[P1-120] A Case of Neuromyelitis Optica Spectrum Disorder Preceded by HyperCKemia
[Introduction] Aquaporin-4 (AQP4) is known as the target of autoantibody produced by neuromyelitis optica (NMO) spectrum disorder patients. In addition to the central nervous system (CNS), AQP4 is also expressed in various non-CNS tissues, including skeletal muscle. Recently, several adult cases of myopathy / hyperCKemia related to neuromyelitis optica (NMO) spectrum disorder are reported, but pediatric cases are rare. Here, we report a pediatric case with NMO spectrum disorder who exhibited hyperCKemia before the onset of optic neuritis.
[Case presentation] A 12-year-old girl was admitted to a nearby hospital because of intractable vomiting. HyperCKemia was noticed on admission. She had become asymptomatic and discharged one month later, but HyperCKemia continued. 5 weeks after onset, she had lost her left visual acuity and transferred to our hospital. Anti-AQP4 antibody was positive. On magnetic resonance Imaging, her left optic nerve was enhanced with gadrinium and T2 high lesions were noticed in the spinal cord, brainstem and brain white matter. Diagnosis of NMO spectrum disorder was made. High-dose methylpredonisolone, oral predonisolone, plasma exchange, intravenous immunoglobulin were not effective to the left optic nerve lesion. On the other hand, CK become normal after first High-dose methylpredonisolone. Combination therapy with oral predonisolone and azathioprine was effective for relapse prevention.
[Conclusion] Intractable vomiting and/or HyperCKemia may be antecedent symptoms of NMO spectrum disorder.
[Case presentation] A 12-year-old girl was admitted to a nearby hospital because of intractable vomiting. HyperCKemia was noticed on admission. She had become asymptomatic and discharged one month later, but HyperCKemia continued. 5 weeks after onset, she had lost her left visual acuity and transferred to our hospital. Anti-AQP4 antibody was positive. On magnetic resonance Imaging, her left optic nerve was enhanced with gadrinium and T2 high lesions were noticed in the spinal cord, brainstem and brain white matter. Diagnosis of NMO spectrum disorder was made. High-dose methylpredonisolone, oral predonisolone, plasma exchange, intravenous immunoglobulin were not effective to the left optic nerve lesion. On the other hand, CK become normal after first High-dose methylpredonisolone. Combination therapy with oral predonisolone and azathioprine was effective for relapse prevention.
[Conclusion] Intractable vomiting and/or HyperCKemia may be antecedent symptoms of NMO spectrum disorder.