[Neuroimaging of Langerhans cell histiocytosis in the central nervous system of children].
Langerhans cell histiocytosis (LCH) is a rare disease characterized by the accumulation within tissues of anomalous dendritic cells similar to Langerhans cells. The clinical presentation varies, ranging from the appearance of a single bone lesion to multisystemic involvement. Central nervous system (CNS) involvement, manifesting as diabetes insipidus secondary to pituitary involvement, has been known since the original description of the disease. Two types of CNS lesions are currently differentiated. The first, pseudotumoral lesions with infiltration by Langerhans cells, most commonly manifests as pituitary infiltration. The second, described more recently, consists of neurodegenerative lesions of the CNS associated with neurologic deterioration. This second type of lesion constitutes a complication of the disease; however, there is no consensus about the cause of this complication. Our objective was to describe the radiologic manifestations of LCH in the CNS in pediatric patients.
Servicio de Radiodiagnóstico, Hospital Universitario Mútua Terrassa, Terrassa, Barcelona, España; Servicio de Radiología Pediátrica, Hospital San Joan de Déu, Esplugues de Llobregat, Barcelona, España. Electronic address: mdelahozpolo@gmail.com.
M
M
De La Hoz Polo
Servicio de Radiología Pediátrica, Hospital San Joan de Déu, Esplugues de Llobregat, Barcelona, España.
M
M
Rebollo Polo
Servicio de Neurología Pediátrica, Hospital San Joan de Déu, Esplugues de Llobregat, Barcelona, España.
C
C
Fons Estupiña
Servicio de Radiología Pediátrica, Hospital San Joan de Déu, Esplugues de Llobregat, Barcelona, España.
J
J
Muchart López
Servicio de Oncología Pediátrica, Hospital San Joan de Déu, Esplugues de Llobregat, Barcelona, España.
O
O
Cruz Martinez
D000328:Adult; D002493:Central Nervous System Diseases / Q000000981:diagnostic imaging*; D002648:Child; D006646:Histiocytosis, Langerhans-Cell / Q000000981:diagnostic imaging*; D006801:Humans; D059906:Neuroimaging
Central nervous system; Diabetes insipidus; Diabetes insípida; Histiocitosis de células de Langerhans; Langerhans cell histiocytosis; Neurodegeneración; Neurodegeneration; Neuroimagen; Neuroimaging; Sistema nervioso central