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.
Copyright © 2011 Elsevier B.V. All rights reserved.
MeSH terms
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Anti-Inflammatory Agents / administration & dosage
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Anti-Inflammatory Agents / therapeutic use
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Aquaporin 4 / immunology*
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Autoantibodies / analysis*
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Brain / pathology
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Facial Nerve Diseases / complications
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Facial Nerve Diseases / pathology
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Female
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Humans
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Injections, Intravenous
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Magnetic Resonance Imaging
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Methylprednisolone / administration & dosage
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Methylprednisolone / therapeutic use
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Middle Aged
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Muscle Weakness / complications
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Myelitis, Transverse / complications*
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Myelitis, Transverse / immunology*
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Neuromyelitis Optica / complications
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Neuromyelitis Optica / immunology
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Prednisolone / administration & dosage
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Prednisolone / therapeutic use
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Scleroderma, Systemic / etiology*
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Spinal Cord / pathology
Substances
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Anti-Inflammatory Agents
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Aquaporin 4
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Autoantibodies
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Prednisolone
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Methylprednisolone