Pain in neuromyelitis optica and its effect on quality of life A cross-sectional study Y. Kanamori, PhD I. Nakashima, MD, PhD Y. Takai, MD S. Nishiyama, MD H. Kuroda, MD, PhD T. Takahashi, MD, PhD C. Kanaoka-Suzuki, MD T. Misu, MD, PhD K. Fujihara, MD, PhD Y. Itoyama, MD, PhD ABSTRACT Objective: To assess the features of pain and its impact on the health-related quality of life (HRQOL) in neuromyelitis optica (NMO). Methods: We analyzed 37 patients with NMO or NMO spectrum disorders seen at the Department of Neurology, Tohoku University Hospital, Sendai, Japan, during the period from November 2008 to February 2009. A total of 35 of them were aquaporin-4 antibody-positive. We used Short Form Brief Pain Inventory (BPI) to assess pain and Short Form 36-item (SF-36) health survey to evaluate the HRQOL. Fifty-one patients with multiple sclerosis (MS) were also studied for comparison. Results: Pain in NMO (83.8%) was far more common than in MS (47.1%). The Pain Severity Index score in BPI was significantly higher in NMO than in MS, and patients’ daily life assessed by BPI was highly interfered by pain in NMO as compared with MS. Pain involving the trunk and both legs was much more frequent in NMO than in MS. SF-36 scores in NMO were lower than MS, espe- cially in bodily pain. Conclusion: Our study showed that pain in NMO is more frequent and severe than in MS and that pain has a grave impact on NMO patients’ daily life and HRQOL. Therapy to relieve pain is ex- pected to improve their HRQOL. Neurology ® 2011;77:652–658 GLOSSARY AQP4 = aquaporin-4; BPI = Brief Pain Inventory; DSS = Disability Status Scale; EDSS = Expanded Disability Status Scale; GFAP = glial fibrillary acidic protein; HRQOL = health-related quality of life; MS = multiple sclerosis; NMO = neuromyelitis optica; RRMS = relapsing-remitting multiple sclerosis; SF-36 = Short Form 36-item health survey; SF-BPI = Short Form Brief Pain Inventory; SPMS = secondary progressive multiple sclerosis; VA = visual acuity. Neuromyelitis optica (NMO) is an inflammatory disease of the CNS characterized by severe optic neuritis and transverse myelitis. 1-5 Its relation to multiple sclerosis (MS) has long been controversial. Other features of NMO in comparison with MS include female preponderance, higher onset age, severe functional disability, mostly negative oligoclonal immunoglobulin G bands, and longitudinally extensive spinal cord lesions (3 vertebral segments). 4,6 Comparative neuropathologic studies in NMO and MS showed that extensive loss of aquaporin-4 (AQP4) and glial fibrillary acidic protein (GFAP) immunoreactivities, cavity formation, gray matter involvement, and perivascular deposition of immunoglobulins and complements are common in NMO. 7 Moreover, AQP4 antibody is specific to NMO 8,9 and pathogenic in experimental studies. 10-13 CSF GFAP levels are remarkably high during relapse in NMO but not in MS. 14,15 Therapy of NMO is also different from that of MS. 16 These findings strongly suggest that NMO is a disease entity distinct from MS. Pain is a common symptom in MS, 17,18 and health-related quality of life (HRQOL) is low 19 and related to pain in MS. 20,21 Though clinical observations strongly suggest that many patients with NMO have pain, pain has never been thoroughly investigated in NMO. From the Department of Neurology (Y.K., I.N., Y.T., S.N., H.K., T.T., C.K.-S., T.M., K.F., Y.I.), Tohoku University School of Medicine, Sendai; Tohoku University Hospital Emergency Center (H.K.), Sendai; Department of Neurology (T.T.), National Yonezawa Hospital, Yonezawa; Department of Multiple Sclerosis Therapeutics (T.M., K.F.), Tohoku University Graduate School of Medicine, Sendai; and National Center Hospital (Y.I.), National Center of Neurology and Psychiatry, Kodaira, Japan. Study funding: Supported by grants-in-aid for scientific research from the Ministry of Education, Culture, Sports, Science and Technology (20390241, 21790828, 22229008) and the Ministry of Health, Labor and Welfare of Japan. Disclosure: Author disclosures are provided at the end of the article. Address correspondence and reprint requests to Dr. Ichiro Nakashima, Department of Neurology, Tohoku University School of Medicine, 1-1 Seiryo- machi, Aoba-ku, Sendai 980-8574, Japan nakashima@med.tohoku.ac.jp 652 Copyright © 2011 by AAN Enterprises, Inc.