Neurodegenerative central nervous system disease as late sequelae of Langerhans cell histiocytosis. Report from the Japan LCH Study Group.
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.
Imashuku
Shinsaku
S
Division of Pediatrics, Takasago-seibu Hospital, 1-10-41 Nakasuji, Takasago, Hyogo Prefecture, Japan 676-0812. shinim95@mbox.kyoto-inet.or.jp
Shioda
Yoko
Y
Kobayashi
Ryoji
R
Hosoi
Gaku
G
Fujino
Hisanori
H
Seto
Shiro
S
Wakita
Hisashi
H
Oka
Akira
A
Okazaki
Nagisa
N
Fujita
Naoto
N
Minato
Toshinori
T
Koike
Kenichi
K
Tsunematsu
Yukiko
Y
Morimoto
Akira
A
D000293:Adolescent; D017668:Age of Onset; D001921:Brain / Q000473:pathology*; D002531:Cerebellum / Q000473:pathology; D054022:Cerebrum / Q000473:pathology; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000453:epidemiology / Q000473:pathology; D006801:Humans; D007223:Infant; D007564:Japan / Q000453:epidemiology; D008279:Magnetic Resonance Imaging; D008297:Male; D019636:Neurodegenerative Diseases / Q000453:epidemiology / Q000209:etiology* / Q000473:pathology; D011149:Pons / Q000473:pathology; D012042:Registries / Q000706:statistics & numerical data; D012720:Severity of Illness Index