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.

Use of computed tomography and diffusion weighted imaging in children with ventricular shunt.

PURPOSE: To evaluate the indications, number, and imaging results of brain computed tomography (CT) and diffusion weighted imaging (DWI) in children with ventriculoperitoneal shunt, to estimate the radiation dose, and to evaluate the effectiveness of DWI. METHODS: This retrospectively study included 54 consecutive patients (boys/girls = 30/24, mean age, 3 ± 4.1 years) with shunt that were placed due to congenital abnormalities-hypoxic ischemic encephalopathy between January 2015 and March 2018. The presence of shunt-related complications (SRC) was assessed using clinical and neuroimaging findings, and the standard reference was accepted as the shunt revision. Size comparisons of ventricles were performed using Evans index and the frontal and occipital horn ratio, and each measurement made by the observers were compared using Bland-Altman analysis. A kappa coefficient and the intraclass correlation coefficient were calculated to assess the agreement between observers. RESULTS: The mean number of hospital admission, number of CT scans, and DWI were 5.8, 4.8, and 1.1, respectively per patient. A significant linear correlation was observed between hospital admission and CT scans (r = 0.288, p = 0.035). The number of CT scans and the cumulative effective dose per patient were higher in patients with SRC than in those without (p < 0.001). The mortality rate due to radiation-induced neoplasia has increased by 0.33% in the study period. The inter-observer agreement was perfect or substantial for the catheter visualization, assessment of the ventricular system on DWI, and for the image quality of DWI between observers (κ = 0.704-1, p ≤ 0.001). No significant difference was found between CT and DWI in the measurements of Evans index and the frontal and occipital horn ratio (p > 0.05). Inter-observer agreements between observers were almost perfect for the Evans index and the frontal and occipital horn ratio (ICC = 0.94-0.99, p < 0.001). CONCLUSIONS: An awareness of the use of CT in children is still inadequate and difficulties in the diagnosis of SRC probably cause the overuse of CT. DWI should be preferred in the diagnosis of SRC and the follow-up of patients. Otherwise, the increase in the prevalence of several diseases, particularly neoplasia, may be inevitable because of the over use of CT.

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.