Efficacy of Intravenous Cyclophosphamide Therapy for Neuromyelitis Optica Spectrum Disorder

Hiroaki Yaguchi; Ken Sakushima; Ikuko Takahashi; Hiroaki Nishimura; Moemi Yashima-Yamada; Masakazu Nakamura; Kazufumi Tsuzaka; Yasunori Maruo; Toshiyuki Takahashi; Ichiro Yabe; Hidenao Sasaki

doi:10.2169/internalmedicine.52.7885

ORIGINAL ARTICLES

Efficacy of Intravenous Cyclophosphamide Therapy for Neuromyelitis Optica Spectrum Disorder

Hiroaki Yaguchi, Ken Sakushima, Ikuko Takahashi, Hiroaki Nishimura, Moemi Yashima-Yamada, Masakazu Nakamura, Kazufumi Tsuzaka, Yasunori Maruo, Toshiyuki Takahashi, Ichiro Yabe, Hidenao Sasaki

Author information

Hiroaki Yaguchi
Department of Neurology, Hokkaido University Graduate School of Medicine, Japan
Ken Sakushima
Department of Neurology, Hokkaido University Graduate School of Medicine, Japan
Ikuko Takahashi
Department of Neurology, Hokkaido University Graduate School of Medicine, Japan
Hiroaki Nishimura
Department of Neurology, Hokkaido University Graduate School of Medicine, Japan
Moemi Yashima-Yamada
Department of Neurology, Hokkaido University Graduate School of Medicine, Japan
Masakazu Nakamura
Department of Neurology, Hokkaido University Graduate School of Medicine, Japan
Kazufumi Tsuzaka
Department of Neurology, Kushiro Rosai Hospital, Japan
Yasunori Maruo
Department of Neurology, Hakodate Municipal Hospital, Japan
Toshiyuki Takahashi
Department of Neurology, Tohoku University Graduate School of Medicine, Japan
Department of Multiple Sclerosis Therapeutics, Tohoku University Graduate School of Medicine, Japan
Ichiro Yabe
Department of Neurology, Hokkaido University Graduate School of Medicine, Japan
Hidenao Sasaki
Department of Neurology, Hokkaido University Graduate School of Medicine, Japan

Keywords: neuromyelitis optica spectrum disorder, intravenous cyclophosphamide, anti-aquaporin 4 antibody, immunosuppressive therapy

JOURNAL OPEN ACCESS

2013 Volume 52 Issue 9 Pages 969-972

DOI https://doi.org/10.2169/internalmedicine.52.7885

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Abstract

Objective Neuromyelitis optica (NMO) is an inflammatory disease that affects the optic nerve and spinal cord. Optic neuritis and longitudinally extensive myelitis associated with systemic autoimmune disease have been recently defined as NMO spectrum disorder (NMOSD). In this study, we report the efficacy of intravenous cyclophosphamide (IVCY) therapy for NMOSD.
Methods Four patients diagnosed with NMOSD were enrolled in this study. The expanded disability status scale (EDSS) score was used to evaluate the degree of severity. All of the patients received intravenous methylprednisolone (IVMP; 1 g/day for three days), and two patients also received plasmapheresis (PP). All of the patients were administered IVCY treatment.
Results Anti-AQP4 antibodies were present in the sera of all patients. All patients exhibited longitudinally extensive transverse myelitis (LETM). Only one patient who fulfilled the criteria for a diagnosis of NMO exhibited optic neuritis. Two patients developed relapse under treatment with low-dose prednisolone (PSL) before the administration of IVCY. The patients in this study exhibited a median improvement in the EDSS score following IVCY treatment from 8.0 to 5.75. Adverse effects were observed in only one patient.
Conclusion This study, despite its retrospective design, demonstrated the therapeutic efficacy of IVCY for NMOSD in both the acute and chronic phases of the disease and determined the IVCY dosage for Japanese women with NMOSD. Additionally, this study provided evidence that for NMOSD patients with severe disabilities, IVCY added to IVMP and PP may be a useful therapeutic modality.

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