, other forms of autoimmune encephalitis have been misdiagnosed as CJD, including Hashimoto’s and voltage-gated potassium channel complex antibody-associated encephalopathies [2, 3]. Here, conversely, we describe two patients presenting with progressive cognitive impairment and NMDAR-Abs with confirmed sCJD at post mortem. A retrospective review of 100 pathologically confirmed cases of sCJD seen by the UK National CJD surveillance unit [4] over the past 2 years identified six tested for NMDAR-Abs at presentation in whom results were available: four were negative and two positive. Both NMDAR-Ab positive cases presented with subacute cognitive decline and behavioral disturbance leading to serum testing for the antibodies. Patient A progressed to an akinetic mute state over 3 months and patient B over 9 months. Both had basal ganglia high signal on MRI brain imaging, a positive 14-3-3 immunoassay and elevated S100b level. Patient A twice had positive serum NMDARAb levels at dilutions up to 1:80. He was given 3 days of intravenous methylprednisolone and continued to deteriorate, dying 9 days later. Patient B deteriorated over 9 months. Some months after discharge to palliative care, his earlier positive serum NMDAR-Ab titre of 1:80 was discovered. Both had sporadic CJD confirmed at post mortem. CSFs were negative for NMDAR-Abs in all six cases. The presentation and clinical evolution in these two cases was consistent with sCJD and the positive NMDARAb results were surprising. Detecting NMDAR-Abs involves the demonstration of autoantibodies binding to a cell line expressing the NR1 and NR2B subunits [5]. All positive sera (tested at 1:20 initially) are checked against cells expressing other antigens, and the cells alone, to exclude any false-positive results [5, 6]. Positive results in healthy individuals or other disease controls are very rare; not found in 160 neurological disease controls [5]. The existence of these antibodies in two out of six patients with confirmed CJD is therefore intriguing. The titres were low, but at a level seen in patients with NMDAR-Ab encephalitis. One possibility is that these antibodies occurred as a secondary event resulting from an immune response to NMDARs released from damaged neurons. An altered composition of NMDAR has been implicated in cell death Electronic supplementary material The online version of this article (doi:10.1007/s00415-012-6489-3) contains supplementary material, which is available to authorized users.

Objectives: Functional (psychogenic or somatoform) symptoms are common in neurology clinics. Cognitive-behavioral therapy (CBT) can be an effective treatment, but there are major obstacles to its provision in practice. We tested the hypothesis that adding CBT-based guided self-help (GSH) to the usual care (UC) received by patients improves outcomes. Methods: We conducted a randomized trial in 2 neurology services in the United Kingdom. Outpatients with functional symptoms (rated by the neurologist as “not at all” or only “somewhat” explained by organic disease) were randomly allocated to UC or UC plus GSH. GSH comprised a self-help manual and 4 half-hour guidance sessions. The primary outcome was self-rated health on a 5-point clinical global improvement scale (CGI) at 3 months. Secondary outcomes were measured at 3 and 6 months. Results: In this trial, 127 participants were enrolled, and primary outcome data were collected for 125. Participants allocated to GSH reported greater improvement on the primary outcome (adjusted common odds ratio on the CGI 2.36 [95% confidence interval 1.17–4.74; p = 0.016]). The absolute difference in proportion “better” or “much better” was 13% (number needed to treat was 8). At 6 months the treatment effect was no longer statistically significant on the CGI but was apparent in symptom improvement and in physical functioning. Conclusions: CBT-based GSH is feasible to implement and efficacious. Further evaluation is indicated. Classification of evidence: This study provides Class III evidence that CBT-based GSH therapy improves self-reported general health, as measured by the CGI, in patients with functional neurologic symptoms.

International audience; Objectives: To determine disability, distress and employment status in new neurology patients with physical symptoms unexplained by organic disease and to compare this with that of patients with symptoms explained by organic disease. Methods: As part of a cohort study ( the Scottish Neurological Symptoms Study) neurologists rated the extent to which each new patient's symptoms were explained by organic disease. In this paper patients whose symptoms were rated as "not at all", or only "somewhat" explained by disease were considered cases and those whose symptoms were "largely" or "completely" explained by disease were designated as controls. All patients completed self-ratings of disability and health status (SF-12) and emotional distress (HADS) and also reported their employment and state financial benefit status. Cases and controls were compared. Results: 3,781 patients were recruited; 1,144 (30%) were cases and 2,637 (70%) controls. Cases had slightly worse physical health status (SF12 score 42 vs 44; difference in means= -1.7 (95% CI -2.5 to -0.9)) and worse mental health status (SF12 score 43 vs 47; difference in means= -3.5 (95% CI -4.3 to -2.7). Unemployment was similar in cases and controls (50% v 50%) but cases were more likely to not to be working because of their health (54% v 37% (of the 50% not working) ; OR 2.0 (95% CI 1.6 to 2.4) and also more likely to be receiving disability related state financial benefits (27% v 22%; OR 1.3 (95% CI 1.1 to 1.6)). Conclusions: New neurology patients with symptoms unexplained by organic disease had greater disability, distress and more disability related state financial benefits than those whose symptoms were explained by disease.

The recent review by Koelen and colleagues[1][1] of psychotherapy for severe somatoform disorder is welcome in highlighting the need for better evidence in this area. It has unfortunately omitted a number of relevant studies, especially relating to conversion disorder. One major reason for this is

Neurologic symptoms such as weakness or abnormal movements that are inconsistent and incongruent with neurologic disease can be described as functional, psychogenic, nonorganic, conversion, or dissociative symptoms. These symptoms often represent a clinical dilemma and a challenge for the clinician. This article provides practical advice on making an accurate diagnosis, options for explaining the diagnosis to the patient, and considering further treatment.

BackgroundPatients whose symptoms are ‘unexplained by disease’ often have a poor symptomatic outcome after specialist consultation, but we know little about which patient factors predict this. We therefore aimed to determine predictors of poor subjective outcome for new neurology out-patients with symptoms unexplained by disease 1 year after the initial consultation.MethodThe Scottish Neurological Symptom Study was a 1-year prospective cohort study of patients referred to secondary care National Health Service neurology clinics in Scotland (UK). Patients were included if the neurologist rated their symptoms as ‘not at all’ or only ‘somewhat explained’ by organic disease. Patient-rated change in health was rated on a five-point Clinical Global Improvement (CGI) scale (‘much better’ to ‘much worse’) 1 year later.ResultsThe 12-month outcome data were available on 716 of 1144 patients (63%). Poor outcome on the CGI (‘unchanged’, ‘worse’ or ‘much worse’) was reported by 482 (67%) out of 716 patients. The only strong independent baseline predictors were patients' beliefs [expectation of non-recovery (odds ratio [OR] 2.04, 95% confidence interval [CI] 1.40–2.96), non-attribution of symptoms to psychological factors (OR 2.22, 95% CI 1.51–3.26)] and the receipt of illness-related financial benefits (OR 2.30, 95% CI 1.37–3.86). Together, these factors predicted 13% of the variance in outcome.ConclusionsOf the patients, two-thirds had a poor outcome at 1 year. Illness beliefs and financial benefits are more useful in predicting poor outcome than the number of symptoms, disability and distress.