ABSTRACT
OBJECTIVE: It may be difficult for clinicians to estimate the prognosis of pediatric acute transverse myelitis (ATM). The aim of this study was to define prognostic factors for relapsing disease and poor outcome in pediatric ATM. METHODS: This prospective cohort study included 49 children, 18 boys and 31 girls (median age 13.1 years, IQR 6.5-16.2) with a first episode of ATM. Factors associated with relapsing disease and poor outcome (Expanded Disability Status Scale (EDSS) ≥ 4) were assessed during a median follow-up of 37 months (IQR 18-75). RESULTS: In total, 14 patients (29%) experienced ≥ 1 relapse(s) and nine patients (18%) had a poor outcome. Factors at onset associated with relapsing disease included higher age (16.1 vs. 11.6 years, p = 0.002), longer time to maximum severity of symptoms (5.5 vs. 3 days, p = 0.01), lower maximum EDSS score (4.0 vs. 6.5, p = 0.003), short lesion on spinal MRI (64 vs. 21%, p = 0.006), abnormalities on brain MRI (93 vs. 44%, p = 0.002) and presence of oligoclonal bands in cerebrospinal fluid (67 vs. 14%, p = 0.004). The only factor associated with poor outcome was presence of a spinal cord lesion on MRI without cervical involvement (56 vs. 14%, p = 0.02). CONCLUSION: Pediatric ATM patients presenting with clinical, radiological and laboratory features associated with multiple sclerosis (MS) are at risk for relapsing disease. In absence of these known MS risk factors at onset of disease these patients are at low risk for relapses. Only a minority of pediatric ATM patients in this cohort have a poor outcome.
Subject(s)
Multiple Sclerosis/diagnosis , Myelitis, Transverse/diagnosis , Acute Disease , Adolescent , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Male , Multiple Sclerosis/metabolism , Multiple Sclerosis/pathology , Multiple Sclerosis/physiopathology , Myelitis, Transverse/metabolism , Myelitis, Transverse/pathology , Myelitis, Transverse/physiopathology , Neuromyelitis Optica/diagnosis , Neuromyelitis Optica/metabolism , Neuromyelitis Optica/pathology , Neuromyelitis Optica/physiopathology , Outcome Assessment, Health Care , Prognosis , RecurrenceABSTRACT
Neuromyelitis optica (NMO) is a rare autoimmune disease affecting the optic nerves and spinal cord. In the majority of NMO patients anti-aquaporin-4 antibodies (AQP4-IgG) are detected. Here we assessed a nationwide incidence of AQP4-IgG-seropositive NMO spectrum disorders (NMOSD) in the Netherlands based on results of one central laboratory. Data were collected since the introduction of the highly sensitive cell-based assay for six consecutive years. Samples from 2795 individual patients have been received; of them 94 (3.4%) were seropositive. Based on the Dutch population with 16.6 million inhabitants, the mean incidence of AQP4-IgG-seropositive NMOSD was calculated at 0.09 per 100,000 people.
ABSTRACT
BACKGROUND: Traumatic brain injury (TBI) is in the developed countries the most common cause of death and disability in childhood. AIM: The purpose of this study is to estimate the incidence of TBI for children and young people in an urbanised region of the Netherlands and to describe relevant characteristics of this group. METHODS: Patients, aged 1 month - 24 years who presented with traumatic brain injury at the Erasmus University Hospital (including the Sophia Children's Hospital) in 2007 and 2008 were included in a retrospective study. Data were collected by means of diagnosis codes and search terms for TBI in patient records. The incidence of TBI in the different referral areas of the hospital for standard, specialised and intensive patient care was estimated. RESULTS: 472 patients met the inclusion criteria. The severity of the Injury was classified as mild in 342 patients, moderate in 50 patients and severe in 80 patients. The total incidence of traumatic brain injury in the referral area of the Erasmus University Hospital was estimated at 113.9 young people per 100.000. The incidence for mild traumatic brain injury was estimated at 104.4 young people, for moderate 6.1 and for severe 3.4 young people per 100.000. CONCLUSION: The ratio for mild, moderate and severe traumatic brain injury in children and young people was 33.7-1.8-1.In the mild TBI group almost 17% of the patients reported sequelae. The finding that 42% of them had a normal brain CT scan at admission underwrites the necessity of careful follow up of children and young people with mild TBI.
