Extensive Brainstem Lesion and Cervical Myelopathy: Overcoming a Grim Prognosis - Case Report

Cognard-type V arteriovenous fistulas are rare forms of dural arteriovenous malformations. This specific subtype is characterised by spinal venous drainage into peri-medullary veins, which presents as progressive or ascending myelopathy in 50% of cases.

A 59-year-old man with 3 weeks of worsening quadriparesis, along with abnormal sensory findings, gait deterioration, and bowel and bladder dysfunction, was admitted to the hospital. MRI revealed extensive T2 hyper-intense signal changes in the brainstem and myelopathy in the cervical spinal cord extending down to C7. The differential diagnosis for extensive myelopathy and brainstem lesions seen on MRI included a tumour, autoimmune inflammation, and suspicion of vascular malformation. The administration of corticosteroids resulted in a worsening of the patient's neurological symptoms. Conventional angiography confirmed the presence of a dural AVF (a small arteriovenous short shunt receiving arterial supply from the middle meningeal and occipital artery) in the territory of the left external carotid artery. A minimally invasive endovascular treatment was successfully performed.

We present this case considering that only 100 patients with Cognard type V fistula have been reported to date. Additionally, we aim to highlight the importance of caution when administering corticosteroids in cases of diagnostic uncertainty.