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Longitudinally extensive transverse myelitis
W Oliver Tobin1, Brian G Weinshenker, Claudia F Lucchinetti
1Department of Neurology, College of Medicine, Mayo Clinic, Rochester, Minnesota, USA.
Purpose Of Review:
Longitudinally extensive transverse myelitis (LETM) is a frequently devastating clinical syndrome which has come into focus for its association with neuromyelitis optica (NMO). Recent advances in the diagnosis of NMO have led to very sensitive and specific tests and advances in therapy for this disorder. LETM is not pathognomonic of NMO, therefore it is important to investigate for other causes of myelopathy in these patients. This review aims to discuss recent advances in NMO diagnosis and treatment, and to discuss the differential diagnosis in patients presenting with LETM.
Recent Findings:
Fluorescence-activated cell sorting and cell binding assays for NMO-IgG are the most sensitive for detecting NMO spectrum disorders. Patients who have a clinical presentation of NMO, who have been tested with older ELISA or immunofluorescence assay and been found to be negative, should be retested with a fluorescence-activated cell sorting assay when available, particularly in the presence of recurrent LETM. Novel therapeutic strategies for LETM in the context of NMO include eculizumab, which could be considered in patients with active disease who have failed azathioprine and rituximab. Thorough investigation of patients with LETM who are negative for NMO-IgG may lead to an alternate cause for myelopathy.
Summary:
LETM is a heterogeneous condition. Novel treatment strategies are available for NMO, but other causes need to be excluded in NMO-IgG-seronegative patients.
Insights
Longitudinally extensive transverse myelitis (LETM) is a heterogeneous condition. New diagnostic tests and treatments for neuromyelitis optica (NMO) are available, but other causes of LETM must be excluded in NMO-IgG-seronegative patients.
Area of Science:
- Neurology
- Immunology
Background:
- Longitudinally extensive transverse myelitis (LETM) is a severe neurological condition often associated with neuromyelitis optica (NMO).
- Advances in NMO diagnosis and therapy have emerged, yet LETM can stem from various causes.
- Investigating alternative etiologies for myelopathy in LETM patients is crucial.
Purpose of the Study:
- To review recent advancements in the diagnosis and treatment of NMO.
- To discuss the differential diagnosis of LETM, particularly in NMO-IgG-seronegative cases.
Main Methods:
- Review of current literature on NMO and LETM.
- Analysis of diagnostic assays for NMO-IgG, including fluorescence-activated cell sorting (FACS).
- Evaluation of therapeutic strategies for NMO spectrum disorders.
Main Results:
- FACS and cell binding assays offer the highest sensitivity for detecting NMO spectrum disorders.
- Patients with suspected NMO and negative results from older assays should be retested using FACS.
- Eculizumab is a potential novel therapy for active NMO, especially after failure of azathioprine and rituximab.
Conclusions:
- LETM represents a diverse group of conditions.
- While NMO has new treatment options, identifying alternative causes is essential for NMO-IgG-seronegative patients.
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