Original Paper Published online: November 23, 2013
Eur Neurol 2014;71:35–41 DOI: 10.1159/000353983
Clinical Manifestations and Spinal Cord Magnetic Resonance Imaging Findings in Chinese Neuromyelitis Optica Patients Aiyu Lin Jiting Zhu Xiaoping Yao Shifang Lin Shenxing Murong Zhiwen Li Department of Neurology, The First Affiliated Hospital of Fujian Medical University, Fuzhou, PR China
Key Words Neuromyelitis optica · Clinical manifestations · Magnetic resonance imaging · Anti-AQP4 antibody
0.001). The data revealed the clinical and MRI characteristics and AQP4 antibody status of NMO in Chinese patients and the correlations between them, which may have important implications for the diagnosis of the disease. © 2013 S. Karger AG, Basel
© 2013 S. Karger AG, Basel 0014–3022/14/0712–0035$39.50/0 E-Mail [email protected] www.karger.com/ene
Introduction
Neuromyelitis optica (NMO, also known as Devic’s disease) is an inflammatory syndrome of the central nervous system that is characterized by severe attacks of optic neuritis and myelitis [1]. Although NMO can present similarly to relapsing-remitting multiple sclerosis (MS), it has now been recognized as a disease entity distinct from MS in terms of both clinical and magnetic resonance imaging (MRI) manifestations. However, the exact pathophysiological basis for the unique clinical features of NMO and their relation to MRI findings in the spinal cord remains poorly understood [2, 3]. In the recent years, serum NMO-immunoglobulin G (IgG) against aquaporin-4 (AQP4) protein has been identified as a highly specific biomarker and part of the criteria for diagnosing NMO and excluding MS [4–11]. However, the sensitivity and specificity for the clinical diagno-
A.L. and J.Z. contributed equally to this work.
Dr. Aiyu Lin or Zhiwen Li Department of Neurology The First Affiliated Hospital of Fujian Medical University Fuzhou 350005 (PR China) E-Mail aiyulina @ yahoo.cn or c1985929 @ 126.com
Downloaded by: Freie Universität Berlin 130.133.8.114 - 5/12/2015 11:54:37 AM
Abstract Neuromyelitis optica (NMO) is a disease distinct from multiple sclerosis in terms of clinical and magnetic resonance imaging (MRI) manifestations. Antibody to aquaporin-4 (AQP4) has been identified as a specific biomarker and part of the diagnostic criteria for NMO. Although it is relatively common in Asia, a comprehensive clinical and imaging evaluation of NMO has not been reported in Chinese patients. Here, we reviewed data from 57 Chinese cases. The patients had an obvious female preponderance (female/male = 8.5: 1), and transverse myelitis (82.5%) and optic neuritis (56.1%) were the most common manifestations. In MRI, longitudinally extensive transverse myelitis (6.9 ± 2.3 segments) dominated the spinal cord lesions, which were mainly (69.7%) distributed in cervical and thoracic cord. However, the length of the lesions was not correlated with onset age, paralysis severity, relapse rate, or duration. Among 29 patients who underwent AQP4 antibody assay, 17 (58.6%) were positive. There was no difference between seropositive and seronegative patients in terms of female preponderance, onset age, relapse rate, and Expanded Disability Status Scale score. However, seropositive patients had significantly more damaged segments (8.3 ± 3.5) than did seronegative patients (4.5 ± 1.6) (p
Eur Neurol 2014;71:35–41 DOI: 10.1159/000353983
Clinical Manifestations and Spinal Cord Magnetic Resonance Imaging Findings in Chinese Neuromyelitis Optica Patients Aiyu Lin Jiting Zhu Xiaoping Yao Shifang Lin Shenxing Murong Zhiwen Li Department of Neurology, The First Affiliated Hospital of Fujian Medical University, Fuzhou, PR China
Key Words Neuromyelitis optica · Clinical manifestations · Magnetic resonance imaging · Anti-AQP4 antibody
0.001). The data revealed the clinical and MRI characteristics and AQP4 antibody status of NMO in Chinese patients and the correlations between them, which may have important implications for the diagnosis of the disease. © 2013 S. Karger AG, Basel
© 2013 S. Karger AG, Basel 0014–3022/14/0712–0035$39.50/0 E-Mail [email protected] www.karger.com/ene
Introduction
Neuromyelitis optica (NMO, also known as Devic’s disease) is an inflammatory syndrome of the central nervous system that is characterized by severe attacks of optic neuritis and myelitis [1]. Although NMO can present similarly to relapsing-remitting multiple sclerosis (MS), it has now been recognized as a disease entity distinct from MS in terms of both clinical and magnetic resonance imaging (MRI) manifestations. However, the exact pathophysiological basis for the unique clinical features of NMO and their relation to MRI findings in the spinal cord remains poorly understood [2, 3]. In the recent years, serum NMO-immunoglobulin G (IgG) against aquaporin-4 (AQP4) protein has been identified as a highly specific biomarker and part of the criteria for diagnosing NMO and excluding MS [4–11]. However, the sensitivity and specificity for the clinical diagno-
A.L. and J.Z. contributed equally to this work.
Dr. Aiyu Lin or Zhiwen Li Department of Neurology The First Affiliated Hospital of Fujian Medical University Fuzhou 350005 (PR China) E-Mail aiyulina @ yahoo.cn or c1985929 @ 126.com
Downloaded by: Freie Universität Berlin 130.133.8.114 - 5/12/2015 11:54:37 AM
Abstract Neuromyelitis optica (NMO) is a disease distinct from multiple sclerosis in terms of clinical and magnetic resonance imaging (MRI) manifestations. Antibody to aquaporin-4 (AQP4) has been identified as a specific biomarker and part of the diagnostic criteria for NMO. Although it is relatively common in Asia, a comprehensive clinical and imaging evaluation of NMO has not been reported in Chinese patients. Here, we reviewed data from 57 Chinese cases. The patients had an obvious female preponderance (female/male = 8.5: 1), and transverse myelitis (82.5%) and optic neuritis (56.1%) were the most common manifestations. In MRI, longitudinally extensive transverse myelitis (6.9 ± 2.3 segments) dominated the spinal cord lesions, which were mainly (69.7%) distributed in cervical and thoracic cord. However, the length of the lesions was not correlated with onset age, paralysis severity, relapse rate, or duration. Among 29 patients who underwent AQP4 antibody assay, 17 (58.6%) were positive. There was no difference between seropositive and seronegative patients in terms of female preponderance, onset age, relapse rate, and Expanded Disability Status Scale score. However, seropositive patients had significantly more damaged segments (8.3 ± 3.5) than did seronegative patients (4.5 ± 1.6) (p
