Foramen magnum arteriovenous fistula presenting as transverse myelitis

Excerpt

A 63-year-old, otherwise healthy, man presented with sudden-onset spastic quadriparesis and excruciating cervical pain. Neurological examination revealed loss of superficial sensation below the C4 level and pyramidal signs on all limbs. MRI disclosed a longitudinal extensive cervical cord lesion, along with peri-medullary venous congestion (Fig. 1). Blood and cerebrospinal fluid analyses were unremarkable. The acute, severe clinical symptomatology and the extensive longitudinal T2 hyperintensity in the cervical spinal cord prompted clinicians to administer large doses of intravenous corticosteroids (i.e., methyl-prednizolone 1000 mg/day). Three days after steroid initiation, clinical worsening occurred presenting as complete quadriplegia. The Foix-Alajouanine-like syndrome was speculated, and further angiographic imaging was pursued. Digital subtraction angiography (DSA) indeed revealed a dural arteriovenous fistula (DAVF) at the foramen magnum (Fig. 2). Subsequent closure of the fistula with an embolic agent lead to mild improvement of the clinical symptoms and signs few days later, but in spite of that the patient remained severely disabled.

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