Pediatric neurology
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Pediatric neurology · Oct 2019
Review Case ReportsFibrocartilaginous Embolism of the Spinal Cord in Children: A Case Report and Review of Literature.
Fibrocartilaginous embolism is assumed to be caused by fibrocartilaginous nucleus pulposus component migration through retrograde embolization to the spinal cord artery. Fibrocartilaginous embolism is currently not well recognized among pediatricians because of its rarity. We describe a previously healthy 15-year-old soccer player who, after kicking a ball, developed progressive weakness in both legs and urinary retention the next day. ⋯ Affected areas of the spinal cord were distributed evenly from the cervical to thoracolumbar regions. Although steroids and anticoagulants were most commonly used, the prognosis was quite poor (mild to severe sequelae with three deaths). Although fibrocartilaginous embolism is a very rare condition, physicians should be aware of the characteristics and include fibrocartilaginous embolism of the spinal cord in their differential diagnosis, especially for physically active patients.
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Pediatric neurology · Oct 2019
Case ReportsAcute Disseminated Encephalomyelitis (ADEM) and Increased Intracranial Pressure Associated With Anti-Myelin Oligodendrocyte Glycoprotein Antibodies.
Antibodies to the myelin oligodendrocyte glycoprotein (MOG) have been identified in about 40% of children with acute disseminated encephalomyelitis (ADEM). The objective of this report is to describe three individuals with fulminant ADEM complicated by increased intracranial pressure associated with the presence of the anti-MOG antibodies. ⋯ Increased intracranial pressure complicating MOG antibody-related ADEM is a unique finding in our cases. This can complicate the clinical picture of ADEM and confers high morbidity. Long-term immunosuppression is warranted in selected cases with persistent seropositivity.
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Pediatric neurology · Sep 2019
Impact of Age and Motor Function in a Phase 1/2A Study of Infants With SMA Type 1 Receiving Single-Dose Gene Replacement Therapy.
This study characterizes motor function responses after early dosing of AVXS-101 (onasemnogene abeparvovec) in gene replacement therapy in infants with severe spinal muscular atrophy type 1 (SMA1). ⋯ The rapid, significant motor improvements among infants with severe SMA1 treated with AVXS-101 at an early age highlight the importance of newborn screening and early treatment and demonstrate the therapeutic potential of AVXS-101 regardless of baseline motor function.
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Osmotic demyelination syndrome is an acute demyelination process that usually occurs several days following an osmotic stress. This syndrome is rare in adults (0.4% to 0.56%) and even more uncommon in children. We performed a review of all reported pediatric osmotic demyelination syndrome patients from 1960 to 2018. ⋯ Sixty percent of the children had a complete neurological recovery. Current management of osmotic demyelination syndrome in children consists of supportive medical care, steroids, and intravenous immunoglobulin. Our review of the literature supports the hypothesis that steroids and immunoglobulins are potentially helpful, although additional controlled studies are needed.
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Pediatric neurology · Mar 2019
ReviewPediatric West Nile Virus-Associated Neuroinvasive Disease: A Review of the Literature.
Over the past two decades, West Nile virus has become the most common arbovirus in North America, leading to several outbreaks and infecting thousands of people. Mosquitos help transmit the virus in the majority of cases, but transmission occurs via blood transfusions, organ transplantation, and possibly pregnancy and breastfeeding. While most infected patients experience mild to no symptoms, thousands of West Nile virus-associated neuroinvasive cases have been reported in the United States, with over 700 cases occurring in children from 2003 to 2016. ⋯ Although older age and chronic disease are risk factors for neuroinvasive West Nile virus disease in adults, the specific factors that influence the risk in pediatric populations have not been fully elucidated. This review summarizes the most recent literature regarding West Nile virus-associated neuroinvasive disease, especially as it pertains to the pediatric population. Moreover, the review describes the epidemiology, clinical, laboratory, and radiographic findings, and outlines the various therapies that have been trialed and potential future research directions.