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Distinctive clinical and neuroimaging characteristics of longitudinally extensive transverse myelitis associated with aquaporin-4 autoantibodies

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Abstract

Longitudinally extensive transverse myelitis (LETM) is a characteristic feature of Neuromyelitis Optica (NMO), but it can also occur in several other inflammatory diseases of the central nervous system (CNS). An IgG autoantibody that binds to aquaporin-4 (AQP4), the predominant water channel of the CNS, is a reliable biomarker of the NMO spectrum disorders, and if detected predicts the recurrence of the myelitis. In this study, we compared the clinical and neuroimaging characteristics of AQP4-IgG+ and AQP4-IgG− LETM patients. Thirty-seven first-ever LETM patients were retrospectively evaluated and divided into two groups according to the presence of AQP4 autoantibodies. AQP4-IgG was detected in the serum and in the cerebrospinal fluid of sixteen patients. The female to male ratio was higher in AQP4-IgG+ patients. Intractable nausea and vomiting and paroxysmal tonic spasms often accompanied the LETM in AQP4-IgG+ patients. T2-weighted spinal cord MRI revealed that inflammatory lesions extending into the brainstem and involving the central grey matter occurred more frequently in AQP4-IgG+ LETM patients. Hypointense lesions on T1-weighted spinal cord MRI were detected more frequently in the seropositive group, and their presence correlated with attack severity. In conclusion, this study provides clinical and spinal cord neuroimaging clues that can help distinguishing AQP4-IgG+ LETM patients.

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Conflicts of interest

The authors declare that they have no conflict of interest.

Ethical standard

All human studies must state that they have been approved by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.

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Authors and Affiliations

  1. Institute of Neurology, Department of Geriatrics Neurosciences and Orthopedics, Catholic University, L. go Gemelli, 8 00168, Rome, Italy

    Raffaele Iorio, Valentina Damato, Massimiliano Mirabella, Amelia Evoli, Alessandro Marti, Domenico Plantone, Giovanni Frisullo & Anna Paola Batocchi

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  1. Raffaele Iorio
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  2. Valentina Damato
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  3. Massimiliano Mirabella
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  4. Amelia Evoli
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  5. Alessandro Marti
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  6. Domenico Plantone
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  7. Giovanni Frisullo
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  8. Anna Paola Batocchi
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Correspondence to Raffaele Iorio.

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Iorio, R., Damato, V., Mirabella, M. et al. Distinctive clinical and neuroimaging characteristics of longitudinally extensive transverse myelitis associated with aquaporin-4 autoantibodies. J Neurol 260, 2396–2402 (2013). https://doi.org/10.1007/s00415-013-6997-9

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  • DOI: https://doi.org/10.1007/s00415-013-6997-9

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