Greene Jacqueline J, Sadjadi Reza, Jowett Nate, Hadlock Tessa
Department of Head and Neck Surgery-Otolaryngology (JJG, NJ, TH), Massachusetts Eye and Ear Infirmary/Harvard Medical School, Boston; and Department of Neurology (RS), Massachusetts General Hospital, Boston.
Neurol Clin Pract. 2021 Oct;11(5):e654-e660. doi: 10.1212/CPJ.0000000000001020.
Slow-onset peripheral facial palsy is far less common than acute-onset peripheral facial palsy and necessitates diagnostic evaluation for benign or malignant tumors or other less common etiologies. In the rare scenario in which no clarifying etiology is discovered following long-term evaluation (no radiographic or hematologic abnormalities and an otherwise unremarkable evaluation), a diagnostic and management dilemma occurs. We present a series of patients with this possible new clinical entity: Facial palsy, Radiographic and Other Workup Negative (FROWN) and propose a management strategy for this diagnosis of exclusion.
A series of 3,849 patients presenting with facial palsy to a tertiary facial nerve center was retrospectively assessed to identify those with progressive loss of facial function over at least 1 month. Exclusion criteria were history, physical or hematologic findings indicative of known diseases associated with facial palsy, and radiographic studies demonstrating a benign or malignant tumor.
Patients with slow-onset facial palsy constituted 5% (190 patients) of the cohort and were ultimately diagnosed with either a benign or malignant neoplasm or other facial nerve pathology. Fourteen patients with slow-onset facial palsy remained without a diagnosis following long-term evaluation and serial imaging. Eleven patients underwent dynamic facial reanimation surgery and facial nerve and muscle biopsy, with no clear histopathologic diagnosis.
Patients with slow-onset facial palsy with negative radiographic and medical evaluations over several years may be characterized as having FROWN, an idiopathic and as yet poorly understood condition, which appears to be amenable to facial reanimation but requires further investigation as to its pathophysiology.
迟发性周围性面瘫远比急性周围性面瘫少见，需要对良性或恶性肿瘤或其他不太常见的病因进行诊断评估。在长期评估后未发现明确病因的罕见情况下（无影像学或血液学异常且其他评估无异常），就会出现诊断和治疗难题。我们报告了一系列患有这种可能的新临床实体的患者：面瘫、影像学及其他检查均为阴性（FROWN），并提出了针对这种排除性诊断的治疗策略。
回顾性评估了3849例到三级面神经中心就诊的面瘫患者，以确定那些面部功能至少持续1个月逐渐丧失的患者。排除标准为有提示与面瘫相关的已知疾病的病史、体格检查或血液学检查结果，以及显示良性或恶性肿瘤的影像学检查。
迟发性面瘫患者占队列的5%（190例患者），最终被诊断为良性或恶性肿瘤或其他面神经病变。14例迟发性面瘫患者经过长期评估和系列影像学检查后仍未确诊。11例患者接受了动态面部重建手术以及面神经和肌肉活检，但未得到明确的组织病理学诊断。
多年来影像学和医学评估均为阴性的迟发性面瘫患者可能被归类为患有FROWN，这是一种特发性且目前了解甚少的疾病，似乎适合进行面部重建，但需要对其病理生理学进行进一步研究。
