Opsoclonus-myoclonus-ataxia syndrome in children.

Opsoclonus-myoclonus-ataxia syndrome is a rare neuroimmunologic disorder typically presenting in previously healthy infants and toddlers. It is characterized by a clinical triad of (1) erratic saccadic intrusions; (2) myoclonus and/or ataxia; (3) behavioral features, typified by developmental plateauing, irritability and insomnia. About half of cases are associated with an underlying neuroblastoma and diagnostic imaging is essential once OMAS is suspected. A thorough workup, including serum, urine, and cerebrospinal fluid studies is critical to identify underlying biomarkers of OMAS itself or neuroblastoma. Historically, many children had relatively poor long-term outcomes, with residual neurologic and/or neuropsychiatry sequelae typical. More recent concepts have emphasized combined immunotherapy regimens that offer hope for better outcomes in children with this remarkable, challenging disease.

Low-dose intrathecal rituximab is a safe and potentially effective treatment for pediatric neuroimmunologic disorders.

Historically, treatment options for refractory neuroimmunologic disorders have been limited. Use of intrathecal rituximab has been described in a few case reports but experience in pediatric patients is limited. Here, we report our experience with intrathecal rituximab in 5 pediatric patients with refractory neuroimmunologic conditions. Patients were identified based on treatment-refractory symptoms despite first and second-line therapies and treated according to a standardized protocol. Although individual outcomes varied, intrathecal rituximab showed a favorable safety profile and was well-tolerated. Three out of five patients showed evidence of a positive clinical response assessed by modified Rankin score or Mitchell-Pike Opsoclonus-Myoclonus score. Findings from this retrospective observational study suggest that intrathecal rituximab is a safe and potentially effective therapy in carefully selected patients with refractory neuroimmunologic disorders despite appropriate first and second-line therapies.

Implementing Routine HIV Screening in an Urban Adolescent Population at a General Pediatric Clinic.

PURPOSE: To increase the rate of routine HIV screening during preventative visits for adolescent patients aged 15 to 21 in a pediatric and adolescent clinic in accordance with national recommendations, which are poorly implemented nationwide. METHODS: This was a quality improvement initiative. Four plan-do-study-act (PDSA) cycles were conducted from May 2016 to February 2020. Interventions included education of and reminders for the multidisciplinary team on guidelines and testing, creation of a standardized workflow, introduction of a rapid point-of-care HIV antibody test (POCT), and implementation of an opt-out, medical assistant/nursing-driven protocol for HIV rapid point-of-care testing. The primary outcome measure was the monthly percentage of adolescents screened for HIV during preventative visits. Data is presented in a p-control chart and means were adjusted for special cause variation according to the Institute for Healthcare Improvement guidelines. RESULTS: Rates of routine HIV screening at preventative visits for youth ages 15 to 21 increased from the pre-intervention rate of 5.16% to a final rate of 41.5% over four PDSA cycles. Mean screening rates were adjusted after introducing the HIV POCT (+18.5%) and after implementing the medical assistant/nursing-driven protocol (+17.9%). CONCLUSIONS: We successfully increased routine HIV screening rates at preventative visits for adolescents at an urban pediatric and adolescent clinic. This was in large part due to testing with a rapid HIV POCT and a clinic protocol allowing medical assistants and nurses to order the test under a physician's name as part of the intake process. Ours can be a model for other clinics.