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. The target audience for this guideline includes primary care physicians and specialists caring for this condition such as infectious diseases specialists, emergency physicians, internists, pediatricians, family physicians, neurologists, rheumatologists, cardiologists and dermatologists in North America.

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. The target audience for this guideline includes primary care physicians and specialists caring for this condition such as infectious diseases specialists, emergency physicians, internists, pediatricians, family physicians, neurologists, rheumatologists, cardiologists and dermatologists in North America.

A Study of Self-Regulated Learning of Child Neurology for Medical Students.

OBJECTIVE: Compare medical student preferences and outcomes after engaging two child neurology clinical scenarios with different feedback formats. METHODS: After IRB exemption, online case sce- narios were presented with periodic multiple choice questions. Cases provided immediate pro- grammed feedback (IPF), or immediate in-depth programmed feedback (IDPF). Anonymous sur- veys collected datafrom students. Resultswere then tabulated and analyzed. RESULTS: Sixty-five of 240 eligible (27%) second-, third-, and fourth-year medical students partici- pated. 'he modest-depth IPF format was preferred by 83% (54/65) of students. The cases did increase interest in child neurology significantly for second- and fourth-year students (P < .01). Students' scores increased significantly in relation to the number of IDPF links accessed (P < .01). Students who were self-characterized as internally motivated were significantly more likely to access in-depth feedback (P < .008). CONCLUSION: Self-regulated learning can be developed, as can an interest in child neurology, when scenarios are offered early in medical training.

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. The AHC has a unique capacity to muster the innate learning drive of these individuals into an organizational mission as it balances the pressures exerted from both the internal and external environments. The AHC as an organization can be viewed as an experimental condition with modifiable variables to which its professionals can react, adapt to, and transform. Organizational learning and change implementation is in essence an experiment in human behavior modification. While all individuals are subject to change, merely assembling them in a single locale determines neither a predictable homogeneous outcome nor the success of their endeavor. This article highlights some of these propositions and offers a philosophical approach to advance the AHC as an organization through the creativity and innovation of its professional ranks.

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.e., cue-type, length of time delay, and response type) were also compared between and across participant groups. Healthy children (N = 26) were found to have higher overall IQ and verbal IQ scores when compared to children with epilepsy (N = 19). Group differences in prospective memory functioning were found in subtests of the RBMT-C but not on the MISTY. Lastly, while there was no significant interaction effect between the groups and MISTY task characteristics, main effects were found across participant groups; all participants performed better on event-based tasks when compared to time-based tasks and on two-minute when compared to 10-minute time delays. Overall, findings suggest potential differences in cognitive functioning, particularly in IQ and prospective memory, in children with idiopathic epilepsy, though due to differences in findings across prospective memory tasks, further research is warranted to more definitively ascertain the extent, if any, of prospective memory deficits in children with epilepsy.

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. The target audience for this guideline includes primary care physicians and specialists caring for this condition such as infectious diseases specialists, emergency physicians, internists, pediatricians, family physicians, neurologists, rheumatologists, cardiologists and dermatologists in North America.

Frequency, prognosis and surgical treatment of structural abnormalities seen with magnetic resonance imaging in childhood epilepsy.

The epidemiology of lesions identified by magnetic resonance imaging (MRI), along with the use of pre-surgical evaluations and surgery in childhood-onset epilepsy patients has not previously been described. In a prospectively identified community-based cohort of children enrolled from 1993 to 1997, we examined (i) the frequency of lesions identified by MRI; (ii) clinical factors associated with 'positive' MRI scans; and (iii) the utilization of comprehensive epilepsy evaluations and neurosurgery. Of the original cohort of 613 children, 518 (85%) had usable MRI scans. Eighty-two (16%) had MRI abnormalities potentially relevant to epilepsy ('positive' scans). Idiopathic epilepsy syndromes were identified in 162 (31%) of whom 3% had positive scans. The remainder had non-idiopathic epilepsy syndromes of which 22% had positive MRI findings. Multiple logistic regression analysis identified non-idiopathic epilepsy and abnormal motor-sensory (neurological) examinations as predictors of a positive MRI scan. Of the non-idiopathic patients with normal neurological exams and who were not pharmacoresistant, 10% had positive MRI scans, including four patients with gliomas. Evaluations at comprehensive epilepsy centres occurred in 54 pharmacoresistant cases. To date 5% of the imaged cohort or 8% of non-idiopathic epilepsy patients have undergone surgical procedures (including vagal nerve stimulator implantation) to treat their epilepsy (n = 22) or for tumours (n = 6) without being drug resistant. Applying our findings to the general population of children in the USA, we estimate that there will be 127/1 000 000 new cases per year of pharmacoresistant epilepsy, and 52/1 000 000 childhood-onset epilepsy patients undergoing epilepsy evaluations. In addition, approximately 27/1 000 000 will have an epilepsy-related surgical procedure. These findings support recommendations for the use of MRI in evaluating newly diagnosed paediatric epilepsy patients, especially with non-idiopathic syndromes, and provide estimates on the utilization of comprehensive evaluations and surgery.

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. Our analyses included 217 cases who were enrolled in the Connecticut Study of Epilepsy between 1993 and 1997 and 217 sibling controls. Information was collected from a parent interview conducted 8-9years after the case was diagnosed with epilepsy. Relative to controls, parents were more likely to report that their case children were slow learners (OR=4.6, P<0.001), had a language disorder (OR=5.8, P<0.001), and had engaged in self-injurious behaviors other than suicide attempts (OR=5.5, P=0.013). Future research should examine whether these conditions first present during childhood influence prognosis into adulthood.

Residual cognitive effects of uncomplicated idiopathic and cryptogenic epilepsy.

We assessed residual cognitive deficits in young people with idiopathic and cryptogenic epilepsy. In the setting of an ongoing prospective study, we invited participants initially diagnosed and enrolled in the cohort 8-9 years earlier to undergo standardized neuropsychological assessment. Sibling controls were invited when available. We analyzed 143 pairs in which cases had idiopathic or cryptogenic epilepsy and both case and control had normal intelligence. Compared with that for siblings, the Full Scale IQ for cases was 3.3 points lower (P=0.01) mainly due to slower processing speed, which was 5.6 points lower (P=0.0004). Word reading (P=0.04) and spelling (P=0.01), but not other scores, were also lower in cases. Remission status and drug use did not influence findings. In young people of normal intelligence with idiopathic or cryptogenic childhood-onset epilepsy, substantial residual effects of epilepsy appear to be confined largely to slower processing speed.

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. She recovered completely with high-dose steroid therapy. The authors review the literature and discuss the diagnostic implications and treatment strategies.

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. The key to diagnosis is a detailed history and careful observation. Despite the large number of discrete entities enumerated herein, common principles in clinical approach are successful and described. Each entity can pose a significant clinical challenge in identification, etiologic pathophysiology, genetics, and management. A simple division is offered here separating those episodes that are associated with an altered mental status or occurring during sleep and those without an altered mental status or occurring while awake.

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. These studies have led to the discovery of a number of gene mutations associated with specific ion channel disturbances that produce familial inherited pain sensitivity and insensitivity syndromes. The sodium channel has been identified as the primary determinant of most of these syndromes. This article focuses on the inherited pain syndromes and their corresponding ion channel mutations. There is hope that through continued research into these ion channels and pain syndromes, targeted drug therapy would be fruitful and beneficial to those afflicted.

Postinfectious myasthenia gravis: report of two children.

We report two children with transient myasthenia gravis preceded by viral illnesses. The first is a 5-year-old boy who developed oculobulbar weakness 2 weeks following a varicella-zoster infection. The second is a 4-year-old boy who developed facial diplegia and dysarthria several weeks following a viral pharyngitis. Myasthenia gravis was diagnosed based on the substantial decremental changes on 3 Hz repetitive motor nerve stimulation studies for the first child and on the positive edrophonium test and complete improvement in symptoms during pyridostigmine therapy for both children. In both cases, the symptoms gradually resolved and have not recurred following discontinuation of pyridostigmine. Molecular mimicry between the acetylcholine receptor and viral proteins might provide the nidus for the immune response in this variant of myasthenia gravis.

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. Herein, advances in the understanding of molecular mechanisms in tuberous sclerosis complex are reviewed, and current guidelines for diagnosis, treatment, follow-up, and management are summarized.

Diagnostic approach in patients with symmetric imaging lesions of the deep gray nuclei.

BACKGROUND: Selective insult to brain deep gray matter nuclei is uncommon, may be congenital or acquired, acute or chronic, and varied in etiology. Determining the etiology relies on history, clinical presentation, laboratory investigations, and lesion pattern on Neuroimaging. REVIEW SUMMARY: We review the differential diagnosis and clinical, laboratory and neuroimaging pattern of conditions that manifest with lesions of deep gray matter nuclei in the context of representative case studies. CONCLUSION: While presentations may vary in individual patients, the essentials of history, clinical examination, laboratory evaluation,and neuroimaging lesion pattern can be efficiently directed to differentiate the various etiologies of deep gray matter nuclei lesions. In this review we focus on the etiologic classification and diagnostic approach to acute and chronic conditions that manifest on neuroimaging with bilateral symmetric lesions of deep gray matter nuclei.

Brain abnormalities in tuberous sclerosis complex.

Tuberous sclerosis complex is an autosomal dominant multisystem disorder. Spontaneous mutations occur in up to 60% of patients with gene loci located on chromosomes 9q34 (TSC1) and 16p13 (TSC2). Diagnosis is established with the identification of various neurocutaneous markers and multiple organ system hamartomas. The variable expression of severity, the potential for cognitive dysfunction, and epilepsy compound the clinical picture. The intracranial abnormalities include the identification of migration and hamartomatous brain lesions, such as tubers, subependymal nodules, and subependymal giant cell astrocytomas. A number of other neuroimaging and morphometric abnormalities coexist, which can be identified with current neuroimaging techniques. This review examines the spectrum of brain abnormalities encountered in tuberous sclerosis complex and presents them as not merely a collection of lesions but more cohesively in the context of a global neuronal migration disorder.