Systemic sclerosis in aquaporin-4 antibody-positive longitudinally extensive transverse myelitis

Diego Franciotta, Elisabetta Zardini, Roberto Caporali, Laura Piccolo, Elisa Alberici, Alfredo Romani, Roberto Bergamaschi, Enrico Marchioni, Mauro Ceroni, Giovanni Piccolo

Research output: Contribution to journalArticlepeer-review


We report on the first patient with a relapsing, anti-aquaporin-4 (AQP-4) antibody-positive, longitudinally extensive transverse myelitis (LETM) who developed systemic sclerosis (SSc). A 62-year-old woman, who presented with bilateral, distal lower limb and perineal numbness, developed clinical manifestations and paraclinical features of SSc. Spinal cord imaging revealed lesions that were consistent with LETM. Patient's serum was positive for neuromyelitis optica (NMO)-IgG/AQP-4 antibodies. High-dose intravenous corticosteroids improved the neurological symptoms. The present case expands the list of autoimmune systemic diseases that occur in neuromyelitis optica spectrum disorders associated with NMO-IgG/AQP-4 antibodies.

Original languageEnglish
Pages (from-to)139-141
Number of pages3
JournalJournal of the Neurological Sciences
Issue number1-2
Publication statusPublished - Apr 15 2011


  • Anti-aquaporin-4 antibody
  • NMO-IgG
  • Systemic sclerosis
  • Transverse myelitis

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology


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