The clinical analyses of neuromyelitis optica spectrum disorder initially presenting with area postrema syndrome in 14 patients / 中华内科杂志

ABSTRACT

Objective To explore the clinical characteristics of the neuromyelitis optica spectrum disorders (NMOSD) with the area postrema syndrome as the initial symptom.Methods A total of 14 cases were enrolled in the study with the diagnose of NMOSD and the area postrema syndrome as the initial symptom.All the clinical data and imaging profiles by the contrasted magnetic resonance imaging (MRI) of the head and spinal cord were collected and analyzed.Results The median age of onset was (38.1 ± 17.0)years old and the gender ratio of female to male was 104.The serum aquaporin-4 (AQP4)-IgG was positive in 11 subjects and several autoimmune antibodies was positive in 7 subjects.The lesions revealed by MRI of the head mainly located in the area postrema and ependymal periphery which often presented as the linear medullary lesion,while linear lesions over three pieces of vertebra were shown by MRI of the spinal cord which mainly in the grey matter and with aH shape around the spinal central canal.Misdiagnose happened in 11 subjects with seven of gastroesophageal reflux disease,two of neurogenic vomiting,one of spinal cord tuberculosis and one of stroke.Conclusions NMOSD should be considered in patients with unexplained intractable nausea,vomiting and/or hiccups lasted for 48 hours or above,especially in those with positive nervous signs.Contrasted MRI and serum AQP4-IgG need to be performed in the suspected patients.Early detection is crucial for patients with NMOSD.