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.