To describe the first reported case of a patient with spinal dural arteriovenous fistula (sDAVF) and “the missing-piece sign” located in the cervical spinal cord. To emphasize the importance that recognizing “the missing-piece sign” can potentially prevent delay in diagnoses and improve outcomes in patients with sDAVF.

The differential diagnosis for longitudinally extensive transverse myelitis (LETM) is very broad. Corticosteroids are a standard treatment for LETM but can worsen symptoms in sDAVF. Delay in diagnosis of sDAVF is associated with poor prognosis. “The missing-piece sign” is a radiographic feature of sDAVF that may facilitate an earlier diagnosis. A retrospective review of 44 patients with intraparenchymal contrast enhancement revealed that 19 (43%) displayed “the missing-piece sign”. The proposed mechanism is due to inconsistency of the spinal cord’s venous system leading to abrupt segments without enhancement having better venous outflow.

An 85-year-old female presented to the hospital with a stuttering course of progressive lower extremity weakness over the past year. She started using a cane a few months prior to presentation. MRI showed a contrast-enhancing lesion involving the brainstem, cervical cord and cervicothoracic junction. There was an area of abrupt non-enhancement in the cervical cord, similar in appearance to what has been termed “the missing-piece sign”. MRA of the spine showed serpiginous vessels. Digital Cerebral Angiography (DCA) showed no sDAVF. She received intravenous corticosteroids and her weakness worsened. She had a delayed repeat DCA, which found a C6 sDAVF. She had obliteration of the sDAVF one year after initial presentation. She is now wheelchair bound.

“The missing-piece sign” can occur in the cervical spinal cord. This sign is an important radiographic feature, in addition to vascular flow voids, to assist with prompt diagnosis, prevent harmful treatments or unnecessary biopsy, and improve outcomes for patients with sDAVF.