Confocal Corneal Microscopy in the Evaluation of Immune-related Motor Neuron Disease Syndrome

Background: To investigate the sensitivity and specificity of confocal corneal microscopy (CCM) in the diagnosis of immune-related motor neuron disease syndrome and evaluation of the response to immunosuppressive therapy. Methods: Seventy-two patients with clinical manifestations of motor neuron disease (MND) were analysed. According to whether they had concomitant rheumatic immune disease or rheumatic immune antibody abnormalities, they were divided into an MND group (33 patients) and an immune-related MND syndrome group (39 patients). Another 10 healthy adults were selected as the control group. All individuals were examined by CCM. Results: For Langerhans cell density (LCD), the area under the ROC curve was 0.8, the best cut-off was 67.7 cells/mm2, the sensitivity was 79.5%, and the specificity was 72.7%. For inferior whorl length (IWL), the area under the ROC curve was 0.674, the best cut-off was 17.41 mm/mm 2 , the sensitivity was 69.2%, and the specificity was 66.7%. After immunosuppressive therapy in 5 patients with immune-related MND syndrome, the LCD was significantly reduced (P0.05). Conclusion: The LCD and IWL are ideal for distinguishing MND from immune-related MND syndrome. The LCD reflects immunotherapy response sensitively.