Rui Li1, Danli Lu1, Hao Li2, Yuge Wang1, Yaqing Shu1, Yanyu Chang1, Xiaobo Sun1, Zhengqi Lu1, Wei Qiu3,4, Zhi Yang5. 1. Department of Neurology, The Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China. 2. Department of Neurology, Maoming People's Hospital, 101 Weimin Road, Maonan District, Maoming, Guangdong, China. 3. Department of Neurology, The Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China. qiuwei120@vip.163.com. 4. Multiple Sclerosis Center, Department of Neurology, The Third Affiliated Hospital of Sun Yat-Sen University, No. 600 Tianhe Road, Guangzhou, Guangdong Province, 510630, China. qiuwei120@vip.163.com. 5. Department of Neurology, Maoming People's Hospital, 101 Weimin Road, Maonan District, Maoming, Guangdong, China. mmyangz@163.com.
Abstract
BACKGROUND: Early stage neuromyelitis optica spectrum disorders (NMOSD) with non-opticospinal manifestations as initial symptoms are easily misdiagnosed; however, data on the full symptom profile are limited. Moreover, the clinical characteristics and long-term outcomes of these patients remain unknown. We sought to analyze the clinical characteristics, imaging features, and long-term outcomes of NMOSD with non-opticospinal manifestations as initial symptoms. METHODS: We retrospectively included relevant patients from our center. Clinical, demographic, magnetic resonance imaging, treatment, and outcome data were compared according to the non-opticospinal vs. opticospinal initial symptoms. RESULTS: We identified 43 (9.13 %) patients with non-opticospinal initial symptoms among 471 patients with NMOSD. Of these, 88.37 % developed optic neuritis/myelitis during an average follow-up period of 6.33 years. All the non-opticospinal symptoms were brain/brainstem symptoms. Most of the symptoms and associated brain lesions were reversible. These patients had a younger onset age (P < 0.001), lower serum aquaporin-4 (AQP4) antibody titers (P = 0.030), and a lower Expanded Disability Status Scale (EDSS) score at onset (P < 0.001) and follow-up (P = 0.041) than NMOSD patients with opticospinal initial symptoms. In addition, EDSS scores reached 3.0 (indicating moderate disability) later than in patients with opticospinal initial symptoms (P = 0.028). CONCLUSIONS: Patients with NMOSD with non-opticospinal initial symptoms have a younger onset age, lower serum AQP4 antibody titers, and better clinical outcomes.
BACKGROUND: Early stage neuromyelitis optica spectrum disorders (NMOSD) with non-opticospinal manifestations as initial symptoms are easily misdiagnosed; however, data on the full symptom profile are limited. Moreover, the clinical characteristics and long-term outcomes of these patients remain unknown. We sought to analyze the clinical characteristics, imaging features, and long-term outcomes of NMOSD with non-opticospinal manifestations as initial symptoms. METHODS: We retrospectively included relevant patients from our center. Clinical, demographic, magnetic resonance imaging, treatment, and outcome data were compared according to the non-opticospinal vs. opticospinal initial symptoms. RESULTS: We identified 43 (9.13 %) patients with non-opticospinal initial symptoms among 471 patients with NMOSD. Of these, 88.37 % developed optic neuritis/myelitis during an average follow-up period of 6.33 years. All the non-opticospinal symptoms were brain/brainstem symptoms. Most of the symptoms and associated brain lesions were reversible. These patients had a younger onset age (P < 0.001), lower serum aquaporin-4 (AQP4) antibody titers (P = 0.030), and a lower Expanded Disability Status Scale (EDSS) score at onset (P < 0.001) and follow-up (P = 0.041) than NMOSD patients with opticospinal initial symptoms. In addition, EDSS scores reached 3.0 (indicating moderate disability) later than in patients with opticospinal initial symptoms (P = 0.028). CONCLUSIONS:Patients with NMOSD with non-opticospinal initial symptoms have a younger onset age, lower serum AQP4 antibody titers, and better clinical outcomes.
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