Clinical presentations of neuromyelitis optica spectrum disorders with ultra-longitudinally extensive transverse myelitis

• Published in: Zhong hua yi xue za zhi Vol. 98; no. 21; p. 1658
• Main Authors: Huang, X, Xu, F F, Qian, H R, Lei, X, Qi, X K, Xia, D Y
• Format: Journal Article
• Language: Chinese
• Published: China 05-06-2018
• Subjects: Adult; Aquaporin 4; Autoantibodies; Female; Humans; Male; Myelitis, Transverse; Neoplasm Recurrence, Local; Neuromyelitis Optica; Retrospective Studies; Recurrence; Aquaporin-4 antibody; Neuromyelitis optica spectrum disorders
• ISSN: 0376-2491
• DOI: 10.3760/cma.j.issn.0376-2491.2018.21.008

To analyze the clinical presentations of neuromyelitis optica spectrum disorders (NMOSD) with ultra-longitudinally extensive transverse myelitis (uLETM), in order to improve the diagnostic accuracy of this disorder.
Twenty-two uLETM patients was recruited and retrospectively analyzed for general clinical characteristics, laboratory tests and MRI characteristics, as well as therapeutic.
(1)The Male-to-female ratio was 1∶6. The median onset age was 31 years old. The duration from the first relapse to the onset was 5.5 months. (2)The positive rate of serum water channel aquaporin-4 antibody (AQP4-Ab) in the acute phase was 86.4%. The positive rate of cerebrospinal fluid (CSF) AQP4-Ab in the acute phase was 69.2%. The positive rate of autoimmune antibodies was 72.7%. There was a remarkable difference (
=-12.632,
=0.000) in serum AQP4-Ab titer levels between with the acute and remission period (median titer of 1∶244.78 to 1∶139.63). There was a remarkable difference (
=-20.161,
=0.000) in geometric mean of serum A