title: MRI features of neurodegenerative Langerhans cell histiocytosis.
abstract: CNS complications of LCH include "space occupying" lesions corresponding to histiocytic granulomas and "neurodegenerative" presentation (ND-LCH) characterized by a progressive cerebellar ataxia. Studies analyzing specifically the MRI presentation of ND-LCH are scarce. We present here the MRIs of 13 patients registered as isolated ND-LCH. Posterior fossa was involved in 12 patients (92%), showing a symmetrical T2 hyperintensity of the cerebellar white matter areas in seven cases with a circumscribed T1 hyperintensity of the dentate nuclei in five cases, definite hyperintense T2 areas in the adjacent pontine tegmentum white matter in nine cases associated with a hyperintensity of the pontine pyramidal tracts in four cases. A cerebellar atrophy was noted in eight cases. The supratentorial region was involved in 11 patients, showing T2 hyperintense lesions in the cerebral white matter in eight cases and a discrete symmetrical T1 hyperintense signal in the globus pallidus in eight patients. A diffuse cortical atrophy was present in three cases and a marked focal atrophy of the corpus callosum in three cases. This series allows us to establish a not previously reported evocative semeiologic MR presentation to precisely orientate to the diagnosis of the pure neurodegenerative form of LCH.
authors.affiliation: Department of Neuroradiology, GH Pitié-Salpêtrière, 47 Bd de l'Hôpital, 75013, Paris, France. nadine.martin-duverneuil@psl.ap-hop-paris.fr
authors.forename: N
authors.initials: N
authors.lastname: Martin-Duverneuil
authors.forename: A
authors.initials: A
authors.lastname: Idbaih
authors.forename: K
authors.initials: K
authors.lastname: Hoang-Xuan
authors.forename: J
authors.initials: J
authors.lastname: Donadieu
authors.forename: T
authors.initials: T
authors.lastname: Genereau
authors.forename: R
authors.initials: R
authors.lastname: Guillevin
authors.forename: J
authors.initials: J
authors.lastname: Chiras
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis*; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D019636:Neurodegenerative Diseases / Q000150:complications / Q000175:diagnosis*; D012189:Retrospective Studies