MRI features of neurodegenerative Langerhans cell histiocytosis

• Published in: European radiology Vol. 16; no. 9; pp. 2074 - 2082
• Main Authors: Martin-Duverneuil, N, Idbaih, A, Hoang-Xuan, K, Donadieu, J, Genereau, T, Guillevin, R, Chiras, J
• Format: Journal Article
• Language: English
• Published: Germany Springer Nature B.V 01-09-2006
• Subjects: Adolescent; Adult; Aged; Ataxia; Atrophy; Autoimmune diseases; Cerebellar ataxia; Cerebellum; Child; Child, Preschool; Corpus callosum; Diagnosis, Differential; Female; Globus pallidus; Histiocytosis; Histiocytosis, Langerhans-Cell - diagnosis; Humans; Infant; Lesions; Magnetic Resonance Imaging; Male; Middle Aged; Neurodegenerative Diseases - complications; Neurodegenerative Diseases - diagnosis; Patients; Pyramidal tracts; Retrospective Studies; Substantia alba; Tegmentum
• ISSN: 0938-7994; 1432-1084
• DOI: 10.1007/s00330-006-0200-y

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.