Analysis Of Clinical Features Of Facial Onset Sensory Motor Neuronopathy Syndrome

Objective:To investigate the clinical characteristics,electrophysiological examination,treatment and prognosis of FOSMN syndrome.Methods:Clinical data of 3 patients with FOSMN syndrome admitted to the department of neurology of the Second Affiliated Hospital of Zhejiang University hospital from July 2014 to July 2018 were analyzed,including symptoms and signs,imaging examination(such as CT and MRI of the brain),electrophysiological examination(such as electromyography and blink reflex),laboratory examination,treatment and follow-up.Results:The 3 patients reported in this paper all started with trigeminal sensory disorder,Case l involved muscles of face,neck and pharynx,case 2 involved face,neck,tongue and upper muscles,case 3 presented involvement of facial and hypopharynx muscles.All the 3 patients showed abnormal blink reflex,prolonged latency of R1,R2 and R2 ',decreased amplitude of sensory nerve in the upper limbs,and improved clinical symptoms after immunotherapy during hospitalization.Conclusion:FOSMN syndrome(facial onset sensory motor neuronopathy syndrome)is rare.The clinical features are movement and sensory disorders of facial onset,progressive to pharynx,neck and upper limbs,with little involvement of lower limbs.Abnormal blink reflex and sensory nerve action potentials(SNAP)in the upper limbs can be detected in patients with FOSMN syndrome.Clinical symptoms may be improved with immunotherapy.