Mild encephalitis/encephalopathy with a reversible splenial lesion in children

PURPOSE:We aimed to present clinical and radiologic characteristics of mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) in children.METHODS:Eight children (5 boys and 3 girls; median age, 5.9 years; age range, 8 months to 14.1 years) diagnosed with MERS between September 2015 and June 2017 were included in the study. We reviewed the patient’s data, including demographic characteristics, prodromal and neurologic symptoms, neurologic examination, magnetic resonance imaging and electroencephalography findings, laboratory findings, treatment, and prognosis.RESULTS:Prodromal symptoms were nausea and vomiting (n=6), diarrhea (n=6), and fever (n=3). Initial neurologic symptoms were seizures (n=4), delirious behavior (n=1), drowsiness (n=1), ataxia (n=1), transient blindness (n=2), abnormal speech (n=2), and headache (n=1). Two patients had a suspected infective agent: urinary tract infection caused by Escherichia coli and gastroenteritis caused by rotavirus. Seven patients had type I lesions, comprising characteristic symmetric ovoid (n=6) and band-shaped (n=1) T2-weighted hyperintense lesions at the spenium of corpus callosum, and one patient had type II lesion with additional symmetric posterior periventricular lesions. The lesions were isointense to mildly hypointense on T1-weighted imaging and did not show enhancement. All lesions displayed restricted diffusion. In all patients, neurologic symptoms completely normalized <48 hours from the onset of symptoms without any sequelae.CONCLUSION:MERS has characteristic imaging features and favorable outcome

Clinical Characteristics and Visual Outcomes of Pediatric Optic Neuritis: A Single Center Experience

INTRODUCTION: The aim of this study was to describe the clinical characteristics, visual outcomes of pediatric patients presenting with first-episode of optic neuritis. METHODS: We reviewed medical records of the patients newly diagnosed with optic neuritis younger than 18 years between January 2014 and December 2018 retrospectively. RESULTS: Twenty-eight patients were included to this study. The mean age at first onset of optic neuritis was 13.2+-3.1 years (range 6.2-17.3 years). The mean follow-up period was 4.2+-3.2 (range 0.6-13.08) years. 7 of 28 (25%) patients had recurrent optic neuritis. Optic neuritis involvement was unilateral in 17 of 28 (60%) patients. Forty percent of the patients had idiopathic optic neuritis. Of the six patients with demyelinating lesions in cranial magnetic resonance imaging (MRI) at the first admission, three were diagnosed with multiple sclerosis (MS) at the time of first optic neuritis attack, and three were diagnosed within 13.4+-4.8 months after the first episode. Eight of 21 optic neuritis patients (38%) had oligoclonal band positivity and the incidence of MS was significantly higher in these patients (p=0.014). The mean visual acuity at nadir was 0.48+-0.27 at admission. Whereas it was 0.74+-0.31 and 0.76+-0.33 at 1 and 6 months respectively. There was a strong correlation between first and sixth-month visual acuity (r=0.98, p=0.00). DISCUSSION AND CONCLUSION: Our study demonstrated that poor visual acuity (worse than 0.5) at 1 month can predict poor vision at 6 months. The patients with demyelinating lesions in cranial MRI at their first optic neuritis episode, are more likely to develop MS during the follow-up

Role of serostatus in pediatric neuromyelitis optica spectrum disorders: A nationwide multicentric study

Background: Neuromyelitis optica spectrum disorders (NMOSD) are immune-mediated inflammatory disorders of the central nervous system (CNS) mostly presenting as optic neuritis and acute myelitis. NMOSD can be associated with seropositivity for aquaporin 4 antibody (AQP4 IgG), myelin oligodendrocyte glycoprotein antibody (MOG IgG), or can be seronegative for both. In this study, we retrospectively examined our seropositive and seronegative pediatric NMOSD patients. Method: Data were collected from all participating centres nationwide. Patients diagnosed with NMOSD were divided into three subgroups according to serology: AQP4 IgG NMOSD, MOG IgG NMOSD, and double seronegative (DN) NMOSD. Patients with at least six months of follow-up were compared statistically. Results: The study included 45 patients, 29 female and 16 male (ratio:1.8), mean age 15.16 ± 4.93 (range 5.5–27) years. Age at onset, clinical manifestations, and cerebrospinal fluid findings were similar between AQP4 IgG NMOSD (n = 17), MOG IgG NMOSD (n = 10), and DN NMOSD (n = 18) groups. A polyphasic course was more frequent in the AQP4 IgG and MOG IgG NMOSD groups than DN NMOSD (p = 0.007). The annualized relapse rate and rate of disability were similar between groups. Most common types of disability were related to optic pathway and spinal cord involvement. Rituximab in AQP4 IgG NMOSD, intravenous immunoglobulin in MOG IgG NMOSD, and azathioprine in DN NMOSD were usually preferred for maintenance treatment. Conclusion: In our series with a considerable number of double seronegatives, the three major serological groups of NMOSD were indistinguishable based on clinical and laboratory findings at initial presentation. Their outcome is similar in terms of disability, but seropositive patients should be more closely followed-up for relapses