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

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

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)2396-2402
Number of pages7
JournalJournal of Neurology
Volume260
Issue number9
DOIs
Publication statusPublished - Sep 2013

Keywords

  • Autoimmune diseases
  • MRI
  • Neuromyelitis optica spectrum disorders
  • Spinal cord

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

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