title: Facial onset sensory and motor neuronopathy.
abstract: Facial onset sensory and motor neuronopathy (FOSMN) is a recently defined slowly progressive motor neuron disorder. It is characterized by facial onset sensory abnormalities which may spread to the scalp, neck, upper trunk and extremities, followed by lower motor neuron deficits. Bulbar symptoms, such as dysarthria and dysphagia, muscle weakness, cramps and fasciculations, can present later in the course of the disease. We search the PubMed database for articles published in English from 2006 to 2016 using the term of "Facial onset sensory and motor neuronopathy". Reference lists of the identified articles were selected and reviewed. Only 38 cases of FOSMN have been reported in the Pubmed database since it was first reported in 2006. Typically, FOSMN present with slowly evolving numbness of the face followed by neck and arm weakness. Reduced or absent of corneal reflexes and blink reflex is the main pathognomonic features of FOSMN. In this review, we summarize the epidemiology, clinical presentation, auxiliary examination, and treatment of all the reported cases of FOSMN. Moreover, we discuss the pathogenesis of this rare disorder. In addition, we propose diagnostic criteria for FOSMN.
authors.affiliation: Department of Neurology, The Affiliated Hospital of Guizhou Medical University, Guiyang, 550004, Guizhou, China.
authors.forename: Qian
authors.initials: Q
authors.lastname: Zheng
authors.affiliation: Department of Neurology, The Affiliated Hospital of Guizhou Medical University, Guiyang, 550004, Guizhou, China.
authors.forename: Lan
authors.initials: L
authors.lastname: Chu
authors.affiliation: Department of Neurology, The Second Xiangya Hospital, Central South University, 139 Renmin Road, Changsha, 410011, Hunan, China.
authors.forename: Liming
authors.initials: L
authors.lastname: Tan
authors.affiliation: Department of Neurology, The Second Xiangya Hospital, Central South University, 139 Renmin Road, Changsha, 410011, Hunan, China. yyzhn@sina.com.
authors.forename: Hainan
authors.initials: H
authors.lastname: Zhang
mesh_terms: D016206:Databases, Bibliographic; D005145:Face / Q000294:innervation*; D006801:Humans; D016472:Motor Neuron Disease / Q000150:complications* / Q000473:pathology*; D014276:Trigeminal Nerve / Q000503:physiopathology*
keywords: ALS; Blink reflex; FOSMN; Sensory and motor neuronopathy; Trigeminal nerve