Severe Behavioral Disturbances Research Articles

Dysphagia in primary progressive aphasia: Clinical predictors and neuroanatomical basis.

Dysphagia is an important feature of neurodegenerative diseases and potentially life-threatening in primary progressive aphasia (PPA) but remains poorly characterized in these syndromes. We hypothesized that dysphagia would be more prevalent in nonfluent/agrammatic variant (nfv)PPA than other PPA syndromes, predicted by accompanying motor features, and associated with atrophy affecting regions implicated in swallowing control. In a retrospective case-control study at our tertiary referral centre, we recruited 56 patients with PPA (21 nfvPPA, 22 semantic variant [sv]PPA, 13 logopenic variant [lv]PPA). Using a pro forma based on caregiver surveys and clinical records, we documented dysphagia (present/absent) and associated, potentially predictive clinical, cognitive, and behavioural features. These were used to train a machine learning model. Patients' brain magnetic resonance imaging scans were assessed using voxel-based morphometry and region-of-interest analyses comparing differential atrophy profiles associated with dysphagia presence/absence. Dysphagia was significantly more prevalent in nfvPPA (43% vs. 5% svPPA and no lvPPA). The machine learning model revealed a hierarchy of features predicting dysphagia in the nfvPPA group, with excellent classification accuracy (90.5%, 95% confidence interval = 77.9-100); the strongest predictor was orofacial apraxia, followed by older age, parkinsonism, more severe behavioural disturbance, and more severe cognitive impairment. Significant grey matter atrophy correlates of dysphagia in nfvPPA were identified in left middle frontal, right superior frontal, and right supramarginal gyri and right caudate. Dysphagia is a common feature of nfvPPA, linked to underlying corticosubcortical network dysfunction. Clinicians should anticipate this symptom particularly in the context of other motor features and more severe disease.

Parenteral medication for the management of acute severe behavioural disturbance (ASBD) in the emergency department

Parenteral medication for the management of acute severe behavioural disturbance (ASBD) in the emergency department

Rivastigmine for the management of anticholinergic delirium

Introduction Anticholinergic agents are commonly taken in overdose, often causing delirium. The spectrum of anticholinergic delirium ranges from mild agitation to severe behavioural disturbance. Physostigmine is an effective treatment for anticholinergic delirium, but its availability is limited. As rivastigmine is readily available, it has been used to manage anticholinergic delirium; however, there is limited research investigating its use. Method This was a retrospective review of patients with anticholinergic delirium treated in two toxicology units with rivastigmine (oral capsule or transdermal patch) from January 2019 to June 2023. The primary outcome was the use of further parenteral treatment (sedation or physostigmine) for delirium post rivastigmine administration. Results Fifty patients were administered rivastigmine for the management of anticholinergic delirium. The median age was 36 years (interquartile range: 25–49 years) and 27 (54 per cent) were females. Features consistent with anticholinergic toxicity included tachycardia in 44 (88 per cent) and urinary retention requiring catheterisation in 40 (80 per cent). Forty-three patients (86 per cent) were treated with physostigmine before rivastigmine administration. Twenty-two were managed with transdermal rivastigmine (most commonly 9.5 mg/24 hour patch), and 28 with oral rivastigmine 6 mg. Further parenteral sedation and/or physostigmine treatment were required more often in patients given transdermal than oral rivastigmine [16/22 (73 per cent) versus 9/28 (32 per cent), P = 0.010)]. No patients had bradycardia or gastrointestinal symptoms following rivastigmine administration. One patient with a history of epilepsy had a seizure, 1.5 hours post physostigmine administration and 7 hours post transdermal rivastigmine. Discussion Rivastigmine has been increasingly used for the management of patients with anticholinergic delirium, due to the lack of availability of physostigmine. In this case series, rivastigmine transdermal patch appeared to be less effective than oral rivastigmine capsules, likely due to its slow onset of action and/or insufficient dose. Conclusion Rivastigmine can be used to treat anticholinergic delirium. In our case series oral rivastigmine appeared more effective than transdermal rivastigmine.

Perampanel-associated Psychiatric Problems

Perampanel is a noncompetitive α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid glutamate receptor antagonist used in the management of seizure disorder. Management of uncontrolled seizures is a challenging task. Perampanel is a recent antiepileptic which comes with a warning of causing behavioral and psychological adverse effects. Here, the authors report an interesting case where the perampanel was started to control refractory seizures and the person developed severe behavioral disturbances. The condition warranted a psychiatry referral, and the problems subsided after stopping perampanel. Clinicians need to be vigilant about perampanel-associated behavioral and psychiatric problems which can become a serious clinical concern.

Acute Severe Behavioral Disturbance Requiring Parenteral Sedation in Pediatric Mental Health Presentations to Emergency Medical Services: A Retrospective Chart Review

To describe the epidemiological factors of mental health presentations in young people to emergency medical services (EMS) and define those experiencing acute severe behavioral disturbance by reviewing parenteral sedation use. We performed a retrospective review of records of EMS attendance for young people (aged <18 years) with mental health presentations between July 2018 and June 2019 to a statewide EMS system in Australia of a population of 6.5 million persons. In addition, epidemiological data and information about parenteral sedation for acute severe behavioral disturbance and any adverse events were extracted from the records and analyzed. A total of 7,816 patients had mental health presentations with a median age of 15 years (IQR 14-17). The majority (60%) were female. These presentations accounted for 14% of all pediatric presentations to EMS. Out of them, 612 (8%) received parenteral sedation for acute severe behavioral disturbance. A number of factors were associated with increased odds of parenteral sedative medication being used, including autism spectrum disorder (odds ratio [OR] 3.3; confidence interval [CI], 2.7 to 3.9), posttraumatic stress disorder (OR 2.8; CI, 2.2 to 3.5) and intellectual disability (OR 3.6; CI, 2.6 to 4.8). The majority (460, 75%) of young people received midazolam as their first-line medication, with the remaining patients being provided ketamine (152, 25%). No serious adverse events were noted. Mental health conditions were a common presentation to EMS. A history of autism spectrum disorder, posttraumatic stress disorder, or an intellectual disability increased the odds of receiving parenteral sedation for acute severe behavioral disturbance. Sedation appears generally safe in the out-of-hospital setting.

Pharmacological emergency management of agitation in children and young people: protocol for a randomised controlled trial of oral medication (PEAChY-O)

IntroductionAcute severe behavioural disturbance (ASBD) is a condition seen with increasing frequency in emergency departments (EDs) in adults and young people. Despite the increasing number of presentations and significant associated...

Pharmacological Emergency management of Agitation in Children and Young people: protocol for a randomised controlled trial of intraMuscular medication (PEAChY-M)

IntroductionAcute severe behavioural disturbance (ASBD) is a condition seen with increasing frequency in emergency departments (EDs) in adults and young people. Despite the increasing number of presentations and significant associated...

CDKL5-associated developmental and epileptic encephalopathy: A long-term, longitudinal electroclinical study of 22 cases

ObjectiveThe study was conducted to analyze the possible diagnostic value of the electroclinical semiology of the epileptic seizures. MethodsWe evaluated the medical records of 17 females and 5 males with CDKL5 deficiency disorder (CDD) considering the long-term evolution, including the polygraphic video-EEG recordings. ResultsWe recognized three disease phases. We found that the seizure semiology was already recognizable in the first phase of the syndrome. In the short-term evolution, all patients had focal motor and 12/21 hypermotor seizures. Both epileptic spasms and myoclonic seizures were already present in more than half of the cases in the first 2 months after onset. In the second phase, the intermediate period, the polymorphic pattern was maintained, but in eight patients the electroclinical pattern of epileptic encephalopathy with hypsarrhythmia appeared. In the long-term period, the seizure polymorphism continued but myoclonic and epileptic spasms diminished. Tonic seizures appeared in the last 2 phases. Progressively, with the aggravation of seizures and paroxysmal EEG abnormalities impairment of the neurocognitive status was observed. Severe behavioral disturbances were seen in eight and autistic-like features in 14. ConclusionCDD is a true developmental and epileptic encephalopathy with a specific etiology characterized by the early appearance of epileptic seizures that quickly become polymorphic and drug resistant in infants that are most often female and already have neurological impairment. Polygraphic video-EEG recordings are important to recognize ictal events of the association of hypermotor seizures, epileptic spasms in clusters, and massive myoclonic jerks, already present at onset.

Management of paediatric acute severe behavioural disturbance in emergency departments across Australia: A PREDICT survey of senior medical staff.

Acute severe behavioural disturbance (ASBD) is a condition seen with increasing frequency in EDs. It poses a significant risk to the patient and those around them. Little is known about the epidemiology or most effective management in the paediatric population. The aim of the present study is to clarify the practice of senior emergency doctors in Australia when managing paediatric ASBD. The present study was a voluntary electronic questionnaire distributed to and undertaken by senior medical staff in EDs affiliated with the Paediatric Research in Emergency Departments International Collaborative (PREDICT) network. Respondents reported on exposure to and confidence in managing paediatric ASBD and their current practices. A total of 227 (33%) clinicians completed the survey between February and May 2020. Most clinicians were caring for at least two young people with ASBD each week (72%), felt confident regarding the majority of components of management and referred to local clinical practice guidelines (69%). Agitation/sedation rating scales were seldom used (19%). There was a significant variation in self-reported management practices. The choice of whether to use medication at all, the medication chosen and route of administration all varied greatly. Respondents were more willing to provide parenteral medication to young people reported as having recreational drug intoxication (84%) than those with neurodevelopment disorders (65%) when the same degree of agitation was reported. Within Australia, there is considerable variation in paediatric ASBD practice, in particular regarding medication provision. Further prospective research is required to inform best clinical practice.

Audit Cycle on Medical Reviews of Seclusion in Medium and Low Secure Learning Disability Units

AimsSeclusion is defined as “the supervised confinement and isolation of a patient, away from other patients, in an area from which the patient is prevented from leaving, where it is of immediate necessity for the purpose of the containment of severe behavioural disturbance which is likely to cause harm to others”. Patients in seclusion require reviews at the frequency set out in the Mersey Care NHS Foundation Trust policy, “The use of seclusion and long-term segregation” (SD28). This is based on the requirements set out in the Chapter 26 of the Mental Health Act 1983 Code of Practice (2015).This audit will look at whether medical reviews for secluded patients in the secure learning disability wards meet with the expectations set out in the Trust Policy. In doing so, the audit will establish whether medical reviews of seclusion meet and uphold the guiding principles of the Mental Health Act Code of Practice as highlighted in Chapter 26.110.MethodsRetrospective audit that collected data from inpatients on secure learning disability wards in Mersey Care. After reviewing data, we actioned plans which involved educating colleagues working in secure services. This was re audited after three months. One month of seclusion reviews was audited in each cycle, which equated to 39 reviews in the first cycle and 100 reviews in the second.ResultsThe re-audit data showed an improvement in most parameters.Re-audit showed that 66% (34%) of the seclusion reviews had an initial medical review within the first hour. The on call consultant was informed in 60% (50%) of the situations and 4 hourly reviews took place in 66% (50%) of scenarios. All MDT reviews took place within 24 hours, Responsible Clinician was present in 100% (67%) of reviews.34% (33%) of MDT reviews had only 2 MDT members.There was 100% compliance with reviewing physical health in both audits. 100% (90%) of the reviews commented on mental health, 72% (20%) commented on medications used, 51% (39%) of reviews commented on level of observations and 89% (48%) included risk assessment. 95% (92%) of reviews assessed need for continuing seclusion. 84% (59%) of reviews commented on reducing restriction in seclusion.ConclusionThis audit cycle has focused on the quality of medical reviews and not just the frequency. The improvement in practice will strengthen the safeguard provided by these reviews.

Family, personal, parental correlates and behavior disturbances in school-aged boys with attention-deficit/hyperactivity disorder (ADHD): a cross-sectional study

BackgroundTo evaluate the relationship among family, personal, parental correlates, and behavioral disturbances in boys with attention-deficit/hyperactivity disorder (ADHD).MethodsWe performed a hospital-based cross-sectional study. School-aged boys who first visited the hospital between 2000 and 2011 with ADHD were identified. Through medical records review, demographic information, family characteristics, personal characteristics, parental characteristics, and the Child Behavior Checklist (CBCL) collected during the first outpatient visit were retrieved. A T-score higher than 63 in the internalizing or externalizing subscale of the CBCL indicated severe behavioral disturbances in each domain. Multivariable logistic regression was used to evaluate the relationship between the correlates and behavioral disturbances. Eligible patients were further classified into groups without behavioral disturbance, with either only severe internalizing or only severe externalizing behaviors, or with both behaviors. Multivariable ordinal logistic regression was used to investigate the association between the correlates and the number of types of behavioral disturbances.ResultsA total of 1855 boys with ADHD were included. In the multivariable logistic regression, family factors, including being first-born, living in a family not with both parents, and family history of mental disorder, were associated with severe internalizing and externalizing behaviors. Personal factors, including prenatal complications, perinatal complications, and medical and psychiatric comorbidities, were associated with severe internalizing behaviors, but only prenatal complications and medical comorbidities were associated with severe externalizing behaviors. Parental factors were only associated with severe externalizing behaviors. A higher paternal education level had a protective effect, but younger motherhood increased the risk. In ordinal logistic regression, these factors were also associated with more types of behavioral disturbances.ConclusionsMultiple factors are related to behavioral disturbances in ADHD. Our study reported the association among family, personal, parental factors, severe internalizing behavior, severe externalizing behavior, and number of behavioral disturbances in boys with ADHD. However, the impacts differed as the behavior phenotypes varied. Further research is needed to better understand the heterogeneity of ADHD behavior.

Expanding the genotype and phenotype spectrum of SYT1-associated neurodevelopmental disorder

PurposeSynaptotagmin-1 (SYT1) is a critical mediator of neurotransmitter release in the central nervous system. Previously reported missense SYT1 variants in the C2B domain are associated with severe intellectual disability, movement disorders, behavioral disturbances, and electroencephalogram abnormalities. In this study, we expand the genotypes and phenotypes and identify discriminating features of this disorder. MethodsWe describe 22 individuals with 15 de novo missense SYT1 variants. The evidence for pathogenicity is discussed, including the American College of Medical Genetics and Genomics/Association for Molecular Pathology criteria, known structure–function relationships, and molecular dynamics simulations. Quantitative behavioral data for 14 cases were compared with other monogenic neurodevelopmental disorders. ResultsFour variants were located in the C2A domain with the remainder in the C2B domain. We classified 6 variants as pathogenic, 4 as likely pathogenic, and 5 as variants of uncertain significance. Prevalent clinical phenotypes included delayed developmental milestones, abnormal eye physiology, movement disorders, and sleep disturbances. Discriminating behavioral characteristics were severity of motor and communication impairment, presence of motor stereotypies, and mood instability. ConclusionNeurodevelopmental disorder–associated SYT1 variants extend beyond previously reported regions, and the phenotypic spectrum encompasses a broader range of severities than initially reported. This study guides the diagnosis and molecular understanding of this rare neurodevelopmental disorder and highlights a key role for SYT1 function in emotional regulation, motor control, and emergent cognitive function.

Management of Psychiatric Disorders during the Perinatal Period.

Management of Psychiatric Disorders during the Perinatal Period.

Serious Suicide Attempt Inaugurating an Acute Psychosis: A Case Report

Acute Psychosis is a severe affection that predominantly affects young people. This disorder may be accompanied by severe behavioral disturbances, which may include self-harming acts. Suicidal behavior may inaugurate acute psychosis, and thus, be unexpected and misunderstood. The prevalence of Suicidal behavior in psychotic disorders is high, but little is known about suicide or severe suicide attempt as the first clinical signs of the disorder. We present here the case of a young man, without psychiatric history, who has committed a severe suicide attempt, preceded by delusional symptoms, requiring emergency evacuation and cardiovascular surgery before the psychiatric intervention.

Holistic approach to undifferentiated acute severe behavioural disturbance in older emergency department patients.

Holistic approach to undifferentiated acute severe behavioural disturbance in older emergency department patients.

Methylphenidate for attention-deficit/hyperactivity disorder in patients with Smith\u2013Magenis syndrome: protocol for a series of N-of-1 trials

BackgroundSmith–Magenis syndrome (SMS) is a rare genetic neurodevelopmental disorder characterized by intellectual disability and severe behavioural and sleep disturbances. Often, patients with SMS are diagnosed with attention-deficit/hyperactivity disorder (ADHD). However, the effectiveness of methylphenidate (MPH), the first-line pharmacological treatment for ADHD, in patients with SMS is unclear. Our objective is to examine the effectiveness of MPH for ADHD symptoms in individuals with SMS, proposing an alternative trial design as traditional randomized controlled trials are complex in these rare and heterogeneous patient populations.Methods and analysisWe will initiate an N-of-1 series of double-blind randomized and placebo-controlled multiple crossover trials in six patients aged ≥ 6 years with a genetically confirmed SMS diagnosis and a multidisciplinary established ADHD diagnosis, according to a power analysis based on a summary measures analysis of the treatment effect. Each N-of-1 trial consists of a baseline period, dose titration phase, three cycles each including randomized intervention, placebo and washout periods, and follow-up. The intervention includes twice daily MPH (doses based on age and body weight). The primary outcome measure will be the subscale hyperactivity/inattention of the Strengths and Difficulties Questionnaire (SDQ), rated daily. Secondary outcome measures are the shortened version of the Emotion Dysregulation Inventory (EDI) reactivity index, Goal Attainment Scaling (GAS), and the personal questionnaire (PQ). Statistical analysis will include a mixed model analysis. All subjects will receive an assessment of their individual treatment effect and data will be aggregated to investigate the effectiveness of MPH for ADHD in SMS at a population level.ConclusionsThis study will provide information on the effectiveness of MPH for ADHD in SMS, incorporating personalized outcome measures. This protocol presents the first properly powered N-of-1 study in a rare genetic neurodevelopmental disorder, providing a much-needed bridge between science and practice to optimize evidence-based and personalized care.Trial registration This study is registered in the Netherlands Trial Register (NTR9125).

Emergency mental health presentations in children with autism spectrum disorder and attention deficit hyperactivity disorder.

To characterise the key features and management of young people presenting to the emergency department (ED) with a mental health (MH) complaint and a known diagnosis of autism spectrum disorder (ASD) or attention deficit hyperactivity disorder (ADHD). Retrospective review of all ED MH presentations in children aged 7-17 years, presenting over a 12-month period from the 1st of January 2018 to the 31st of December 2018, to the Royal Children's Hospital in Melbourne, Australia. Univariate analyses were carried out to examine the relationship between an underlying diagnosis of ASD and/or ADHD and a number of key presentation variables. Relative risks (RRs) and 95% confidence intervals (CIs) were calculated for ED management outcomes. There were 374 presentations in this cohort, representing 28% of the total MH presentations in 2018. The most common reason for presentation was acute severe behavioural disturbance. Young people with ASD and ADHD were at increased risk of having an acute crisis team response activated (ASD RR 2.3, CI 1.6-3.3, ADHD RR 2.2, CI 1.2-4.1). Compared to those without either diagnosis, young people with ASD were more likely to be physically restrained (RR 2.8, CI 1.7-4.6), managed in seclusion (RR 3.3, CI 1.7-6.4) and to receive medication to assist with behavioural de-escalation (RR 2.8, CI 1.6-4.9). Children with ASD and/or ADHD represent one-quarter of all children presenting to the ED with MH complaints. They experience high rates of acute severe behavioural disturbance. Future research is needed to co-design, implement and evaluate better approaches for their management.

Functional brain imaging using 18F-fluorodeoxyglucose positron emission tomography/computerized tomography in 138 patients with Kleine-Levin syndrome: an early marker?

Kleine–Levin syndrome is a rare disorder characterized by relapsing-remitting episodes of severe hypersomnia, cognitive impairment, apathy, derealization and behavioural disturbances. Between episodes, most patients experience normal sleep, mood and behaviour, but they may have some residual abnormalities in brain functional imaging; however, the frequency, localization and significance of abnormal imaging are unknown, as brain functional imaging have been scarce and heterogenous [including scintigraphy 18F-fluorodeoxyglucose positron emission tomography/computerized tomography (FDG-PET/CT) and functional MRI during resting state and cognitive effort] and based on case reports or on group analysis in small groups. Using visual individual analysis of 18F-fluorodeoxyglucose positron emission tomography/computerized tomography at the time of Kleine–Levin syndrome diagnosis, we examined the frequency, localization and clinical determinants of hypo- and hypermetabolism in a cross-sectional study. Among 179 patients with Kleine–Levin syndrome who underwent 18F-fluorodeoxyglucose positron emission tomography/computerized tomography, the visual analysis was restricted to the 138 untreated patients studied during asymptomatic periods. As many as 70% of patients had hypometabolism, mostly affecting the posterior associative cortex and the hippocampus. Hypometabolism was associated with younger age, recent (<3 years) disease course and a higher number of episodes during the preceding year. The hypometabolism was more extensive (from the left temporo-occipital junction to the entire homolateral and then the bilateral posterior associative cortex) at the beginning of the disorder. In contrast, there was hypermetabolism in the prefrontal dorsolateral cortex in half of the patients (almost all having concomitant hypometabolism in the posterior areas), which was also associated with younger age and shorter disease course. The cognitive performances (including episodic memory) were similar in patients with versus without hippocampus hypometabolism. In conclusion, hypometabolism is frequently observed upon individual visual analysis of 18F-fluorodeoxyglucose positron emission tomography/computerized tomography during asymptomatic Kleine–Levin syndrome periods; it is mostly affecting the posterior associative cortex and the hippocampus and is mostly in young patients with recent-onset disease. Hypometabolism provides a trait marker during the first years of Kleine–Levin syndrome, which could help clinicians during the diagnosis process.

Toothache

IntroductionMental retardation (RM) is defined as by a deficient intellectual capacity as well as by alterations of the adaptive capacity that are externalized in two or more functional areas (Personal autonomy, Communication, Orientation in the environment, Work and Free time).ObjectivesPresent a patient with a severe behavioural disturbance with an associated intellectual deficit, who remained hospitalized for 2 months and after observing an oral alteration her symptoms improved.MethodsA descriptive study of a clinical caseResults54-year-old woman, single. You have a moderate intellectual disability. In January 2019, she began mental health consultations with a diagnosis of adjustment disorder, on treatment with aripiprazole 5 mg/day, mirtazapine 15 mg/day, lorazepam 0.5 mg/day and dipotassium clorazepate 10 mg/day. Went to the emergency room with mutism, hyporesponsiveness and refuse to intake, having lost 25 kg in 6 months. Abdominal and thoracic CT and upper gastrointestinal endoscopy without significant findings. Consultation with otorhinolaryngology, dermatology, traumatology without significant findings. Odontostomatology consultation: Deep cavities are observed, so it is necessary to carry out extractions of the pieces in poor condition. After this intervention, the patient returns to accept oral intake.ConclusionsPeople with intellectual disabilities have a wide range of medical problems that in many cases are directly associated with the underlying disease or syndrome and, in others, with poor physical health due to problems in basic self-care skills or the ability to express verbally. Usually, the first manifestation of pain is an alteration in behaviour, which must be taken into account when making a differential diagnosis.DisclosureNo significant relationships.

Frontotemporal dementia subtypes based on behavioral inhibition deficits.

IntroductionWe aimed to investigate phenotypic heterogeneity in the behavioral variant of frontotemporal dementia (bvFTD) through assessment of inhibition deficits.MethodsWe assessed occurrences of 16 behavioral inhibition deficits from video recordings of 15 bvFTD patients (early stage) and 15 healthy controls (HC) in an ecological setting. We extracted dimensions of inhibition deficit and analyzed their correlations with cognitive and clinical measures. Using these dimensions, we isolated patient clusters whose atrophy patterns were explored.ResultsAfter identifying two patterns of inhibition deficit (compulsive automatic behaviors and socially unconventional behaviors), we isolated three behavioral clusters with distinct atrophy patterns. BvFTD‐G0 (N = 3), an outlier group, showed severe behavioral disturbances and more severe ventromedial prefrontal cortex/orbitofrontal cortex atrophy. Compared to bvFTD‐G1 (N = 6), bvFTD‐G2 (N = 6) presented higher anxiety and depression along with less diffuse atrophy especially in midline regions.DiscussionIdentifying clinico‐anatomical profiles through behavior observation could help to stratify bvFTD patients for adapted treatments.