Department of Otolaryngology-Head and Neck Surgery, University of Illinois at Chicago School of Medicine, Chicago, IL; and.
Departments of Otolaryngology-Head and Neck Surgery and.
J Clin Neuromuscul Dis. 2022 Sep 1;24(1):55-58. doi: 10.1097/CND.0000000000000402.
Facial onset sensory and motor neuronopathy syndrome (FOSMN) is a rare motor neuron disorder characterized by facial sensory and motor aberrations that progress to the upper limbs. We present a case of FOSMN-like syndrome that has characteristics of FOSMN but is confined to the craniofacial region.
Retrospective chart review and review of the literature.
A 70-year-old woman presented with a 1-month history of progressive bilateral facial sensory loss and weakness affecting the trigeminal and hypoglossal nerves. Within 12 months, she developed debilitating weakness affecting her lower and midface bilaterally. After an extensive workup, a diagnosis of FOSMN-like syndrome was made, as symptoms failed to progress to the upper extremities.
This case demonstrates a unique presentation of FOSMN that we classify as FOSMN-like syndrome. Clinicians must maintain a high index of suspicion when a patient presents with clinical features characteristic of FOSMN syndrome without progression of symptoms distal to the craniofacial region because it may represent a FOSMN-like syndrome.
面部起始感觉运动神经元病综合征（FOSMN）是一种罕见的运动神经元疾病，其特征为面部感觉和运动异常，逐渐发展至上肢。我们报告了一例具有 FOSMN 特征但局限于头面部的 FOSMN 样综合征病例。
回顾性病历审查和文献复习。
一名 70 岁女性出现渐进性双侧面部感觉丧失和累及三叉神经和舌下神经的无力症状，病史 1 个月。12 个月内，她出现双侧下和中面部的严重无力。经过广泛的检查，诊断为 FOSMN 样综合征，因为症状没有进展到上肢。
本病例表明 FOSMN 具有独特的表现，我们将其归类为 FOSMN 样综合征。当患者出现符合 FOSMN 综合征的临床特征且症状没有向头面部以外的部位进展时，临床医生必须保持高度怀疑，因为这可能代表 FOSMN 样综合征。
