Clinical and imaging characteristics of pediatric neuromyelitis optica spectrum disorders

Objective To analyze the clinical and imaging characteristics of pediatric neuromyelitis optica spectrum disorders(NMOSD) in children. Methods The clinical data, imaging manifestations and follow-up data of 16 NMOSD patients at Department of Pediatric Neurology, Shandong Provincial Hospital Affiliated to Shandong University between July 2013 and September 2017 were respectively analyzed. Results In 16 patients, initial presentations included optica neuritis(ON) in 5 cases, longitudinally extensive transverse myelitis(LETM) in 6 cases, and among them there were 2 cases with acute disseminated encephalomyelitis and 3 cases with both ON and LETM. Eleven cases received aquaporin-4(AQP4) antibody examination and 4 cases were found seropositive.One case out of 7 detected cases was found AQP4 antibody positive in cerebrospinal fluid.Eleven cases received optica magnetic resonance imaging (MRI), and 8 cases were found abnormal signals in optic nerve and optica chiasma.The spinal cord MRI showed 13 cases with LETM manifestations, and abnormal signals were found in vertebral segments(5-13), and among them 1 case had cervical cord, 3 cases were thoracic cord and 9 cases were both of the above.Lesions in the cervical cord in 2 cases were extended upward to the medulla.Fifteen cases received brain MRI and all of them had brain lesions, which were mainly involved in the central and subcortical white matter, thalamus, corpus callosum, brainstem, the junction of spinal cord and medulla, cerebellum, and so on.All patients received treatment for acute attacks with high-dose Methylprednisolone and/or gamma globulin and got obvious relief.Two cases with recurrent ON received treatment of Rituximab and their vision became improved.Fifteen patients were followed up, and 2 cases had limb disorders and 4 cases had visual impairment, other patients had no clinical symptoms. Conclusions Pediatric NMOSD has a diverse clinical pre-sentation at the onset disease.Those who are initial diagnosed acute myelitis, ON and acute disseminated encephalomyelitis should be considered the possibility of NMOSD.Antibody to AQP4 testing can assist the diagnosis.The typical imaging characters of NMOSD children are abnormal signals in the high expression area of AQP4.Intracranial lesions are more common in children.The acute treatment includes the high-dose Methylprednisolone and gamma globulin.Rituximab can be used for the recurrent patients. Key words: Neuromyelitis optica spectrum disorders; Aquaporin-4; Child