Neuromyelitis optica preceded by hyperCKemia episode

N. Suzuki, T. Takahashi, M. Aoki, T. Misu, S. Konohana, T. Okumura, H. Takahashi, S. Kameya, K. Yamaki, T. Kumagai, K. Fujihara, Y. Itoyama

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BACKGROUND: Neuromyelitis optica (NMO) is a disease of the CNS characterized by severe optic neuritis and longitudinally extended transverse myelitis. Recent studies suggest that anti-aquaporin-4 (AQP4) antibodies, NMO-specific biomarkers, are pathogenic and target AQP4-expressing astrocytes in NMO, although an additional event (T-cell response or infection) should occur for anti-AQP4 antibodies and complements to pass through the blood-brain barrier and cause the CNS lesions. AQP4 is the major water channel in the CNS, but it is also expressed in fast-twitch skeletal muscle fibers. However, muscle diseases have not been described in NMO. METHODS: We retrospectively examined the serologic database of 733 cases of NMO with anti-AQP4 antibody at the Department of Neurology, Tohoku University School of Medicine. The serum samples were sent to our laboratory for testing anti-AQP4 antibody from around the country during the period from 2006 to 2009. RESULTS: We found 3 anti-AQP4 antibody-positive female patients (7, 34, and 67 years old) with NMO who had episodes of prominent hyperCKemia (12,520, 19,415, and 59,660 IU/L) with general fatigue some weeks before the onset of optic neuritis. HyperCKemia was transient without any treatment in all patients, but recurred once in one of them. CONCLUSIONS: These cases suggest that hyperCKemia may be involved in the pathogenesis of neuromyelitis optica (NMO) in a fraction of patients. The causes of transient hyperCKemia are unknown. Further studies are needed to know the frequency of hyperCKemia in NMO and clarify its pathogenic role.

元の言語English
ページ(範囲)1543-1545
ページ数3
ジャーナルNeurology
74
発行部数19
DOI
出版物ステータスPublished - 2010 5 11

ASJC Scopus subject areas

  • Clinical Neurology

フィンガープリント Neuromyelitis optica preceded by hyperCKemia episode' の研究トピックを掘り下げます。これらはともに一意のフィンガープリントを構成します。

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    Suzuki, N., Takahashi, T., Aoki, M., Misu, T., Konohana, S., Okumura, T., Takahashi, H., Kameya, S., Yamaki, K., Kumagai, T., Fujihara, K., & Itoyama, Y. (2010). Neuromyelitis optica preceded by hyperCKemia episode. Neurology, 74(19), 1543-1545. https://doi.org/10.1212/WNL.0b013e3181dd445b