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Dissociative (non-epileptic) seizures are one of the three major causes of transient loss of consciousness. As such, their treatment cannot be left to superspecialised experts. In this article I draw on personal experience to suggest ways to tackle some challenges that commonly arise after diagnosing dissociative seizures, focusing on three issues: “I want to know what is wrong with me,” “I hear what you are saying but it doesn’t apply to me” and “What if I have a seizure?” The suggestions detail both actions and words that may help at a crucial point in the patient’s journey. If handled well, the process can leave the patient better equipped to understand their seizures and to engage in further treatment; if handled badly, patients may be left more traumatised, angry and with additional disability.

A growing body of evidence suggests more catastrophising cognitions are present in patients with psychogenic non-epileptic seizures (pwPNES) compared to patients with epilepsy (PWE). However, the perseverative aspect of catastrophic thinking including repetitive negative thinking (RNT) has had little attention. In this study pwPNES (n=26) and PWE (n=29) completed self-reported questionnaires including Perseverative Thinking Questionnaire to assess levels of RNT and a modified version of the Safety Behaviors and Catastrophizing Scale to assess catastrophising of seizures. Participants also completed the PHQ-9 and GAD-7 to provide measures of anxiety and depression. Patients with PNES reported significantly higher levels of RNT, catastrophising of seizures, and depression and anxiety than patients with epilepsy. Additionally, the effect of RNT was independent to effects of all other factors in its contribution to PNES suggesting it would serve as a good target for psychological therapy.ajwhitfield1878@gmail.com

BackgroundThere are three common causes of Transient Loss of Consciousness (TLOC), syncope, epileptic and psychogenic nonepileptic seizures (PNES). Many individuals who have experienced TLOC initially receive an incorrect diagnosis and inappropriate treatment. Whereas syncope can be distinguished from the other two causes relatively easily with a small number of yes/no questions, the differentiation of the other two causes of TLOC is more challenging. Previous qualitative research based on the methodology of Conversation Analysis has demonstrated that epileptic and nonepileptic seizures are described differently when patients talk to clinicians about their TLOC experiences. One particularly prominent difference is that epileptic seizure descriptions are characterised by more formulation effort than accounts of nonepileptic seizures.AimThis research investigates whether features likely to reflect the level of formulation effort can be automatically elicited from audio recordings and transcripts of speech and used to differentiate between epileptic and nonepileptic seizures.MethodVerbatim transcripts of conversations between patients and neurologists were manually produced from video and audio recordings of interactions with 45 patients (21 epilepsy and24 PNES). The subsection of each transcript containing the patients account of their first seizure was manually extracted for the analysis. Seven automatically detectable features were designed as markers of formulation effort. These features were used to train a Random Forest machine learning classifier.ResultsThere were significantly more hesitations and repetitions in descriptions of first epileptic than nonepileptic seizures. Using a nested leave-one-out cross validation approach, 71% of seizures were correctly classified by the Random Forest classifier.ConclusionsThis pilot study provides proof of principle that linguistic features that have been automatically extracted from audio recordings and transcripts could be used to distinguish between epileptic seizures and PNES and thereby contribute to the differential diagnosis of TLOC. Future research should explore whether additional observations can be incorporated into a diagnostic stratification tool. Moreover, future research should explore the performance of these features when they have been extracted from transcripts produced by automatic speech recognition and when they are combined with additional information provided by patients and witnesses about seizure manifestations and medical history.

Objectives/AimsWe used our automated cognitive assessment tool to explore whether responses to questions probing recent and remote memory could aid in distinguishing between patients with early neurodegenerative disorders and those with Functional Cognitive Disorders (FCD).Hypotheses: pwFCD would have no significant differences in pause to speech ratio and measures of linguistic complexity compared to healthy controls. pwFCD would have significant differences in pause to speech ratio and measures of linguistic complexity compared to pwMCI and pwAD.MethodsWe recruited 15 participants with FCD, MCI and AD each as well as 15 healthy controls. Participants answered 12 questions posed by the ‘Digital Doctor’. Automatic processing of the audio-recorded answers involved automatic speech recognition including detecting length of pauses. Two questions probe recent memory, exploring knowledge of current affairs. Two probe remote memory, asking for autobiographical details.We analysed the data using: Pause to speech time ratio. Moving average type token ratio (MATTR): An automated measure of vocabulary richness. Computerised propositional idea density rater (CPIDR): An automated measure of propositional idea density.ResultsThere was a significant difference in the pause to speech ratio for recent memory questions for HC versus AD (P=0.0012) and MCI (pConclusionsThis study rejects both hypotheses. However, the data supports the application of linguistic measures to recent and remote memory questions in distinguishing those with MCI & AD from HC’s. Further work will investigate the utility of incorporating additional measures of lexical and grammatical complexity (word frequency, sentence structure). Longitudinal study will provide insights into which features may predict stability in FCD and HC’s and progression from MCI to AD, supporting the system’s promise as a monitoring tool.

IntroductionReferrals to specialist memory clinics have increased out of proportion to the incidence of dementia. Time and financial pressures are consequently exerted on a service striving to deliver high quality care. We have developed a fully automated ‘Digital Doctor’ with the aim of providing pre-clinic risk stratification and ongoing monitoring for patients with memory concerns.MethodsWe recruited 15 participants with Functional Memory Disorder (FMD), Mild Cognitive Impairment (MCI) and Alzheimer’s disease each as well as 15 healthy controls. Participants answered 12 questions posed by the ‘Digital Doctor’. Audio and visual data is analysed using diarization and automatic speech recognition tools and machine learning classifiers.ResultsThe ‘Digital Doctor’ can distinguish between neuro-degenerative dementia and FMD with an accuracy of 95%. We will have results of a 4-way classification accuracy (HC, FMD, MCI & AD) at time of conference.DiscussionWe demonstrate the potential value of the ‘Digital Doctor’ as a stratification and triage tool. Accuracy will be improved with greater number of users and inclusion of fluency and picture description data. Patients at low risk could avoid the burden of a clinic appointment, whilst patients with higher risk could benefit from a more streamlined service.

The mimics and chameleons of functional symptoms in neurology could be a whole textbook of neurology. Nevertheless, there are some recurring themes when things go wrong, notably diagnostic bias introduced by the presence or absence of psychiatric comorbidity or life events, neurological diseases that look 'weird' and lack of appreciation of the more unusual features of functional symptoms themselves.

AimsTo quantify the frequency, characteristics, geographical variation and costs of emergency hospital care for suspected seizures.DesignCross-sectional study using routinely collected data (Hospital Episode Statistics).SettingThe National Health Service in England 2007–2013.ParticipantsAdults who attended an emergency department (ED) or were admitted to hospital.ResultsIn England (population 2011: 53.11 million, 41.77 million adults), suspected seizures gave rise to 50 111 unscheduled admissions per year among adults (≥18 years). This is 47.1% of unscheduled admissions for neurological conditions and 0.71% of all unscheduled admissions. Only a small proportion of admissions for suspected seizures were coded as status epilepticus (3.5%) and there were a very small number of dissociative (non-epileptic) seizures. The median length of stay for each admission was 1 day, the median cost for each admission was £1651 ($2175) and the total cost of all admissions for suspected seizures in England was £88.2 million ($116.2 million) per year. 16.8% of patients had more than one admission per year. There was significant geographical variability in the rate of admissions corrected for population age and gender differences and some areas had rates of admission which were consistently higher than the average.ConclusionsOur data show that suspected seizures are the most common neurological cause of admissions to hospital in England, that readmissions are common and that there is significant geographical variability in admission rates. This variability has not previously been reported in the published literature. The cause of the geographical variation is unknown; important factors are likely to include prevalence, deprivation and clinical practice and these require further investigation. Dissociative seizures are not adequately diagnosed during ED attendances and hospital admissions.

The distinction between the different causes of blackouts is an important and challenging clinical task. Given that treatment is very diagnosis-specific, therapeutic success depends entirely on the correct categorisation of the problem. However, despite impressive technological advances in brain imaging and improved access to tests such as video-EEG monitoring and tilt-table testing, the act of taking and interpreting the patient’s history is still the most important diagnostic tool in the evaluation of patients presenting with blackouts; in many if not most cases it provides the only diagnostic pointers. Here we discuss the potential and limitations of using factual information ( what patients say about their attacks) and summarise the findings of a number of recent studies which suggest that taking note of how patients talk can help get the diagnosis right.

Objectives: To determine whether patients with psychogenic non-epileptic seizures (PNES) have evidence of maladaptive personality, and whether they have a single or several different typical pathological personality profiles. Methods: Patients were recruited from the department of epileptology, Bonn, Germany. In all, 85 patients with PNES and 63 with epilepsy completed a postal questionnaire including the dimensional assessment of personality pathology – basic questionnaire (DAPP-BQ). The DAPP-BQ was also completed by 100 healthy volunteers. The groups were compared and the PNES group was subjected to cluster analysis. Results: Patients with PNES had a greater degree of personality abnormality than clinical and non-clinical controls. There were several clusters of personality pathology. The profile of the largest cluster (n = 43) resembled that found in borderline personality disorder, that of the second largest (n = 37) was characterised by an overly controlled personality, that of the third (n = 4) was similar to the profile in avoidant personality disorder. Outcome differed between clusters. Conclusions: Maladaptive personality is common in patients with PNES. PNES are associated with several distinct profiles of pathological personality. This is relevant because outcome differed between profiles.

Introduction Suspected seizures are a common reason for admission to hospital. Rates of unscheduled admission are of interest as it may reflect the quality of local services for epilepsy. Methods We searched the Hospital Episodes Statistics (HES) in-patient database for unscheduled/emergency NHS admissions in adults in England between 1 April 2007 – 31 March 2013, and categorised them by ICD-10 diagnosis (epilepsy/seizure/convulsion, G40, G41, R56.8). Variation in admission was analysed by funnel plots. Results There were 8 17 030 admissions for neurological conditions during the study period (mean=1 36 171 per year). 38.9% of these were for epilepsy/seizure/convulsion, and this comprised the major neurological cause for admission (0.75% of all emergency admissions). There was geographical variability in the rate of admissions. There were peaks of rates of admission in middle and old age. Men were admitted more commonly than women. The median length of stay was 1 day. The median cost per admission was £1650 (IQR £1090 to 1856, range £0 to £217,998) and the mean total cost per year to the NHS in England was £93 million. Conclusions Suspected seizures are an important cause of neurological admissions to hospital. The significant geographical variability may reflect local variation in quality of care.

Objective To describe two different cases of misrepresentations of functional neurological disorders in mass media (television) and review ethical issues faced by the health professional on viewing these. Method Case series, narrative review and conceptual analysis Results In the first case, a documentary shows emergency management of an individual in apparent status epilepticus who receives intensive invasive management. Follow-up in the documentary suggests the patient retains a diagnosis of epilepsy. However a viewing clinician believes the attack displayed to be non-epileptic in nature. The second case concerns the talk show appearance of a woman experiencing functional neurological symptoms including memory loss and weakness. During the course of the interview, several incorrect statements regarding the aetiology, management and prognosis of functional neurological disorders are made that may lead to inappropriately catastrophizing interpretations of the condition. A viewing clinician is concerned about the effect of these misleading statements on others who may experience similar symptoms. Such cases raise challenging questions regarding clinical workers’ professional responsibilities in ‘informal’ contexts outside established professional-patient relationships, and their relationship with health journalists in mainstream media. The clinician must deliberate whether and how to engage with: the patients involved; the teams responsible for their care; the media outlets producing the content; and the viewing public. We find neither internalist approaches to professional responsibility (such as virtue ethics or care ethics), nor externalist ones (such as the ‘social contract’ model) are able adequately to engage with such situations – the former invoking norms grounded in pre-existing professional-patient relationships, the latter’s guidance being too abstract. We explore these questions through an instrumentalised virtue ethics, emphasising the non-complacency and practical wisdom of virtue ethics, but grounding the normative authority of virtue in a physician’s wider responsibility to serve public health. Conclusion Health workers’ caring responsibilities extend beyond established professional-patient relationships to informal encounters, but must be acted on in a way sensitive to the concrete particulars of the individual case, and to the limitations of the health worker’s knowledge. Health workers can also promote better health through working toward public understanding of conditions in their field, and should engage with healthcare journalists to convey how specific vulnerabilities of patients experiencing conditions they are expert in lead to moral hazard.

Objective The clinical differentiation of memory complaints attributable to progressive neurodegenerative disorders (NDD) leading to dementia and similar complaints due to FMD is a difficult task. Underlying factors include time constraints, vagueness of presenting symptoms and lack of training in early recognition. We created a teaching aid using video clips from memory clinics applying key findings of our conversation analysis studies. Method Participatory design and focus groups were used to produce, develop and validate a digital film based learning resource to aid diagnosis. Four case studies (2 FMD and 2 NDD) were shown following a teaching session with the new teaching aid to one primary care focus group (n=7) and at a regional dementia training day (n=25). Results Focus groups found the resource relevant for integration into practice, meeting the target audience needs. Clinicians (n=32) were able to make a diagnosis but scoring depended on complexity of case. Conclusion Further data collection and development is required but this resource can be developed as an on-line training module on dementia diagnosis.

Objective The putative links between abnormal emotion processing and Functional Neurological Symptoms (FNS) suggest that this patient group might benefit from a psychotherapeutic intervention targeting the perception, naming, tolerance, and expression of emotions. Brief Augmented Psychodynamic Interpersonal Therapy, an intervention designed to address emotion processing difficulties, has previously been associated with improvements in health-related quality of life and psychological distress in patients with FNS. The objective of this study was therefore to determine whether improvements in clinical symptomology following Brief Augmented Psychodynamic Interpersonal Therapy, were associated with improvements in emotion processing. A second exploratory objective was to see if there were any identifying characteristics of patients who made a clinically significant improvement in emotion processing. Method battery of self-report questionnaires was administered to patients with FNS (n=44), prior to and following a course of Brief Augmented Psychodynamic Interpersonal Therapy. Emotion Processing was measured using the Emotion Processing Scale (EPS-25). The Short Form 36 (SF-36), Clinical Outcomes in Routine Evaluations (CORE 10), Patient Health Questionnaire (PHQ-15), and the Brief Illness Perception Questionnaire (BIPQ) measured health-related quality of life, psychological distress, somatic symptom severity, and illness perception respectively. Clinically significant improvements on the EPS-25 were identified using the Reliable Change Index. Patients were subsequently categorised as making a clinically significant improvement or not, and compared on all available demographic and clinical symptomology variables. Results We observed a significant reduction on the EPS-25 (p=0.049) following Brief Augmented Psychodynamic Interpersonal Therapy for FNS. Change scores on the EPS-25 correlated positively with improvements on the mental health subscale of the SF-36 (r=0.634) and the CORE-10 (r=0.673). BIPQ scores were significantly lower (p=0.013) and SF-36 scores were significantly higher (p=0.020) post-therapy. 22% of patients made a clinically significant improvement on the EPS-25. However, we were unable to identify any demographic or clinical symptomology variables which differed significantly between patients who made a clinically significant improvement in emotion processing and those who did not. Conclusion Emotion processing in patients with FNS improved following treatment with Brief Augmented Psychodynamic Interpersonal Therapy. This improvement was related to a reduction in psychological distress and improved mental health-related quality of life. Patients also perceived their symptoms as less threatening and experienced an improved health-related quality of life following intervention. Further work is required to establish characteristics which could identify patients who are particularly likely to improve in emotion processing with Brief Augmented Psychodynamic Interpersonal Therapy for FNS.

Introduction Conversation Analysis (CA) can help with the differential diagnosis of seizure disorders. We investigated if CA could be used in the memory clinic to distinguish neurodegenerative (NDD) from functional memory disorders (FMD). Methods We recruited consecutive, patients newly referred to the Neurology-led memory Clinic. Consultations were video & audio recorded. All participants underwent detailed Neuropsychology testing and MRI. Results 111 patients of 178 approached were recruited (20 ND, 24 FMD, 87 other). We identified profiles of 14 interactional features that can distinguish NDD from FMD consultations based on encounters with 15 patients with NDD and 15 with FMD. Features of NDD included an inability to answer compound questions fully, inability to give detailed examples of memory failures, shorter length of turn and reduced complexity of replies. Prospective analysis of an additional 10 encounters proved that Conversation Analysts could use these features to predict the diagnoses of FMD and ND with high sensitivity and specificity. Conclusions Simple differences in the communication behaviour of patients can help to distinguish between ND and FMD, suggesting that a targeted observation of interactional features could improve screening for ND in primary or secondary or care settings.

AimRecent public policy documents have emphasised the need for healthcare practitioners to give patients choice. As part of a larger qualitative project investigating how neurologists give patients choices, we explored whether evidence of patient choice is associated with higher patient satisfaction.MethodFourteen neurologists and 223 patients were recruited in neurology outpatient clinics in Glasgow and Sheffield. All participants completed post-appointment questionnaires assessing whether choice was offered or perceived. Clinicians also rated the extent to which symptoms were medically explained. Patients completed the Medical Interview Satisfaction Scale-21 (MISS-21).ResultsTwo dominant factors contributed to the total MISS-21 scale, ‘rapport’ and ‘distress-relief’. Regression analysis showed that the extent to which symptoms were medically explained predicted most of the variance on both subscales and the total MISS-21 score. Spearman's rho correlations showed greater patient satisfaction if symptoms were medically explained and if no choice had been offered or perceived.ConclusionThere was no evidence that giving patients choices in interaction increased patient satisfaction with clinical encounters. Our findings suggest that patient satisfaction is most strongly influenced by the extent to which neurologists perceive symptoms as medically explained.

Objective We describe the roll-out of a brief psycho-education intervention for individuals with non-Epileptic Attack Disorder (NEAD), which has been developed for healthcare professionals with limited experience in delivering psychological treatments. Method The psycho-education package is comprised of four one-to-one sessions. The first session provides an explanation of the NEAD diagnosis and introduces the patient to idea that mind and body are linked. The second session teaches behavioural strategies for controlling seizures, including sensory grounding, relaxation and imagery. The third session focuses on identifying and reducing avoidance behaviours that may have been adopted due to seizure symptoms. The final session focuses on values and going forward. Eighteen nurses and therapists attended a training to deliver this package. The two day training intervention included lectures and interactive workshops with actors. The initial roll out stage involved fourteen NHS trusts across the country. Ten months following training, therapists were asked to complete a survey to assess the extent they used this intervention and that they found it to be relevant, comprehensive, and sufficient. Patients with NEAD who attended for psycho-education were also asked to complete pre and post questionnaires including the following measures; Work and Social Adjustment scale (WSAS), Brief Illness Perception Questionnaire (Brief IPQ), Clinical outcomes in routine evaluation (CORE) and the EuroQoL-5D3L and NewQOL quality of life measures. Results Since the training, the package has been delivered in four centres. Recruitment and data collection are ongoing. Preliminary analysis of the pre and post participant data shows that following intervention, participants viewed their seizures as significantly less threatening (IPQ, p=0.002), and reported significantly less distress (CORE, p=0.01) The collection of therapist feedback about the use of the intervention is ongoing. Conclusion It is feasible for healthcare professionals with minimal training in psychological intervention to deliver a brief psycho-education package for individuals with NEAD. Further analysis is needed to understand the full effectiveness of this intervention but preliminary results show that this intervention may have positive effects on the way individuals view their seizures and on their overall wellbeing.

Objective The illness perceptions of the relatives of patients with functional neurological symptom disorders (FNSD) and the relation to patients9 illness perceptions have been little studied. We aimed to compare illness perceptions of relatives of patients with FNSD to those held by patients themselves. We used control pairs with neurological diseases (ND) to examine the specificity of the findings to FNSD. Method Patients with FNSD (functional limb weakness and psychogenic non-epileptic seizures) and patients with ND causing limb weakness and epilepsy, and their relatives, completed adapted versions of the Illness Perception Questionnaire Revised (IPQ-R). Results We included 112 pairs of patients with FNSD and their relatives and 60 ND patient and relative pairs. Relatives of patients with FNSD were more likely to endorse psychological explanations and, in particular stress as a causal factor than patients with FNSD (p Conclusion The main difference in illness perceptions between relatives and patients with FNSD was a tendency for relatives to see psychological factors as more relevant than patients. Some other differences were observed between FNSD relatives and patients but the same differences were also seen in ND pairs. These other differences were therefore not specific to FNSD. Discussion about possibly relevant psychological factors with patients suffering from FNSD may be helped by including relatives.

Objective Our overall aim is to evaluate the clinical and cost effectiveness of specifically adapted Cognitive Behavioural Therapy (CBT) (plus Standardised Medical Care – SMC) in comparison to SMC alone for outpatients with dissociative seizures (DS), within a pragmatic, multi-centre UK-based RCT. This will redress the limited evidence base for psychotherapy in this patient group. Method Adults with DS but without currently active epilepsy will receive their diagnosis and information from a neurologist/epilepsy specialist and their seizure occurrence will be monitored. Three months post diagnosis they will be reviewed by a psychiatrist with interest and expertise in DS who will represent the diagnosis and deliver an information leaflet. Those continuing to experience DS in the previous 8 weeks and meeting other inclusion criteria will, if willing, be consented, undergo baseline assessments and be randomised to receive either 12 sessions of CBT (plus a booster session) as well as SMC or SMC alone. We aim to randomise 298 patients (149 to each arm). DS frequency will be monitored fortnightly and follow-ups will occur at 6 and 12 months post randomisation. An intention to treat analysis will evaluate effectiveness in outcomes relating to seizure frequency severity and freedom, mood and psychological state, quality of life and health service use. A qualitative study will investigate participants’ experiences of the interventions and barriers to participation that might affect subsequent implementation in the NHS. Results Multiple sites in England, Wales and Scotland have been enlisted. Recruitment in neurology clinics has commenced. Training for service user advisors, research workers and CBT therapists has been delivered. Conclusion The design and progress will be described. This is the largest planned trial for patients with DS to date. See ISRCTN05681227 for further details. FUNDING: NIHR HTA reference 12/26/01.

Objective To examine cognitive responses to stress-related stimuli in patients with seizures and to explore whether the response patterns differ in patients with epilepsy, patients with psychogenic non-epileptic seizures (PNES) and healthy individuals. We also aimed to investigate self-perceived and physiological stress responses in patients with epilepsy and those with PNES, compared to healthy individuals, and to examine the relationships between the cognitive, self-perceived and physiological stress responses in patients with seizures. Method A group of 54 patients with refractory seizures admitted for diagnostic video-telemetry assessment (epilepsy n=21, PNES n=26) and 21 healthy volunteers completed self-report questionnaires and performed an emotional Stroop test. Cognitive responses were investigated by exploring attentional biases in responses to neutral versus threatening stimuli comprising generally and socially threatening as well as somatic and seizure-related words. Heart rate variability (HRV) parameters were extracted from resting electrocardiogram recordings taken prior to the Stroop test and used as physiological stress markers. Results Patients with seizures, particularly those with epilepsy, showed greater attentional biases towards threatening information than healthy individuals across all word categories (ps Conclusion Many patients perceive stress as the commonest trigger of their seizures; however, the relationship between psychological and physiological processes relating stress and seizures has been poorly understood. Our findings show that patients with seizures are characterised by a pattern of abnormal attentional vigilance to threatening stimuli, high self-perceived stress and an altered autonomic nervous system responses. The relationships between attentional biases and HRV in patients with epilepsy suggest there may be a reciprocally causative relationship between cognitive responses, PNS tone and seizures. The long-term experience of stress and seizures could lead to a diminished PNS tone, which could exacerbate patients9 attentional responses to stressors. In turn, the biased attentional responses could trigger more frequent physiological stress responses, causing further alterations of the autonomic nervous system and further increasing patients9 vulnerability to stress and seizures.

The 2009 Dementia strategy promoted a ‘memory clinic in every town’. We investigated the patient mix seen in a neurology-led memory clinic. Retrospective review (2004, 2006, 2012) attendees to memory clinic. Prospective review memory clinic from October 2012–Dec 2013. Survey to neurologists in Specialist Interest Group in Cognition (ABN). Survey of local GPs. Results Percentage of attendees with benign memory complaints increased from 30% & 32% in 2004 and 2006 to 55% in 2012. Oct 2012–Dec 2013 >50% attendees do not have dementia or MCI. 9 responders: A mean of 27% of attendees of neurology-led memory clinics in the UK have ‘benign memory complaints’. The following terms were used: ▸ Attentional amnestic disorder, ▸ Attentional cognitive complaints, ▸ Worried well, ▸ Subjective memory complaints, ▸ Subjective memory impairment, ▸ Normal cognitive ageing, ▸ Hypocondrial, ▸ ‘Stress related.’ 4. GPs used; ‘worried well’, ‘benign senescent forgetfulness’, ‘Possible dementia’, ‘mild neurocognitive disorder’ and ‘late life forgetfulness’ & treated with antidepressants but also referred to psychology or memory clinic. People attending memory clinic frequently do not have dementia and currently there is no consensus for diagnostic label or treatment.

Introduction Phenytoin is an effective anticonvulsant for focal epilepsy with the advantage of single daily administration. Its popularity however is in decline, partly because of perception of adverse effects that include gum hypertrophy, hirsutism, osteoporosis and cerebellar ataxia. Whilst phenytoin has been shown to be toxic to cerebellar Purkinje cells in vitro, the frequency, clinical and radiological phenotype and the mechanism of cerebellar degeneration in vivo remains unclear. In this study we aim to identify the frequency and characterise the clinical and radiological phenotype of patients with phenytoin–associated ataxia. Methods Patients were recruited from the epilepsy clinics, Royal Hallamshire Hospital, Sheffield, UK. HLA type and tests for other causes of ataxia were undertaken. The severity of the ataxia was rated using the Scale for the Assessment and Rating of Ataxia (SARA). Patients with clinical evidence of ataxia underwent a 3T MRI including spectroscopy of the cerebellum and had serum phenytoin levels estimated. Results Twenty five patients aged between 32–77 years were recruited. Eleven had focal epilepsy, 6 generalised and 8 unclassified. Age at diagnosis ranged from childhood to 74 years with duration 2–67 years. Fifteen patients (60%) continued taking phenytoin from the time of diagnosis. Mean duration of phenytoin treatment was 21 years and mean dose 350mg. Seventeen patients (68%) complained of poor balance and 13/25 (52%) had clinical evidence of ataxia on examination. All 13 had gait ataxia and the majority also had limb ataxia. SARA scores ranged from 3–17 (median 6). Gait, stance and heel–shin slide subgroups of the SARA scale were mostly affected. Eight out of 17 (47%) patients had the HLA DQ2/DQ8 type, strongly associated with autoimmune ataxias. Volumetric 3T MRI imaging and spectroscopy data were available in 6 of the 13 patients with ataxia. Cerebellar atrophy and abnormal spectroscopy of the vermis was found in three patients. Two of these patients had circulating antigliadin antibodies that signify sensitivity to gluten. Two patients with no ataxia but who had taken phenytoin for more than 20 years had no cerebellar atrophy and normal spectroscopy. Mean phenytoin level was 18 mg/L (5–20 mg/L). One patient had a serum phenytoin level of 65.8 mg/L. Conclusion 68% of patients on long–term phenytoin complained of poor balance and 52% had clinical evidence of ataxia. Cerebellar abnormalities on imaging were seen in 3/6 (50%) patients with ataxia. The primarily vermian involvement seen is similar to that of autoimmune ataxias. In those patients with vermian abnormalities an additional potential cause of ataxia was identified in the form of serological evidence of sensitivity to gluten. It is possible that the mechanism of cerebellar damage in patients on long term phenytoin may involve other mechanisms such as immune mediated damage in genetically (HLA DQ2/DQ8) susceptible individuals and/or sensitivity to gluten.

In ordinary neurological practice, the diagnosis of psychogenic non-epileptic seizures (PNES) is often made by exclusion, or on the grounds that seizures lack features of epilepsy. With the exception of linguistic and interactional markers in the conversation with the patient about their symptoms,1 few ‘positive’ signs of PNES have been described. Therefore, this effort to improve our knowledge of the clinical semiology of PNES is welcome. Hubsch et al 2 used multiple correspondence analysis and cluster analysis of video-EEG recordings of PNES to identify five clinical subtypes of attack. The authors conclude that their classification of PNES may provide useful criteria for clinical diagnosis, presumably by giving clinicians a series of ‘models’ they can hold in their heads for evaluation of clinical descriptions of …

Abnormalities of smell and taste have been described in some neurodegenerative diseases including Alzheimer's dementia, idiopathic Parkinson's disease, Huntington's chorea, Korsakoff's syndrome, Pick's disease, the parkinsonian dementia complex of Guam, and amyotrophic lateral sclerosis.1 Hyposmia and hypogeusia are a feature of normal aging but they have not been recorded as a prominent early feature in previous reports of variant Creutzfeldt-Jakob disease (vCJD).2-5 We describe a patient with vCJD whose first symptoms included deficits of taste and smell. At the time of his initial neurological assessment, this 54 year old ceramic tiler had a 12 month history of loss of taste and smell, anxiety, low mood, and unusually short temper. He first became aware that something was wrong when he lost the ability to differentiate the taste of tea from that of beer. Loss of taste and personality change progressed gradually. He began to crave vanilla ice cream although he had never liked sweet foods in the past. He became …