Remembering to remember: prospective memory in children with epilepsy.

Prospective memory, the ability to remember to perform an intended act in the future, is a complex process that involves several stages and cognitive domains. This study sought to investigate prospective memory functioning in children with idiopathic epilepsy using tasks from the Rivermead Behavioral Memory Test for Children (RBMT-C) and the Memory for Intentions Screening Test for Youth (MISTY). Performances on prospective memory task characteristics of the MISTY (i.

Clinical Practice Guidelines by the Infectious Diseases Society of America (IDSA), American Academy of Neurology (AAN), and American College of Rheumatology (ACR): 2020 Guidelines for the Prevention, Diagnosis and Treatment of Lyme Disease.

This evidence-based clinical practice guideline for the prevention, diagnosis, and treatment of Lyme disease was developed by a multidisciplinary panel representing the Infectious Diseases Society of America (IDSA), the American Academy of Neurology (AAN), and the American College of Rheumatology (ACR). The scope of this guideline includes prevention of Lyme disease, and the diagnosis and treatment of Lyme disease presenting as erythema migrans, Lyme disease complicated by neurologic, cardiac, and rheumatologic manifestations, Eurasian manifestations of Lyme disease, and Lyme disease complicated by coinfection with other tick-borne pathogens. This guideline does not include comprehensive recommendations for babesiosis and tick-borne rickettsial infections, which are published in separate guidelines.

Clinical Practice Guidelines by the Infectious Diseases Society of America (IDSA), American Academy of Neurology (AAN), and American College of Rheumatology (ACR): 2020 Guidelines for the Prevention, Diagnosis and Treatment of Lyme Disease.

This evidence-based clinical practice guideline for the prevention, diagnosis, and treatment of Lyme disease was developed by a multidisciplinary panel representing the Infectious Diseases Society of America (IDSA), the American Academy of Neurology (AAN), and the American College of Rheumatology (ACR). The scope of this guideline includes prevention of Lyme disease, and the diagnosis and treatment of Lyme disease presenting as erythema migrans, Lyme disease complicated by neurologic, cardiac, and rheumatologic manifestations, Eurasian manifestations of Lyme disease, and Lyme disease complicated by coinfection with other tick-borne pathogens. This guideline does not include comprehensive recommendations for babesiosis and tick-borne rickettsial infections, which are published in separate guidelines.

Clinical Practice Guidelines by the Infectious Diseases Society of America, American Academy of Neurology, and American College of Rheumatology: 2020 Guidelines for the Prevention, Diagnosis, and Treatment of Lyme Disease.

This evidence-based clinical practice guideline for the prevention, diagnosis, and treatment of Lyme disease was developed by a multidisciplinary panel representing the Infectious Diseases Society of America (IDSA), the American Academy of Neurology (AAN), and the American College of Rheumatology (ACR). The scope of this guideline includes prevention of Lyme disease, and the diagnosis and treatment of Lyme disease presenting as erythema migrans, Lyme disease complicated by neurologic, cardiac, and rheumatologic manifestations, Eurasian manifestations of Lyme disease, and Lyme disease complicated by coinfection with other tick-borne pathogens. This guideline does not include comprehensive recommendations for babesiosis and tick-borne rickettsial infections, which are published in separate guidelines.

Inherited Pain Syndromes and Ion Channels.

Individuals rely on the perception of pain to avoid injury, to signal disease, and to warn about tissue inflammation and damage. However, the inheritance of inappropriate, extreme, or inadequate pain production is a source of significant human suffering. Substantial progress has been made in our understanding of the genetics and pathophysiology of pain through the study of individuals and families with several specific inherited pain syndromes.

Tuberous sclerosis complex.

Tuberous sclerosis complex is an autosomal-dominant, neurocutaneous, multisystem disorder characterized by cellular hyperplasia and tissue dysplasia. The genetic cause is mutations in the TSC1 gene, found on chromosome 9q34, and TSC2 gene, found on chromosome 16p13. The clinical phenotypes resulting from mutations in either of the 2 genes are variable in each individual.

Similar trends in serum VEGF-D levels and kidney angiomyolipoma responses with longer duration sirolimus treatment in adults with tuberous sclerosis.

Context: We have previously shown that serum VEGF-D is elevated at baseline, correlates with kidney angiomyolipoma size at baseline and 12 months, and decreases with sirolimus treatment in adults with tuberous sclerosis complex (TSC). To further investigate the utility of serum VEGF-D for longer term monitoring of TSC kidney disease, we present VEGF-D level results with 24 month follow-up.

Objective: To compare 24 month VEGF-D levels in two subgroups of sirolimus treated patients (OFF SIROLIMUS AFTER 12 MONTHS or ON SIROLIMUS AFTER 12 MONTHS).

SMART syndrome (stroke-like migraine attacks after radiation therapy) in adult and pediatric patients.

SMART syndrome (stroke-like migraine attacks after radiation therapy) is a rare condition that involves complex migraines with focal neurologic findings in patients following cranial irradiation for central nervous system malignancies. Little is known about the mechanisms behind the disorder, making successful treatment challenging. We report 2 new cases of SMART syndrome in pediatric patients as well as review all documented cases of the syndrome.

The ebb and flow model: a philosophy of organizational learning in the academic health center.

Academic health centers (AHCs) have traditionally been a vibrant locale for cutting-edge medical research, androgogic education and innovative clinical care for the most vexing diseases. While these pursuits have coexisted and flourished, the realities of the health-care business environment have demanded reformatting and emulation of a corporate organizational model. This evolution has impacted the core identities of the AHC and challenged individual medical-educators, clinician-scientists and basic science investigators to persist and succeed in this milieu.

Autonomic dysfunction in childhood Guillain-Barré syndrome.

This investigation correlated incidence and degree of autonomic dysfunction with the degree of motor impairment in children hospitalized with Guillain-Barré syndrome. Motor weakness varies, as does the effect on autonomic function including heart rate, vasomotor stability, sweating, continence, and blood pressure. After Institutional Review Board approval, hospitalized patients with Guillain-Barré syndrome <19 years were included for retrospective chart review.

Multicenter phase 2 trial of sirolimus for tuberous sclerosis: kidney angiomyolipomas and other tumors regress and VEGF- D levels decrease.

Background: Tuberous sclerosis (TSC) related tumors are characterized by constitutively activated mTOR signaling due to mutations in TSC1 or TSC2.

Methods: We completed a phase 2 multicenter trial to evaluate the efficacy and tolerability of the mTOR inhibitor, sirolimus, for the treatment of kidney angiomyolipomas.

Results: 36 adults with TSC or TSC/LAM were enrolled and started on daily sirolimus.

The relationship between the neuromodulator adenosine and behavioral symptoms of autism.

The neuromodulator adenosine is an endogenous sleep promoter, neuroprotector and anticonvulsant, and people with autism often suffer from sleep disruption and/or seizures. We hypothesized that increasing adenosine can decrease behavioral symptoms of autism spectrum disorders, and, based on published research, specific physiological stimuli are expected to increase brain adenosine. To test the relationship between adenosine and autism, we developed a customized parent-based questionnaire to assess child participation in activities expected to influence adenosine and quantify behavioral changes following these experiences.

Clinical categorization of childhood syncope.

Syncope is a self-limited loss of consciousness produced by cerebral hypoperfusion/hypoxia. The objective of this study was to categorize the etiology and determine the frequency of concurrent epilepsy and whether laboratory testing is diagnostically useful. This was an institutional review board-approved retrospective chart review.

Parental report of behavioral and cognitive diagnoses in childhood-onset epilepsy: A case-sibling-controlled analysis.

Evidence from multiple sources has highlighted the increased burden of cognitive, behavioral, and psychiatric disorders in childhood-onset epilepsy. Some of this increased morbidity, however, is attributable to underlying structural and metabolic insults. We assessed whether cognitive/behavioral/psychiatric disorders are associated with epilepsy of unknown or presumed genetic cause in young people with epilepsy (cases) compared with sibling controls.

A 16-year-old girl with bilateral visual loss and left hemiparesis following an immunization against human papilloma virus.

We report the course of a 16-year-old girl who presented with near complete visual loss associated with chiasmal neuritis and a biopsy proven tumefactive demyelinating lesion on magnetic resonance imaging (MRI) in association with a recent immunization against human papilloma virus.

Acute tumefactive demyelinating lesions in a pediatric patient with known diagnosis of multiple sclerosis: review of the literature and treatment proposal.

Tumefactive demyelinating lesions are a known but uncommon complication of multiple sclerosis, a disease rarely reported in children. This is the case of a 16-year-old African American patient with multiple sclerosis, who developed 2 tumefactive demyelinating lesions. Review of the literature and our own experience helped formulate an algorithm for therapeutic options during an acute attack.

Childhood orbital pseudotumor: case report and literature review.

Orbital pseudotumor, also known as idiopathic orbital inflammation, is a benign, idiopathic, noninfectious, and nonneoplastic clinical syndrome with an inflammatory mass within the orbit and no identifiable cause. This disease is rare in the pediatric population, especially in very young children. In this article, the authors describe a 2-year-old girl who was presented with right eye ptosis, near-complete ophthalmoplegia, decreased visual acuity, and pupillary changes.

Paroxysmal nonepileptic events of childhood.

The paroxysmal nonepileptic events of childhood are a group of disorders, syndromes, and phenomena that mimic true epileptic seizures. Clinical experience and a clear description of the event in question will usually lead to a correct categorization. They span in age from neonate to young adult and are apt to be the most common diagnostic challenges clinicians face regularly.

MR imaging and prognosis of hypoxic-ischemic leukoencephalopathy.

Objective: To correlate prognosis of hypoxic-ischemic white-matter injury with magnetic resonance imaging (MRI).

Methods: In a retrospective review at an urban medical center, three adult and three pediatric patients with predominant white-matter involvement caused by hypoxic- ischemic injury were evaluated over a 15-month period.

Results: Five patients with MRI-defined hypoxic-ischemic leukoencephalopathy had a favorable outcome, and one with associated diffusion-weighted abnormality of deep gray structures died.