以嗜睡为主要临床表现的视神经脊髓炎谱系疾病分析OA

Objective To investigate the clinical features of patients with neuromyelitis optica spectrum disorders(NMOSD)mainly manifesting as somnolence,and to improve the awareness of this disease among clinicians.Methods A retro-spective analysis was performed for the medical records of nine patients with NMOSD who were admitted to Xuanwu Hospital,Capital Medical University,from May 2021 to May 2024 and had the main clinical manifestation of somnolence,including general information,cranial and/or spinal MRI findings,polysomnography(PSG)results,cerebrospinal fluid(CSF)routine parameters,aquaporin-4(AQP4)antibody,the level of orexin in CSF,treatment response,and prognosis.Results The age of onset in the nine patients ranged from 18 to 62 years,with a median of 30(24,47)years,and the male/female ratio was 1∶8.Among the nine patients,six had the initial symptom of somnolence,three developed somnolence after other symptoms,and five had the manifesta-tions of other diencephalic syndromes.All nine patients were tested for AQP4 in CSF and serum and were found positive for AQP4 antibody in CSF and/or serum.All nine patients had abnormal signals in both thalami and para-third ventricles on cranial MRI.Three patients underwent PSG and were found to have a shortened sleep latency and sleep architecture disorder,among whom one had the electrophysiological manifestation of narcolepsy and one had the manifestation of hypoventilation in severe sleep apnea.The level of orexin in CSF was measured for two patients,and the results showed that the level of orexin was lower than the normal level in both patients.All nine patients received glucocorticoid pulse therapy,and some patients were given intravenous immuno-globulin and/or subsequent sequential immunotherapy.All patients had a significant improvement in the symptom of somnolence at discharge.Conclusion In the core symptoms of NMOSD,acute diencephalic syndrome manifesting as somnolence is relatively rare in clinical practice and is easily misdiagnosed in the early stage,and the possibility of NMOSD should be considered for unex-plained somnolence.Imaging examination and AQP4 antibody detection are of significant value for the diagnosis of this disease,and sleep monitoring can provide an objective assessment of sleep status.Immunotherapy is the main treatment method for this dis-ease,and most patients have a good prognosis.