Hypotheses about sub-optimal hydration in the weeks before coronavirus disease (COVID-19) as a risk factor for dying from COVID-19.

To address urgent need for strategies to limit mortality from coronavirus disease 2019 (COVID-19), this review describes experimental, clinical and epidemiological evidence that suggests that chronic sub-optimal hydration in the weeks before infection might increase risk of COVID-19 mortality in multiple ways. Sub-optimal hydration is associated with key risk factors for COVID-19 mortality, including older age, male sex, race-ethnicity and chronic disease. Chronic hypertonicity, total body water deficit and/or hypovolemia cause multiple intracellular and/or physiologic adaptations that preferentially retain body water and favor positive total body water balance when challenged by infection.

Quantitative MRI analysis in children with multiple sclerosis: a multicenter feasibility pilot study.

Background: Pediatric multiple sclerosis (MS) is a rare disorder with significant consequences. Quantitative MRI measurements may provide significant insights, however multicenter collaborative studies are needed given the small numbers of subjects. The goal of this study is to demonstrate feasibility and evaluate lesion volume (LV) characteristics in a multicenter cohort of children with MS.

Pediatric multiple sclerosis.

In the past 5 years, there has been an exponential growth in the knowledge about multiple sclerosis (MS) in children and adolescents. Recent publications have shed light on its diagnosis, pathogenesis, clinical course, and treatment. However, there remain several key areas that require further exploration.

Switching multiple sclerosis patients with breakthrough disease to second-line therapy.

Background: Multiple sclerosis (MS) patients with breakthrough disease on immunomodulatory drugs are frequently offered to switch to natalizumab or immunosuppressants. The effect of natalizumab monotherapy in patients with breakthrough disease is unknown.

Methods: This is an open-label retrospective cohort study of 993 patients seen at least four times at the University of California San Francisco MS Center, 95 had breakthrough disease on first-line therapy (60 patients switched to natalizumab, 22 to immunosuppressants and 13 declined the switch [non-switchers]).

Multiple sclerosis therapies in pediatric patients with refractory multiple sclerosis.

Background: Currently available disease-modifying therapies (DMTs) are known to be only partially effective in adults with multiple sclerosis (MS). Little is known about pediatric patients with MS who experience refractory disease while receiving first-line DMTs.

Objective: To assess the occurrence and management of refractory disease in a group of pediatric patients with MS treated with first-line DMTs approved for adult patients within a network of pediatric MS centers in the United States.

Health-related quality of life is reduced in pediatric multiple sclerosis.

The health-related quality of life of children with multiple sclerosis was compared with that of healthy children and of those with other neurologic diseases. The Pediatric Quality of Life Inventory Version 4.0 was administered to children with multiple sclerosis and clinically isolated syndrome and their parents (proxy reporters) at the University of California, San Francisco (UCSF), Regional Pediatric Multiple Sclerosis Center.

Vitamin D status is associated with relapse rate in pediatric-onset multiple sclerosis.

Objective: We sought to determine if vitamin D status, a risk factor for multiple sclerosis, is associated with the rate of subsequent clinical relapses in pediatric-onset multiple sclerosis.

Methods: This is a retrospective study of patients with pediatric-onset multiple sclerosis or clinically isolated syndrome who were consecutively recruited into a prospective cohort at their clinical visit at the pediatric multiple sclerosis center of University of California, San Francisco or State University of New York at Stony Brook. Of 171 eligible patients, 134 (78%) with multiple sclerosis/clinically isolated syndrome were included in the cohort; a further 24 were excluded from this analysis due to lack of available serum (n = 7) or lack of follow-up (n = 17).

Treatment of multiple sclerosis in children and adolescents.

Importance Of The Field: Pediatric multiple sclerosis is an acquired inflammatory, demyelinating CNS disorder associated with recurrent episodes of neurologic dysfunction. Precise diagnosis is increasingly important as disease modifying therapies have been developed in adults and introduced into pediatric practice.

Areas Covered In This Review: Literature published over the past two decades relating to pharmacologic treatment of multiple sclerosis (MS) in adults and children is reviewed, with emphasis on current publications.

Pediatric multiple sclerosis.

Pediatric multiple sclerosis (MS) accounts for up to 5% of all MS cases. Work conducted over the past 5 years has provided new information about the treatment, pathogenesis, demographics, and natural history of this disorder. Genetic and environmental factors seem to exert critical influences on its development.

Difference in disease burden and activity in pediatric patients on brain magnetic resonance imaging at time of multiple sclerosis onset vs adults.

Objective: To compare initial brain magnetic resonance imaging (MRI) characteristics of children and adults at multiple sclerosis (MS) onset.

Design: Retrospective analysis of features of first brain MRI available at MS onset in patients with pediatric-onset and adult-onset MS.

Setting: A pediatric and an adult MS center.

Pediatric multiple sclerosis.

The diagnosis of multiple sclerosis (MS) in a child remains challenging, given the limited diagnostic criteria and the somewhat poorly defined overlap with acute disseminated encephalomyelitis. Although there are many similarities between pediatric-onset and adult-onset MS, an earlier age at disease presentation seems to be associated with specific features such as more frequent encephalopathy, seizures, and brainstem and cerebellar symptoms during the first event. In addition, the initial brain MRI scan of younger patients shows more frequent involvement of the posterior fossa and higher numbers of ovoid, ill-defined T2-bright foci that often partially resolve on the follow-up scan, thereby challenging early diagnosis.

Sorting through the pediatric MS spectrum with brain MRI.

Distinguishing between a first episode of multiple sclerosis and acute disseminated encephalomyelitis in children who present with an initial demyelinating event can be a clinical challenge. New brain MRI criteria that aim to differentiate these clinical presentations, and revised McDonald MRI criteria specific for the pediatric population, are both worthy of note.

Pediatric multiple sclerosis.

Diagnosing multiple sclerosis (MS) in a child is challenging because of the limited diagnostic criteria and their overlap with acute disseminated encephalomyelitis. Pediatric-onset MS patients are more likely to be male, have seizures, and have brainstem and cerebellar symptoms than adults, and are less likely to have spinal cord symptoms than adults. They mostly experience a relapsing-remitting course.

Eating disorder and metabolism in narcoleptic patients.

Study Objective: To evaluate eating behavior and energy balance as a cause of increased body mass index (BMI) in narcolepsy.

Design: Case controlled pilot study.

Settings: University hospital.

Functional imaging of cataplexy during status cataplecticus.

Study Objective: To identify the neural structures and pathways underlying cataplexy during status cataplecticus in a narcoleptic patient, using brain perfusion single photon emission computed tomography (SPECT).

Methods: A 68-year-old woman with hypocretin-deficient narcolepsy-cataplexy suffered status cataplecticus after having stopped clomipramine. She underwent a 99mTc-ethylcysteinate dimer brain SPECT during an episode of cataplexy; this image was compared with her brain SPECT during an intervening asymptomatic period.

Pediatric multiple sclerosis.

Multiple sclerosis (MS) occurs at all ages of the pediatric population. Childhood MS may represent up to 10% of all MS cases. Establishing the diagnosis of MS in a child is complicated by the limited diagnostic criteria and the possibility of significant clinical and magnetic resonance imaging (MRI) overlap with acute disseminated encephalomyelitis and other pediatric diseases.

[Osteopontin, a multi-faceted molecule].

Osteopontin (OPN) was initially isolated from bovine bone cortex, as a complex syalilated phospho-glyco-protein of around 60 kDa, with many postranslational modifications. It has been long considered a structural bone protein linking bone cells to the bone extracellular matrix (osteo : bone, pontin : bridge). It has been cloned for the first time in 1986.

The genetics of narcolepsy.

Human narcolepsy is a genetically complex disorder. Family studies indicate a 20-40 times increased risk of narcolepsy in first-degree relatives and twin studies suggest that nongenetic factors also play a role. The tight association between narcolepsy-cataplexy and the HLA allele DQB1*0602 suggests that narcolepsy has an autoimmune etiology.

Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine.

An expanded polyglutamine domain in huntingtin underlies the pathogenic events in Huntington disease (HD), characterized by chorea, dementia and severe weight loss, culminating in death. Transglutaminase (TGase) may be critical in the pathogenesis, via cross-linking huntingtin. Administration of the TGase competitive inhibitor, cystamine, to transgenic mice expressing exon 1 of huntingtin containing an expanded polyglutamine repeat, altered the course of their HD-like disease.