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

doi:10.1016/j.jns.2011.01.011

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 journal › Article › peer-review

Abstract

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 language	English
Pages (from-to)	139-141
Number of pages	3
Journal	Journal of the Neurological Sciences
Volume	303
Issue number	1-2
DOIs	https://doi.org/10.1016/j.jns.2011.01.011
Publication status	Published - Apr 15 2011

Keywords

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

ASJC Scopus subject areas

Clinical Neurology
Neurology

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10.1016/j.jns.2011.01.011

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title = "Systemic sclerosis in aquaporin-4 antibody-positive longitudinally extensive transverse myelitis",

abstract = "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.",

keywords = "Anti-aquaporin-4 antibody, NMO-IgG, Systemic sclerosis, Transverse myelitis",

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

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AU - Franciotta, Diego

AU - Zardini, Elisabetta

AU - Caporali, Roberto

AU - Piccolo, Laura

AU - Alberici, Elisa

AU - Romani, Alfredo

AU - Bergamaschi, Roberto

AU - Marchioni, Enrico

AU - Ceroni, Mauro

AU - Piccolo, Giovanni

PY - 2011/4/15

Y1 - 2011/4/15

N2 - 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.

AB - 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.

KW - Anti-aquaporin-4 antibody

KW - NMO-IgG

KW - Systemic sclerosis

KW - Transverse myelitis

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

Abstract

Keywords

ASJC Scopus subject areas

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