Neurodegenerative central nervous system disease as late sequelae of Langerhans cell histiocytosis. Report from the Japan LCH Study Group
Year: 2008
Type: article
Study design: Observational Study
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.
Source: Haematologica
Authors: S. Imashuku, Y. Shioda, R. Kobayashi, G. Hosoi, H. Fujino, S. Seto, H. Wakita, A. Oka, N. Okazaki, N. Fujita, T. Minato, K. Koike, Y. Tsunematsu, A. Morimoto, the Japan LCH Study Group (JLSG)
Institutions: National Center For Child Health and Development, Hokkaido University, Osaka Prefectural Medical Center, Kishiwada City Hospital, Japanese Red Cross Narita Hospital, Sasebo City General Hospital, Hiroshima Red Cross Hospital & Atomic-bomb Survivors Hospital, Toyooka Hospital, Shinshu University, Kyoto Prefectural University of Medicine
Language: English
Subfield: Hematology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $2,157
OpenAlex ID: W2050158629