Transient fourth nerve palsy preceding cervical myelopathy due to carotid-cavernous fistula

Carotid-cavernous fistulas (CCFs) are rare intracranial vascular malformations. Among the various classifications available, the most recently proposed highlights the strong correlation between venous drainage pattern and clinical presentation. We present the case of a woman in her 70s with a history of transient palsy of the fourth cranial nerve who presented with subacute cervical myelopathy, which was caused by a CCF with venous drainage into the peribulbar and perimedullary plexus. Given this atypical presentation of CCF and the diagnostic challenges it poses, we conducted a comprehensive PubMed search looking for CCFs presenting with cervical myelopathy and our results confirmed their rarity and allowed us to identify clinical elements that may help clinicians diagnose and manage this potentially treatable condition.