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Background Patients with cancer are often at risk or suffer from some form of malnutrition, which could be attributed to their disease progression or the adverse effects of cancer treatment. Protected mealtime and offering assistance during mealtime may not improve their nutritional status but would allow them to enjoy their meal and improve patient satisfaction. Objectives The aim of this project was to promote best practice in protecting mealtimes in hematology-oncology patients. Methods The current project adopted a clinical audit methodology using the Joanna Briggs Institute Practical Application of Clinical Evidence System tool. Four audit criteria that represent best practice recommendations for protected mealtimes were used. A pre-implementation audit was conducted followed by introduction of multiple interventions identified from the Getting Research into Practice analysis. A post implementation audit was conducted to determine the change of practice. In addition, a sustaining audit was conducted at six months after implementing the follow-up plan. Results The four criteria showed an improvement: nurses ensured a pleasant eating environment around the patient cubicle (from 43% to 93% compliance), all nurses received education on mealtime care (no change - 100% sustenance), nurses conducted an assessment of the patients on admission on their ability to eat (from 95% to 100% compliance) and minimized unnecessary clinical interventions during mealtimes (92% to 89% compliance). The decline of 3% in the compliance rate of the last criterion could be attributed to the high turnover of patients during the post implementation audit period. The post implementation results at six months showed audit results being sustained at 100% compliance in all criteria. Conclusion The results of this project demonstrated that improvement in best practice is possible in a busy oncology ward in the presence of great leadership, teamwork, empowerment and role modeling.

The first comprehensive description of Gilles de la Tourette syndrome as a neurological condition dates back to 1885 when Georges Gilles de la Tourette (1857–1904) published his case series of nine patients sharing the clinical triad of tics, echolalia, and coprolalia. At the time, Gilles de la Tourette was working at the Salpetriere Hospital in Paris, France, under the guidance of Jean-Martin Charcot (1825–1893), one of the fathers of modern neurology. It was in fact Charcot who credited Gilles de la Tourette with the description of the syndrome that was named after him and gave him eponymous fame. Gilles de la Tourette's character was described as talented but erratic, and his life was relatively short for current standards but far from uneventful. Gilles de la Tourette syndrome, the complex neurodevelopmental disorder characterized by multiple motor and vocal tics, is undoubtedly his main legacy. The importance of the 1885 article was greatly underestimated at the time of its publication, partly because Gilles de la Tourette's main interests lied in the field of hysteria and hypnosis. Throughout the first half of the XX century, his name was progressively neglected, as the psychoanalytic paradigm that prevailed associated tics with rare and somewhat bizarre psychologically driven manifestations. However, Gilles de la Tourette's posthumous fame resurged during the 1960s, concomitant to a paradigm shift whereby the development of neurobiological models drove the renaissance of the scientific study of tic disorders, together with a reappraisal of Gilles de la Tourette's initial contribution.

Introduction: Converging evidence from both clinical and experimental studies has shown that Tourette syndrome (TS) is not a unitary condition, but a cluster of multiple phenotypes, which encompass both tics and specific behavioural and cognitive symptoms (mainly attention-deficit and hyperactivity disorder and obsessive-compulsive disorder). Methods: We conducted a narrative review of the recent literature on the cognitive neuropsychiatry of TS. Results: Although clinical research has shown that TS is not associated with cognitive deficits per se, the findings of recent studies have suggested the presence of subtle alterations in specific cognitive functions. A promising line of research on imitative behaviour could provide a common background for the alterations in executive control and social cognition observed in TS. Two different (but not mutually exclusive) neurocognitive theories have recently suggested that TS could originate from altered perception-action binding and social decision-making dysfunction, respectively. Conclusions: Since the presence of behavioural comorbidities influences individualised treatment approaches, it is likely that a more precise characterisation of TS phenotypes, including cognitive aspects, will result in improved levels of care for patients with tic disorders.

Aim: Correctly diagnosing repetitive behaviors in patients with Tourette syndrome (TS) can be challenging. The differential diagnosis between tics and stereotypies is of particular importance, because of treatment implications. Methods: We assessed the prevalence and clinical characteristics of stereotypies in a large sample of adult patients with TS attending a specialist clinic. Results: Mild stereotypies were reported by 21/148 patients (14.2%). Patients with stereotypies were significantly more likely to have a comorbid diagnosis of Asperger syndrome, attention-deficit and hyperactivity disorder, and obsessive-compulsive disorder, compared with patients without stereotypies. Multiple linear regression analysis revealed that the presence of Asperger syndrome significantly predicted stereotypy severity. Conclusion: Stereotypies are not rare in adults with TS and other neurodevelopmental conditions, especially Asperger syndrome.

Motor and vocal tics have long been recognised as the core features of Tourette syndrome (TS). However, patients’ first-person accounts have consistently reported that these involuntary motor manifestations have specific sensory correlates. These sensory symptoms are often described as feelings of mounting inner tension (“premonitory urges”) and are transiently relieved by tic expression. Multimodal hypersensitivity to external stimuli, perceived as triggers and/or exacerbating factors for specific tic symptoms, is also commonly reported by patients with TS. This article focuses on the rapidly expanding literature on the clinical and neurobiological aspects of the premonitory urge and multimodal hypersensitivity in patients with TS, with particular attention to pathophysiological mechanisms and possible treatment implications. These findings suggest that TS is a neurobehavioural condition characterised by intrinsic perceptual abnormalities involving the insula and sensorimotor areas, in addition to basal ganglia dysfunction. Further research will clarify the role of sensory symptoms in TS, as well as the effects of external sensory input on underlying motor abnormalities.

Nonepileptic attack disorder (NEAD) is a medical condition commonly seen in neuropsychiatry services, often as a differential diagnosis of other neuropsychiatric conditions. Recommendations by the International League Against Epilepsy (ILAE) Nonepileptic Seizures Task Force propose a four-level hierarchical approach to the diagnosis of NEAD, based on history, witnessed event, and electroencephalographic (EEG) investigation. We set out to provide the first description of the diagnostic levels of patients with NEAD at a specialist neuropsychiatry clinic. Comprehensive clinical data from 148 consecutive patients with NEAD attending the specialist Neuropsychiatry Clinic run by a single Consultant in Behavioral Neurology were retrospectively reviewed. Patients with NEAD were primarily referred to neuropsychiatry by Consultant Neurologists (n = 94; 63.5%). The majority of patients were female (n = 108; 73.0%), with a disease duration of 7.9 years (standard deviation: 10.4). Anxiety was the most common comorbidity (n = 43; 26.7%). Categorization of patients according to the ILAE Nonepileptic Seizures Task Force criteria was mainly based on clinical features and EEG findings, as only 7 (4.7%) patients had attacks witnessed by a specialist. The largest diagnostic categories were ‘possible’ (n = 54; 36.5%) and ‘clinically established’ (n = 40; 27.0%), followed by ‘documented’ (n = 12; 8.1%) and ‘probable’ (n = 5; 3.4%). In 125 patients (84.4%), EEGs were performed. Selective serotonin reuptake inhibitors were the most frequently prescribed psychotropic medications (n = 48; 32.4%); 89 patients (60.1%) received behavioral therapy. There were no differences in pharmacological or behavioral management strategies across the patients categorized under different diagnostic levels. Patients with NEAD seen within neuropsychiatry settings are mainly assigned ‘possible’ and ‘clinically established’ levels of diagnostic certainty. Difficulty in capturing typical clinical events witnessed by an experienced clinician while on video-EEG can limit the clinical application of the ‘documented’ diagnostic level. If appropriate, active interventions can be implemented irrespective of diagnostic levels to minimize delays in the neuropsychiatric care pathways.

Aim:To systematically review the available literature on the prevalence and clinical characteristics of headache in patients with Tourette syndrome (TS), a neurodevelopmental condition characterized by the chronic presence of motor and vocal tics.Design:We conducted a systematic literature review of original studies using three clinical databases (Medline, EMBASE and PsycInfo), according to the standards outlined in the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Outcome measures included both tic severity and tic frequency.Results:Our systematic literature review identified six studies on the prevalence and characteristics of headache in patients with TS (data on paediatric patients were available from five studies and data on adult patients from four studies). The proportion of patients with TS who had a diagnosis of headache ranged from 29% to 62%. Migraine was the most commonly investigated headache disorder in this patient population. The findings of the reviewed studies showed that the prevalence of migraine in patients with TS ranges between 13% and 43% and is significantly higher compared to the two control groups ( p < 0.05).Discussion:The prevalence of headache in patients with TS could be higher than in the general population, with the most reliable data being available for migraine. Overall, the literature on the prevalence and characteristics of headache in TS is sparse and highly heterogeneous. Further research on the prevalence and clinical correlates of migraine, tension-type headache and other types of headache in patients with TS across the lifespan is needed to inform clinical practice aimed at improving health-related quality of life in patients with tics.

Misophonia is characterized by a negative reaction to a sound with a specific pattern and meaning to a given individual. In this paper, we review the clinical features of this relatively common yet underinvestigated condition, with focus on co-occurring neurodevelopmental disorders. Currently available data on the putative pathophysiology of the condition can inform our understanding and guide the diagnostic process and treatment approach. Tinnitus retraining therapy and cognitive behavior therapy have been proposed as the most effective treatment strategies for reducing symptoms; however, current treatment algorithms should be validated in large population studies. At the present stage, competing paradigms see misophonia as a physiological state potentially inducible in any subject, an idiopathic condition (which can present with comorbid psychiatric disorders), or a symptomatic manifestation of an underlying psychiatric disorder. Agreement on the use of standardized diagnostic criteria would be an important step forward in terms of both clinical practice and scientific inquiry. Areas for future research include phenomenology, epidemiology, modulating factors, neurophysiological underpinnings, and treatment trials.

Neuropsychiatry services provide specialist input into the assessment and management of behavioral symptoms associated with a range of neurological conditions, including epilepsy. Despite the centrality of epilepsy to neuropsychiatry and the recent expansion of neuropsychiatry service provision, little is known about the clinical characteristics of patients with epilepsy who are routinely seen by a specialist neuropsychiatry service. This retrospective study filled this gap by retrospectively evaluating a naturalistic series of 60 consecutive patients with epilepsy referred to and assessed within a neuropsychiatry setting. Fifty-two patients (86.7%) had active epilepsy and were under the ongoing care of the referring neurologist for seizure management. The majority of patients (N = 42; 70.0%) had a diagnosis of localization-related epilepsy, with temporal lobe epilepsy as the most common epilepsy type (N = 37; 61.7%). Following clinical assessment, 39 patients (65.0%) fulfilled formal diagnostic criteria for at least one psychiatric disorder; nonepileptic attack disorder (N = 37; 61.7%), major depression (N = 23; 38.3%), and generalized anxiety disorder (N = 16; 26.7%) were the most commonly diagnosed comorbidities. The clinical characteristics of patients seen in specialist neuropsychiatry settings are in line with the results from previous studies in neurology clinics in terms of both epilepsy and psychiatric comorbidity. Our findings confirm the need for the development and implementation of structured care pathways for the neuropsychiatric aspects of epilepsy, with focus on comorbid nonepileptic attacks and affective and anxiety symptoms. This is of particular importance in consideration of the impact of behavioral symptoms on patients' health-related quality of life.

Introduction: Tourette syndrome is a neurodevelopmental disorder characterized by multiple motor tics and at least one vocal/phonic tic. Clinical phenotypes show a wide variability, often incorporating behavioral symptoms. The exact pathophysiology of Tourette syndrome is unknown, however genetic vulnerability and alterations in dopaminergic neurotransmission have consistently been reported. Other biochemical pathways, including histaminergic neurotransmission, are likely to be involved but have received relatively little attention until recently. Areas covered: We conducted a systematic literature review focusing on the role of histaminergic neurotransmission and its pharmacological modulation in Tourette syndrome. We identified a number of relevant original studies published over the last five years, mainly focusing on genetic aspects. Expert opinion: There is converging evidence from recent studies supporting the hypothesis that histaminergic neurotransmission may play a role in the pathophysiology of Tourette syndrome. Most studies focused on the role of the histidine decarboxylase gene and the potential usefulness of histidine decarboxylase knockout mice as an experimental model for studying neurochemical function in Tourette syndrome. There have been no large scale studies assessing the use of histaminergic medications in the management of Tourette syndrome. This would be an important area for future research, with direct implications for the clinical management of selected phenotypes.

ObjectiveThe discipline of clinical neuropsychiatry currently provides specialised services for a number of conditions that cross the traditional boundaries of neurology and psychiatry, including non-epileptic attack disorder. Neurophysiological investigations have an important role within neuropsychiatry services, with video-electroencephalography (EEG) telemetry being the gold standard investigation for the differential diagnosis between epileptic seizures and non-epileptic attacks. This article reviews existing evidence on best practices for neurophysiology investigations, with focus on safety measures for video-EEG telemetry.MethodsWe conducted a systematic literature review using the PubMed database in order to identify the scientific literature on the best practices when using neurophysiological investigations in patients with suspected epileptic seizures or non-epileptic attacks.ResultsSpecific measures need to be implemented for video-EEG telemetry to be safely and effectively carried out by neuropsychiatry services. A confirmed diagnosis of non-epileptic attack disorder following video-EEG telemetry carried out within neuropsychiatry units has the inherent advantage of allowing diagnosis communication and implementation of treatment strategies in a timely fashion, potentially improving clinical outcomes and cost-effectiveness significantly.ConclusionThe identified recommendations set the stage for the development of standardised guidelines to enable neuropsychiatry services to implement streamlined and evidence-based care pathways.

Joanna H Cox,1 Stefano Seri,2,3 Andrea E Cavanna,2,4,5 1Heart of England NHS Foundation Trust, 2School of Life and Health Sciences, Aston Brain Centre, Aston University, 3Children’s Epilepsy Surgery Programme, The Birmingham Children’s Hospital NHS Foundation Trust, 4Department of Neuropsychiatry, Birmingham and Solihull Mental Health NHS Foundation Trust, Birmingham, 5Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology and UCL, London, UK Abstract: Tourette syndrome is a childhood-onset chronic tic disorder characterized by multiple motor and vocal tics and often accompanied by specific behavioral symptoms ranging from obsessionality to impulsivity. A considerable proportion of patients report significant impairment in health-related quality of life caused by the severity of their tics and behavioral symptoms and require medical intervention. The most commonly used medications are antidopaminergic agents, which have been consistently shown to be effective for tic control, but are also associated with poor tolerability because of their adverse effects. The newer antipsychotic medication aripiprazole is characterized by a unique mechanism of action (D2 partial agonism), and over the last decade has increasingly been used for the treatment of tics. We conducted a systematic literature review to assess the available evidence on the efficacy and safety of aripiprazole in pediatric patients with Tourette syndrome and other chronic tic disorders (age range: 4–18 years). Our search identified two randomized controlled trials (involving 60 and 61 participants) and ten open-label studies (involving between six and 81 participants). The majority of these studies used two validated clinician-rated instruments (Yale Global Tic Severity Scale and Clinical Global Impression scale) as primary outcome measures. The combined results from randomized controlled trials and open-label studies showed that aripiprazole is an effective, safe, and well-tolerated medication for the treatment of tics. Aripiprazole-related adverse effects (nausea, sedation, and weight gain) were less frequent compared to other antidopaminergic medications used for tic management and, when present, were mostly transient and mild. The reviewed studies were conducted on small samples and had relatively short follow-up periods, thus highlighting a need for further trials to assess the long-term use of aripiprazole in pediatric patients with Tourette syndrome and other chronic tic disorders with measurement of its efficacy using both clinician-rated and self-report scales. Keywords: Tourette syndrome, tics, aripiprazole, efficacy, tolerability

Tourette syndrome is a neurodevelopmental disorder characterized by multiple tics and commonly associated with behavioral problems, especially obsessive-compulsive disorder and attention-deficit hyperactivity disorder (ADHD). The presence of specific personality traits has been documented in adult clinical populations with Tourette syndrome but has been underresearched in younger patients. We assessed the personality profiles of 17 male adolescents with Tourette syndrome and 51 age- and gender-matched healthy controls using the Minnesota Multiphasic Personality Inventory-Adolescent version, along with a standardized psychometric battery. All participants scored within the normal range across all Minnesota Multiphasic Personality Inventory-Adolescent version scales. Patients with Tourette syndrome scored significantly higher than healthy controls on the Obsessiveness Content Scale only (P = .046). Our findings indicate that younger male patients with Tourette syndrome do not report abnormal personality traits and have similar personality profiles to healthy peers, with the exception of obsessionality traits, which are likely to be related to the presence of comorbid obsessive compulsive symptoms rather than tics.

How different types of tic disorders are diagnosed and the appropriate therapeutic options for their management, with a focus on Tourette syndrome.

Bipolar disorder is a severe affective disorder which can present in adolescence, or sometimes earlier, and often requires a pharmacotherapeutic approach. The phenomenology of bipolar disorder in children and adolescents appears to differ from that of adult patients, prompting the need for specific pharmacotherapy guidelines for long-term management in this patient population. Current treatment guidelines were mainly developed based on evidence from studies in adult patients, highlighting the requirement for further research into the pharmacotherapy of children and adolescents with bipolar disorder. This review compares and critically analyzes the available guidelines, discussing the recommended medication classes, their mechanisms of action, side effect profiles and evidence base.

Although the majority of people with epilepsy have a good prognosis and their seizures can be well controlled with pharmacotherapy, up to one-third of patients can develop drug-resistant epilepsy, especially those patients with partial seizures. This unmet need has driven considerable efforts over the last few decades aimed at developing and testing newer antiepileptic agents to improve seizure control. One of the most promising antiepileptic drugs of the new generation is zonisamide, a benzisoxazole derivative chemically unrelated to other anticonvulsant agents. In this article, the authors present the results of a systematic literature review summarizing the current evidence on the efficacy and tolerability of zonisamide for the treatment of partial seizures. Of particular interest within this updated review are the recent data on the use of zonisamide as monotherapy, as they might open new therapeutic avenues.

Objective: Patients with Tourette syndrome (TS) often report characteristic sensory experiences, also called premonitory urges (PUs), which precede tic expression and have high diagnostic relevance. This study investigated the usefulness of a scale developed and validated in children and adolescents-the Premonitory Urge for Tics Scale (PUTS, Woods et al., 2005 [13])-for the assessment of PUs in adult patients with TS. Method: Standard statistical methods were applied to test the psychometric properties of the PUTS in 102 adult TS outpatients recruited from two specialist clinics in the United Kingdom. Results: The PUTS showed good acceptability and endorsement rates, with evenly distributed scores and low floor and ceiling effects. Item-total correlations were moderate to strong; PUTS total scores were significantly correlated with quantitative measures of TS severity. The PUTS showed excellent internal consistency reliability (Cronbach's alpha=0.85) and Spearman's correlations demonstrated satisfactory convergent and discriminant validity. Conclusions: Although originally devised to assess urges to tic in young patients with TS, the PUTS demonstrated good psychometric properties in a large sample of adults recruited at specialist TS clinics. This instrument is therefore recommended for use across the life span as a valid and reliable self-report measure of sensory experiences accompanying tic expression. © 2013 The Japanese Society of Child Neurology.

Tourette’s syndrome is a tic disorder that is often associated with behavioural symptoms. Diagnostic criteria are based on the presence of both motor and vocal tics; because of its varied presentations, the syndrome has the potential to be misdiagnosed. Prevalence is higher than commonly assumed; coprolalia is relatively rare (10-30%) and not required for diagnosis. The syndrome can cause serious distress and compromise health related quality of life. The main management strategies include psychoeducation, behavioural techniques, and drugs. Service provision is patchy even in developed countries and patients of all ages often “fall through the net” between neurology and psychiatry.

Over the last few years, zonisamide has been proposed as a potentially useful medication for patients with focal seizures, with or without secondary generalization. Since psychiatric adverse effects, including mania, psychosis, and suicidal ideation, have been associated with its use, it was suggested that the presence of antecedent psychiatric disorders is an important factor associated with the discontinuation of zonisamide therapy in patients with epilepsy. We, therefore, set out to assess the tolerability profile of zonisamide in a retrospective chart review of 23 patients with epilepsy and comorbid mental disorders, recruited from two specialist pediatric (n=11) and adult (n=12) neuropsychiatry clinics. All patients had a clinical diagnosis of treatment-refractory epilepsy after extensive neurophysiological and neuroimaging investigations. The vast majority of patients (n=22/23, 95.7%) had tried previous antiepileptic medications, and most adult patients (n=9/11, 81.8%) were on concomitant medication for epilepsy. In the majority of cases, the psychiatric adverse effects of zonisamide were not severe. Four patients (17.4%) discontinued zonisamide because of lack of efficacy, whereas only one patient (4.3%) discontinued it because of the severity of psychiatric adverse effects (major depressive disorder). The low discontinuation rate of zonisamide in a selected population of patients with epilepsy and neuropsychiatric comorbidity suggests that this medication is safe and reasonably well-tolerated for use in patients with treatment-refractory epilepsy. Given the limitations of the present study, including the relatively small sample size, further research is warranted to confirm this finding. © 2013 Elsevier Inc.

Objective Zonisamide is a newer antiepileptic drug indicated as adjunctive therapy in the treatment of adult patients with partial-onset seizures, with or without secondary generalization. Following isolated reports of zonisamide-induced mania, other behavioural adverse effects, including psychosis and suicidal ideation have been associated with its use, and it was suggested that past psychiatric history is among the factors associated with discontinuation of zonisamide therapy in patients with epilepsy. We therefore set out to assess the tolerability profile of zonisamide in this particular group of patients with epilepsy, who are at risk of developing adverse reactions to zonisamide. Method This study investigated the prevalence and characteristics of adverse effects resulting from the use of zonisamide in a retrospective chart review of patients with epilepsy and co-morbid cognitive and/or behavioural problems, recruited from the specialist neuropsychiatry clinic at the National Centre for Mental Health, BSMHFT and University of Birmingham. Results We identified 12 eligible patients (3 males, mean age 36 years, range 16-59 years). All patients had a clinical diagnosis of treatment-refractory epilepsy (9=temporal lobe epilepsy), supported by neurophysiological and neuroimaging findings, and had concomitant and/or previous antiepileptic medications (11=levetiracetam, 8=carbamazepine, 6=lamotrigine, 6=valproate). In our neuropsychiatric sample, 6 patients had a previous diagnosis of depression, 2 anxiety disorders, 2 learning disability, 2 neurodevelopmental disorders (Tourette syndrome and autism) and 1 psychosis. Co-morbid non-epileptic attack disorder was documented in 4 patients. In the majority of cases, zonisamide (mean maintenance dose=212.5mg daily, range 50-500 mg daily) was well tolerated and behavioural adverse effects were not severe. Three patients (25.0%) discontinued zonisamide over the observation period (mean duration 15 months, range 1-48 months). The main reasons for discontinuation were lack of efficacy on seizure control (two cases) and emerging depression as an adverse effect (one case). Conclusion This preliminary observation of relatively low discontinuation rate of zonisamide in a selected population of patients with epilepsy and neuropsychiatric comorbidity prompts further research to establish whether this medication is a safe treatment option for vulnerable patients with treatment-refractory epilepsy.

The neural bases of altered consciousness in patients with epilepsy during seizures and at rest have raised significant interest in the last decade. This exponential growth has been supported by the parallel development of techniques and methods to investigate brain function noninvasively with unprecedented spatial and temporal resolution. In this article, we review the contribution of magnetoencephalography to deconvolve the bioelectrical changes associated with impaired consciousness during seizures. We use data collected from a patient with refractory absence seizures to discuss how spike–wave discharges are associated with perturbations in optimal connectivity within and between brain regions and discuss indirect evidence to suggest that this phenomenon might explain the cognitive deficits experienced during prolonged 3/s spike–wave discharges.This article is part of a Special Issue entitled Epilepsy and Consciousness.