To emphasize the significance of early detection of vascular malformations in patients presenting with LETM-like symptoms for prompt and effective intervention.

Longitudinally extensive transverse myelitis (LETM) is a spinal cord syndrome with inflammation and neurologic dysfunction that affects motor, sensory, and autonomic tracts. LETM is typically associated with neuromyelitis optica spectrum disorder (NMOSD), but it can also result from other conditions, including vascular abnormalities. We present three cases of LETM-like symptoms that were later attributed to vascular malformations.

We retrospectively reviewed the cases of three patients who presented with imaging features of LETM and were initially thought to have a demyelinating disorder [multiple sclerosis (MS), NMOSD]. The patients were treated with steroids and plasmapheresis empirically. When symptoms persisted and worsened, and work-up for demyelinating diseases was negative, repeat imaging revealed a possible vascular etiology.

Repeat MRI revealed spinal cord edema and possible flow voids extending over several vertebral segments and spinal angiogram was performed. Two patients were found to have spinal dural arteriovenous fistula (SDAVF), and one patient had a posterior fossa dural arteriovenous fistula. All three patients underwent surgery to correct the vascular malformations. After surgery, all patients reported a complete reversal of their LETM-like symptoms.

These three cases of LETM-like symptoms were initially attributed to demyelinating disorders but were later diagnosed as vascular malformations. Early recognition of vascular malformations can lead to proper treatment and prevent worsening of symptoms. In all three cases, surgery to correct the vascular malformation resulted in a complete reversal of symptoms. It is essential to consider vascular abnormalities in the differential diagnosis of LETM-like symptoms, especially in patients with slow disease progression, atypical presentations, negative workup or with poor treatment response.