Adapting prescribing criteria for amyloid-targeted antibodies for adults with Down syndrome.

Prior authorization criteria for Federal Drug Administration (FDA) approved immunotherapeutics, among the class of anti-amyloid monoclonal antibodies (mAbs), established by state drug formulary committees, are tailored for adults with late-onset Alzheimer's disease. This overlooks adults with Down syndrome (DS), who often experience dementia at a younger age and with different diagnostic assessment outcomes. This exclusion may deny DS adults access to potential disease-modifying treatments. To address this issue, an international expert panel convened to establish adaptations of prescribing criteria suitable for DS patients and parameters for access to Centers for Medicare & Medicaid Services (CMS) registries. The panel proposed mitigating disparities by modifying CMS and payer criteria to account for younger onset age, using alternative language and assessment instruments validated for cognitive decline in the DS population. The panel also recommended enhancing prescribing clinicians' diagnostic capabilities for DS and initiated awareness-raising activities within healthcare organizations. These efforts facilitated discussions with federal officials, aimed at achieving equity in access to anti-amyloid immunotherapeutics, with implications for national authorities worldwide evaluating these and other new disease-modifying therapeutics for Alzheimer's disease.

Consensus statement of the international summit on intellectual disability and Dementia related to post-diagnostic support.

OBJECTIVES: Post diagnostic support (PDS) has varied definitions within mainstream dementia services and different health and social care organizations, encompassing a range of supports that are offered to adults once diagnosed with dementia until death. METHOD: An international summit on intellectual disability and dementia held in Glasgow, Scotland in 2016 identified how PDS applies to adults with an intellectual disability and dementia. The Summit proposed a model that encompassed seven focal areas: post-diagnostic counseling; psychological and medical surveillance; periodic reviews and adjustments to the dementia care plan; early identification of behaviour and psychological symptoms; reviews of care practices and supports for advanced dementia and end of life; supports to carers/ support staff; and evaluation of quality of life. It also explored current practices in providing PDS in intellectual disability services. RESULTS: The Summit concluded that although there is limited research evidence for pharmacological or non-pharmacological interventions for people with intellectual disability and dementia, viable resources and guidelines describe practical approaches drawn from clinical practice. Post diagnostic support is essential, and the model components in place for the general population, and proposed here for use within the intellectual disability field, need to be individualized and adapted to the person's needs as dementia progresses. CONCLUSIONS: Recommendations for future research include examining the prevalence and nature of behavioral and psychological symptoms (BPSD) in adults with an intellectual disability who develop dementia, the effectiveness of different non-pharmacological interventions, the interaction between pharmacological and non-pharmacological interventions, and the utility of different models of support.

Consensus statement of the International Summit on Intellectual Disability and Dementia related to end-of-life care in advanced dementia.

BACKGROUND: Adults with intellectual disability are affected by dementia at equivalent and elevated rates, many surviving into advanced age. End of life care and support considerations come into play among these individuals when most are in the advanced stage of dementia. METHODS: A preliminary report summarizing available literature and making initial recommendations was developed by a workgroup, reviewed by all conference participants and then was finalized by the workgroup. RESULTS: The International Summit on Intellectual Disability and Dementia produced a report on End of life care in advanced dementia that provides a synthesis statement which encompasses defining the state of advanced dementia, proposes use of palliative care services (including hospice) and recommends special efforts for enabling advanced directives and advance care planning prior to the extensive progression of dementia. The Summit further recommended that when aiding adults with advanced dementia, the following be undertaken: integrative efforts between intellectual disability and palliative care providers, specialized training for carers on end of life care and supports, and involvement of adults with intellectual disability early on in their advance care planning. CONCLUSIONS: The Consensus recommendations will ensure greater and more appropriate support at end of life for persons with intellectual disabilities and advanced dementia.

Noninvasive risk stratification for sudden death in asymptomatic patients with Wolff-Parkinson-White syndrome.

Sudden cardiac death (SCD) as the first clinical manifestation of Wolff-Parkinson-White (WPW) syndrome is a well-documented, although rare occurrence. The incidence of SCD in patients with WPW ranges from 0% to 0.39% annually. Controversy exists regarding risk stratification for patients with preexcitation on surface electrocardiogram (ECG), particularly in those who are asymptomatic. This article focuses on the role of risk stratification using exercise and pharmacologic testing in patients with WPW pattern on ECG.

The Importance of Integrated Care for Individuals With Intellectual and Developmental Disabilities.

People with intellectual and developmental disabilities (IDDs) commonly have complex healthcare challenges. An IDD is a condition that is a result of an abnormality beginning during a person's neurodevelopment, often occurring in utero but also possibly occurring up to age 18. Any injury or maldevelopment of the nervous system can often result in lifelong health complications in this population, including those involving intellect, language, motor skills, vision, hearing, swallowing, behavior, autism, seizures, digestion, and many other areas. Individuals with IDDs often have multiple health complications, and their care is usually provided by a number of different healthcare providers, including a primary care provider, various healthcare specialists who focus on their particular areas of concern, an oral health provider, and a behavioral specialist(s), if needed. The American Academy of Developmental Medicine and Dentistry appreciates that integrated care is essential to providing care to those with IDDs. The name of the organization itself includes both medical and dental aspects, and the organization's guiding principles include the concepts of integrated care, person-centered and family-centered approaches, and a deep appreciation for the importance of values and inclusion in a community. Continuing to provide education and training to healthcare practitioners is a key to improving health outcomes for individuals with IDDs. Additionally, focusing on the importance of integrated care will ultimately lead to a reduction in health disparities and improve access to quality healthcare services.

Feasibility of early discharge after implantable cardioverter-defibrillator procedures.

INTRODUCTION: Registry data demonstrate considerably low complication rates after implantable cardioverter-defibrillator (ICD) procedures for primary prevention of sudden death. Yet standard of care includes postimplant overnight in-hospital observation that may levy substantial unnecessary financial burden on health care systems. In appropriate patients, discharge soon after implant could translate into significant cost savings, if such practice does not result in complications. We applied a simple clinical algorithm to assess feasibility of discharge on the same day of ICD implantation in patients at low risk for procedural complications. METHODS: We prospectively randomized primary prevention ICD candidates at low risk for complications (not pacing-dependent or requiring bridging heparin anticoagulation) to next-day discharge with overnight in-hospital observation, or same-day discharge with remote monitoring for 24 hours after ICD implant. Implants were performed via cephalic vein access, and randomization occurred after 4-hours clinical observation and device interrogation. All patients were followed for a minimum of 6 weeks to assess acute procedural complications. RESULTS: 71 patients comprised the study cohort (mean age 62, 79% male) after 3 were excluded. The most common indication for ICD implant was ischemic cardiomyopathy with ejection fraction ≤35%. Device data obtained through 24-hour remote monitoring was comparable to 4-hour postimplant parameters in same-day discharge patients. No acute complications occurred in same-day discharge patients; 1 next-day discharge patient developed pneumothorax. CONCLUSION: ICD implantation with same-day discharge is reasonable in patients at low risk for complications. Remote monitoring can be useful in indicating lead-parameter stability during the immediate postoperative period.

Caring for Individuals With Intellectual and Developmental Disabilities in the COVID-19 Crisis.

Individuals with intellectual and developmental disabilities (IDDs) are among our most medically vulnerable neurologic patient population. As such, they are at particular risk of psychosocial and medical harm during the coronavirus disease 2019 (COVID-19) pandemic. Here, we highlight strategies to decrease potential infectious exposures and ensure continued optimal neurologic care for individuals with IDD during the COVID-19 pandemic. Finally, in a climate of potential medical resource restriction, we offer some suggestions for advocacy on behalf of individuals with IDD.

Reversible Mechanical Atrioventricular Block Caused By A Steerable Introducer Sheath During Transseptal Catheterization.

A 62-year-old woman presents for pulmonary vein isolation (PVI) for paroxysmal atrial fibrillation. During transseptal catheterization (TSC) the patient sustained mechanical injury to the atrioventricular node (AVN) with consequent complete heart block (CHB). Injury to the AVN and CHB recovered after approximately forty minutes. The patient subsequently underwent a successful PVI with the remainder of the hospital stay uneventful. We present a case of reversible injury to the AVN caused by a steerable introducer sheath during TSC and discuss the mechanisms of injury as well as potential measures to avoid such a complication in the future.

In memory of tachycardia: A wide complex tachycardia in a young male.

We present a case of a 24-year-old male with palpitations and a wide complex tachycardia. Baseline electrocardiogram (ECG) after termination of tachycardia demonstrates a normal rhythm but with inferior/anterolateral T-wave inversions (TWIs). Electrophysiologic study confirmed the diagnosis of posterior fascicular ventricular tachycardia successfully terminated by anatomic ablation of the left posterior fascicle. TWIs on the patient's baseline ECG were consistent with cardiac memory.

Consensus Statement of the International Summit on Intellectual Disability and Dementia Related to Nomenclature.

A working group of the 2016 International Summit on Intellectual Disability and Dementia was charged to examine the terminology used to define and report on dementia in publications related to intellectual disability (ID). A review of related publications showed mixed uses of terms associated with dementia or causative diseases. As with dementia research in the non-ID population, language related to dementia in the ID field often lacks precision and could lead to a misunderstanding of the condition(s) under discussion, an increasingly crucial issue given the increased global attention dementia is receiving in that field. Most articles related to ID and dementia reporting clinical or medical research generally provide a structured definition of dementia or related terms; social care articles tend toward term use without definition. Toward terminology standardization within studies/reports on dementia and ID, the Summit recommended that a consistent approach is taken that ensures (a) growing familiarity with dementia-related diagnostic, condition-specific, and social care terms (as identified in the working group's report); (b) creating a guidance document on accurately defining and presenting information about individuals or groups referenced; and

Treatment Resistant Epilepsy in Autism Spectrum Disorder: Increased Risk for Females.

The male:female ratio in autism spectrum disorder (ASD) averages greater than 4:1 while the male:female ratio of ASD with epilepsy averages less than 3:1. This indicates an elevated risk of epilepsy in females with ASD; yet, it is unknown whether phenotypic features of epilepsy and ASD differ between males and females with this comorbidity. The goal of this study is to investigate sex differences in phenotypic features of epilepsy and ASD in a prospective sample of 130 children and young adults with an initial ASD diagnosis and subsequent epilepsy diagnosis. All participants were characterized by standardized diagnostic inventories, parent/caregiver completed questionnaires, and medical/academic record review. Diagnostic classifications of epilepsy, ASD, and intellectual disability were performed by board certified neurologists and a pediatric neuropsychologist. Results demonstrated a lower male:female ratio (1.8:1) in individuals with ASD and treatment-resistant epilepsy relative to those with ASD and treatment-responsive epilepsy (4.9:1), indicating a higher risk of treatment-resistant epilepsy in females. Mild neuroimaging abnormalities were more common in females than males and this was associated with increased risk of treatment-resistance. In contrast, ASD symptom severity was lower in females compared with males. Findings distinguish females with ASD and epilepsy as a distinct subgroup at higher risk for a more severe epilepsy phenotype in the context of a less severe ASD phenotype. Increased risk of anti-epileptic treatment resistance in females with ASD and epilepsy suggests that comprehensive genetic, imaging, and neurologic screening and enhanced treatment monitoring may be indicated for this subgroup. Autism Res 2016, 9: 311-320. © 2015 International Society for Autism Research, Wiley Periodicals, Inc.

Guidelines for dementia-related health advocacy for adults with intellectual disability and dementia: National Task Group on Intellectual Disabilities and Dementia Practices.

Increasing numbers of adults with intellectual disabilities (ID) are living into old age. Though this indicates the positive effects of improved health care and quality of life, the end result is that more adults with ID are and will be experiencing age-related health problems and also exhibiting symptoms of cognitive impairment and decline, some attributable to dementia. Early symptoms of dementia can be subtle and in adults with ID are often masked by their lifelong cognitive impairment, combined with the benign effects of aging. A challenge for caregivers is to recognize and communicate symptoms, as well as find appropriate practitioners familiar with the medical issues presented by aging adults with lifelong disabilities. Noting changes in behavior and function and raising suspicions with a healthcare practitioner, during routine or ad hoc visits, can help focus the examination and potentially validate that the decline is the result of the onset or progression of dementia. It can also help in ruling out reversible conditions that may have similar presentation of symptoms typical for Alzheimer's disease and related dementias. To enable caregivers, whether family members or staff, to prepare for and advocate during health visits, the National Task Group on Intellectual Disabilities and Dementia Practices has developed guidelines and recommendations for dementia-related health advocacy preparation and assistance that can be undertaken by provider and advocacy organizations.

Delivery of epilepsy care to adults with intellectual and developmental disabilities.

Epilepsy is common in people with intellectual and developmental disabilities (IDD). In adulthood, patients with IDD and epilepsy (IDD-E) have neurologic, psychiatric, medical, and social challenges compounded by fragmented and limited care. With increasing neurologic disability, there is a higher frequency of epilepsy, especially symptomatic generalized and treatment-resistant epilepsies. The causes of IDD-E are increasingly recognized to be genetic based on chromosomal microarray analysis to identify copy number variants, gene panels (epilepsy, autism spectrum disorder, intellectual disability), and whole-exome sequencing. A specific genetic diagnosis may guide care by pointing to comorbid disorders and best therapy. Therapy to control seizures should be individualized, with drug selection based on seizure types, epilepsy syndrome, concomitant medications, and comorbid disorders. There are limited comparative antiepileptic drug data in the IDD-E population. Vagus nerve and responsive neural stimulation therapies and resective surgery should be considered. Among the many comorbid disorders that affect patients with IDD-E, psychiatric and sleep disorders are common but often unrecognized and typically not treated. Transition from holistic and coordinated pediatric to adult care is often a vulnerable period. Communication among adult health care providers is complex but essential to ensure best care when these patients are seen in outpatient, emergency room, and inpatient settings. We propose specific recommendations for minimum care standards for people with IDD-E.

Neuropsychiatric effects of cardiovascular drug therapy.

Various cardiovascular drugs have been shown to have neuropsychiatric effects that can be harmful or therapeutically beneficial to patients. As an example, both sedation and mental depression have been described in patients receiving centrally acting antihypertensive drugs and beta-adrenergic blockers, related to their antiadrenergic actions. In contrast, because of these antiadrenergic actions, agents like clonidine have been used to treat opiate, alcohol, and nicotine withdrawal, while beta blockers have been used to treat symptoms of performance anxiety, migraine, and psychocardiac disorders. Some antiarrhythmic drugs have been associated with delirium, and digitalis toxicity has been shown to cause hallucinations, mania, euphoria, and depression. The calcium-channel blocker verapamil has been used as an adjunctive treatment in patients with bipolar disorders. Since neuropsychiatric symptoms are seen in patients with cardiovascular disease, clinicians should be aware of the possible relationship between these symptoms and concurrent cardiovascular drug therapy.

The National Task Group on Intellectual Disabilities and Dementia Practices consensus recommendations for the evaluation and management of dementia in adults with intellectual disabilities.

Adults with intellectual and developmental disabilities (I/DD) are increasingly presenting to their health care professionals with concerns related to growing older. One particularly challenging clinical question is related to the evaluation of suspected cognitive decline or dementia in older adults with I/DD, a question that most physicians feel ill-prepared to answer. The National Task Group on Intellectual Disabilities and Dementia Practices was convened to help formally address this topic, which remains largely underrepresented in the medical literature. The task group, comprising specialists who work extensively with adults with I/DD, has promulgated the following Consensus Recommendations for the Evaluation and Management of Dementia in Adults With Intellectual Disabilities as a framework for the practicing physician who seeks to approach this clinical question practically, thoughtfully, and comprehensively.

Hyperkalemia induced T wave oversensing leading to loss of biventricular pacing and inappropriate ICD shocks.

Inappropriate ICD shocks remain a common problem. Double counting of single ventricular events can occur with biventricular ICDs implanted before univentricular sensing was available. Often this is due to a tachyarrhythmia or loss of left ventricular capture. This report describes a patient who developed hyperkalemia during hemodialysis, received inappropriate ICD shocks and experienced loss of biventricular pacing due to T wave rather than QRS double counting. Oversensing was abolished by reducing the potassium content of the dialysis bath. This underscores the need for careful interpretation of saved electrograms to determine the cause for, and appropriate treatment of, device related problems.