Neurodegenerative central nervous system disease as late sequelae of Langerhans cell histiocytosis. Report from the Japan LCH Study Group |
| |
Authors: | Imashuku Shinsaku,Shioda Yoko,Kobayashi Ryoji,Hosoi Gaku,Fujino Hisanori,Seto Shiro,Wakita Hisashi,Oka Akira,Okazaki Nagisa,Fujita Naoto,Minato Toshinori,Koike Kenichi,Tsunematsu Yukiko,Morimoto Akira Japan LCH Study Group |
| |
Affiliation: | Division of Pediatrics, Takasago-seibu Hospital, 1-10-41 Nakasuji, Takasago, Hyogo Prefecture, Japan 676-0812. shinim95@mbox.kyoto-inet.or.jp |
| |
Abstract: | Clinical features, brain magnetic resonance imaging findings and EDSS scores of 11 patients with neurodegenerative central nervous system Langerhans cell histiocytosis were analyzed in Japan. All patients initially had multi-system-type Langerhans cell histiocytosis; 8 at 1-2 years of age and 3 at a later age. Neurodegenerative central nervous system Langerhans cell histiocytosis disease developed after a median time interval of 3.9 years from initial diagnosis. With a median follow-up of 4.5 years, 6 patients showed progression of disease with an EDSS score >3. This study demonstrates the importance of early detection of neurodegenerative central nervous system Langerhans cell histiocytosis by brain magnetic resonance imaging, particularly in the follow-up of patients who developed multi-system-type Langerhans cell histiocytosis in early infancy. |
| |
Keywords: | |
本文献已被 PubMed 等数据库收录! |
|