Lupus Presenting as Transverse Myelitis

Thank you to Dr. Cobb for presenting a very interesting case today of neuropsychiatric lupus presenting as transverse myelitis.

 T2 weighted MRI with evidence of transverse myelitis

T2 weighted MRI with evidence of transverse myelitis

Transverse myelitis is acute or subacute neurologic dysfunction of motor, sensory and autonomic nerves within the spinal cord. According to the Journal of Neurology article from 2002, 80-94% have numbness, parasthesias or band-like dyesthesias often with a clearly defined sensory level. About 50% will lose all movement of their legs. And, nearly all have bladder dysfunction with incontinencne of inability to void. To make the diagnosis, there should be no evidence of cord compression and, there should be evidence of inflammation of the spinal cord either via CSF (pleocytosis, elevated IgG) or via gadolinium enhanced MRI. The key steps in evaluation are listed below.

TM evaluation.png

The prognosis is mixed with roughly 1/3 recovering without sequelae, 1/3 with moderate disability and 1/3 with severe disability. Notably, rapid the progression, back pain and spinal shock confer a worse prognosis.

The differential for tranverse myelitis is relatively broad. If it involves 3 or more vertebrae it is defined as longitudinally extensive transverse myelitis and has a slightly more narrow differential listed below from the Journal of Neurologic Clinics, 2013.

LETM ddx.png

Treatment involves glucocorticoids for the inflammation and managment of the underlying cause. For neuropsychiatric lupus presenting as transverse myelitis this may include azathioprine or cycophosphamide. According to the Annals of Rheumatic Diseases in 2010, severe or refractory cases, plasma exchange, IVIg and rituximab may be utilized.

For even more information on transverse myelitis, please see the blog from November 13, 2017 on neurosarcoidosis.