Most sources describe LETM as a rare and serious inflammatory process that expands over more than three vertebrae, (5-10) though some have described it as more than four.
3 of mean age year at LETM, respectively), more likely to present with a classic Devic phenotype, and more likely to have conus involvement and early urinary retention.
The outcome depends on whether it is inflammatory LETM with good prognosis.
At this point, the evaluation of a patient with LETM essentially relies on the MRI of the brain and spinal cord where a hyperintense lesions are seen on T2-weighted images associated with rheumatological work-up (vasculitis in particular), CSF analysis as well as more specific antibodies, such as NMO-IgG, soluble IL-2R, anti-ENA antibodies, and onconeuronal antibodies (5); novel inflammatory markers, such as IL-6 or other proteins in their signaling pathways, may represent markers of disease severity and potential therapeutic targets.
Management of LETM should be tailored to the underlying process.