Hofmeyr Louis, Herbst Gerrida, Pretorius Elias, Sarembock Brian, Taylor Kathryn, Roytowski David
Netcare Christiaan Barnard Memorial Hospital, Cape Town, South Africa.
Division of Otorhinolaryngology, Tygerberg Academic Hospital, University of Stellenbosch, Cape Town, South Africa.
Front Neurol. 2022 Jun 9;13:874451. doi: 10.3389/fneur.2022.874451. eCollection 2022.
Primary IgG4-related disease (IgG4-RD) of the temporal bone is a rare condition. Unlike typical petrous apicitis or Gradenigo syndrome, our patient presented exclusively with unilateral cranial nerve VI palsy and symptoms of diplopia. Skull base imaging demonstrated a destructive bony lesion in the petrous apex. Imaging and systemic investigations were insufficient to support a diagnosis. The diagnosis was achieved histologically after acquiring the specimen by middle cranial fossa craniotomy and temporal bone biopsy. This case report is thought to be the first published description of a diagnosis of IgG4-RD proven with the middle cranial fossa approach.
We describe a 29-year-old female with primary IgG4-RD of the petrous apex of the temporal bone. This patient presented with a few-month history of left-sided headache and recent-onset diplopia due to paralysis of cranial nerve VI. Imaging demonstrated a petrous apex lesion, and comprehensive systemic investigations could not reach a diagnosis. A middle cranial fossa craniotomy and a biopsy of the temporal bone lesion were undertaken to establish the diagnosis. Histological confirmation of IgG4-RD was proven. Following treatment with corticosteroids, the patient experienced complete recovery and resolution of her symptoms.
This study describes a case of primary IgG4-RD of the petrous apex of the temporal bone that presented with diplopia and was diagnosed by middle fossa craniotomy and temporal bone biopsy. To the best of our knowledge, this is the first case description where primary diagnosis was made based on middle cranial fossa craniotomy and temporal bone biopsy.
颞骨原发性IgG4相关疾病（IgG4-RD）是一种罕见病症。与典型的岩尖炎或Gradenigo综合征不同，我们的患者仅表现为单侧展神经麻痹和复视症状。颅底影像学检查显示岩尖有骨质破坏病变。影像学和全身检查不足以支持诊断。通过中颅窝开颅术获取标本并进行颞骨活检后，经组织学确诊。本病例报告被认为是首次发表的经中颅窝入路证实诊断为IgG4-RD的描述。
我们描述了一名29岁患有颞骨岩尖原发性IgG4-RD的女性。该患者有几个月的左侧头痛病史，近期因展神经麻痹出现复视。影像学检查显示岩尖病变，全面的全身检查未能明确诊断。进行了中颅窝开颅术和颞骨病变活检以明确诊断。组织学证实为IgG4-RD。经皮质类固醇治疗后，患者症状完全缓解。
本研究描述了一例以复视为表现的颞骨岩尖原发性IgG4-RD病例，通过中颅窝开颅术和颞骨活检确诊。据我们所知，这是首例基于中颅窝开颅术和颞骨活检做出原发性诊断的病例描述。
