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  • Review Article
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Longitudinal extensive transverse myelitis—it's not all neuromyelitis optica

Nature Reviews Neurology volume 7, pages 688–698 (2011)Cite this article

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Abstract

Longitudinal extensive transverse myelitis (LETM) is defined as a spinal cord lesion that extends over three or more vertebrae, as seen on MRI of the spine. The clinical presentation of a patient with LETM is often dramatic and can consist of paraparesis or tetraparesis, sensory disturbances, and gait, bladder, bowel and/or sexual dysfunction. LETM is a characteristic feature of neuromyelitis optica, but such spinal lesions can also occur in various other autoimmune and inflammatory diseases that involve the CNS—such as multiple sclerosis, sarcoidosis or Sjögren syndrome—or in infectious diseases with CNS involvement. Patients with a neoplastic disorder or traumatic spinal cord injury can also present with longitudinal spinal lesions. In this Review, the signs and symptoms that suggest various etiologies and differential diagnoses of LETM are described, and illustrated by educational case studies. The best therapeutic options for patients with each diagnosis are also discussed.

Key Points

  • Longitudinal extensive transverse myelitis (LETM) is a rare but disabling condition, defined as a lesion of the spinal cord that extends over three or more vertebrae on MRI

  • A thorough work-up of patients with suspected LETM is essential to determine the underlying cause of the lesion

  • Evaluation of a patient with LETM should include MRI of the spinal cord and the brain, investigation of cerebrospinal fluid, and detection of cause-specific markers in the blood

  • Therapeutic strategies for patients with LETM will depend on the underlying cause of the lesion, and appropriate treatment should be initiated as early as possible

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Figure 1: MRI scans from a patient with Sjögren syndrome.
Figure 2: MRI scans from a patient with tuberculosis.
Figure 3: MRI scan from a patient with spinal cord lymphoma.
Figure 4: Radiological features of conditions associated with LETM.
Figure 5: MRI scans of the spinal cord lesion in a patient with anterior spinal artery infarction.
Figure 6: Karnaugh–Veitch diagram outlining the clinical parameters to be considered in the differential diagnosis of LETM.

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Acknowledgements

We thank V. Westphal for help with figure preparation. Written consent for publication was obtained from the patients described in the case studies or their responsible relatives.

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Authors and Affiliations

  1. Department of Neurology, Hannover Medical School, Carl-Neuberg-Strasse 1, Hannover, 30625, Germany

    Corinna Trebst, Elke Verena Voss & Martin Stangel

  2. Department of Neuroradiology, Hannover Medical School, Carl-Neuberg-Strasse 1, Hannover, 30625, Germany

    Peter Raab

  3. Department of Neurology, Medical University of Vienna, Waehringer Guertel 18–20, Vienna, 1090, Austria

    Paulus Rommer

  4. Department of Neurology, University of Rostock, Gehlsheimer Strasse 20, Rostock, 18147, Germany

    Mazen Abu-Mugheisib & Uwe K. Zettl

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All authors contributed to researching data for the article and made substantial contributions to discussions of the content. C. Trebst, P. Raab, E. V. Voss and M. Stangel wrote the article, and undertook review and/or editing of the manuscript before submission.

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Trebst, C., Raab, P., Voss, E. et al. Longitudinal extensive transverse myelitis—it's not all neuromyelitis optica. Nat Rev Neurol 7, 688–698 (2011). https://doi.org/10.1038/nrneurol.2011.176

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