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Front Neurol


Case Report: Diagnosis of Petrous Apex IgG4-Related Disease by Middle Cranial Fossa Craniotomy and Temporal Bone Biopsy.


Hofmeyr L, Herbst G, Pretorius E, Sarembock B, Taylor K, Roytowski D
Front Neurol. 2022; 13:874451.
PMID: 35756934.


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.