难治性颅底假单胞菌骨髓炎伴格拉登尼戈综合征:早期吞咽困难和晚期视神经麻痹

F. Mammarella, A. Loperfido, Gianluca Velletrani, F. Casorati, Alessandro Stasolla, Stefano Di Girolamo, G. Bellocchi
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摘要

格拉登尼戈综合征(GS)是一种罕见的疾病,以中耳炎、三叉神经分布区疼痛和外展神经麻痹为特征。典型的三联征并不常见,因此诊断工作极具挑战性。具体来说,诊断方法包括病史、全面的耳鼻喉科检查、纯音听力图和放射学检查,如造影剂增强计算机断层扫描和头颈部磁共振成像。广谱抗生素是一线治疗药物,如静脉注射头孢曲松和甲硝唑。在此,我们介绍一例 71 岁的男性病例,他既往有中耳炎病史,且 2 型糖尿病控制不佳。他因面部疼痛、左侧咽喉麻痹、吞咽困难和耳痛就诊。患者接受了广谱抗生素治疗,但临床症状未见好转。影像学检查显示,病变部位宽大且界限不清,病变中心位于咀嚼间隙,并累及附近所有结构。患者接受了多次活检,但未获得肿瘤的明确组织诊断。2 个月后,患者出现延迟性 VI 颅神经麻痹,为 GS 提供了证据。尽管不完全,文献中也有 GS 的描述,但没有一个病例表现出潜伏性外展神经缺损。据我们所知,这是唯一一例延迟出现的外展神经麻痹病例。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Refractory Pseudomonas Osteomyelitis of the Skull Base With Gradenigo’s Syndrome: Early Dysphagia and Late Abducens Nerve Palsy
Gradenigo’s syndrome (GS) is a rare entity characterized by otitis media, pain in the trigeminal nerve distribution and abducens nerve palsy. The classic triad is uncommon, making the diagnostic workup challenging. Specifically, the diagnostic approach includes medical history, a complete otorhinolaryngological examination, a pure-tone audiogram and radiological investigation such as contrast-enhanced computed tomography scan and magnetic resonance imaging of head and neck. Broad-spectrum antibiotics are the first-line treatment, such as intravenous (IV) ceftriaxone and IV metronidazole. Here, we present the case of a 71-year-old man with a previous history of otitis media and poorly controlled type 2 diabetes mellitus. He presented to our attention with facial pain, left hemilarynx paralysis, dysphagia and otorrhea. The patient was treated with broad-spectrum antibiotics without any clinical improvement. Imaging evaluations demonstrated the presence of wide and poorly defined pathological material with epicenter in the masticatory space, involving all nearby structures. The patient underwent multiple biopsies without obtaining a definitive tissue diagnosis of neoplasia. After 2 months, the patient developed delayed VI cranial nerve palsy, providing evidence of GS. Although incomplete, GS has been described in the literature; however, none of the cases exhibited a latent abducent deficit. To the best of our knowledge, this is the only case with a delayed onset of abducens nerve palsy.
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