Idiopathic aquaporin-4 antibody negative longitudinally extensive transverse myelitis

Background: Longitudinally extensive transverse myelitis (LETM) is a characteristic manifestation of neuromyelitis optica (NMO). However, not all patients with LETM are positive for aquaporin-4 (AQP4) antibodies. We evaluated the characteristics of idiopathic isolated LETM negative for AQP4 antibodi...

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Bibliographic Details
Published in:Multiple sclerosis 2015-05, Vol.21 (6), p.710-717
Main Authors: Hyun, Jae-Won, Kim, Su-Hyun, Huh, So-Young, Kim, Woojun, Yun, Junglim, Joung, AeRan, Sato, Douglas Kazutoshi, Fujihara, Kazuo, Kim, Ho Jin
Format: Article
Language:English
Subjects:
Adolescent
Adult
Age of Onset
Aquaporin 4 - immunology
Autoantibodies - blood
Female
Glial Fibrillary Acidic Protein - cerebrospinal fluid
Humans
Longitudinal Studies
Male
Middle Aged
Myelin-Oligodendrocyte Glycoprotein - immunology
Myelitis, Transverse - blood
Myelitis, Transverse - cerebrospinal fluid
Myelitis, Transverse - classification
Myelitis, Transverse - physiopathology
Registries
Sex Factors
Young Adult
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Summary:Background: Longitudinally extensive transverse myelitis (LETM) is a characteristic manifestation of neuromyelitis optica (NMO). However, not all patients with LETM are positive for aquaporin-4 (AQP4) antibodies. We evaluated the characteristics of idiopathic isolated LETM negative for AQP4 antibodies. Methods: From the National Cancer Center registry of inflammatory diseases of the central nervous system, patients with LETM as an initial manifestation and follow-up for at least two years were enrolled. Their medical records and MRIs were reviewed retrospectively. AQP4 antibody was confirmed by three different validated methods at least three times. Cerebrospinal fluid (CSF) glial fibrillary acidic protein (GFAP) levels were measured to investigate astrocyte damage. Results: Among 108 patients with first-ever LETM, 55 were positive for AQP4 antibodies (P-LETM) and 53 were consistently negative. Of them, seven were later diagnosed with seronegative NMO, and four were positive for MOG antibodies. The remaining 42 patients (N-LETM) showed several features distinct from P-LETM: male predominance, older age of onset, milder clinical presentation, spinal cord confinement and absence of combined autoimmunity. CSF GFAP levels were not increased in N-LETM but were markedly elevated in P-LETM. Conclusions: Idiopathic isolated N-LETM is not that rare among first-ever LETM, and has many features distinct from P-LETM where astrocytic damage is evident.
ISSN:1352-4585
1477-0970
DOI:10.1177/1352458514551454