Quadriplegia in a Child With Spinal Arteriovenous Metameric Syndrome.

A 6-year-old boy experienced sudden-onset neck pain followed by rapidly progressive weakness in all 4 extremities. Physical examination demonstrated port-wine stains over the nuchal area (Figure 1A). Neurologically, the patient was quadriplegic, lacking deep tendon and cremasteric reflexes, with a sensory level corresponding to C4 segment. Spinal MRI revealed a large extradural-intradural AVM lesion associated with hematomyelia in the cervical spine (Figure 1B), better depicted by catheter angiography (Figure 1C). Based on the data obtained thus far, a diagnosis of spinal arteriovenous metameric syndrome (SAMS) was made. After starting prednisolone therapy followed by endovascular embolization, the quadriparesis improved, and physiotherapy helped the patient regain full strength a month later.