Authors: N MartinDuverneuil A Idbaih K HoangXuan J Donadieu T Genereau R Guillevin J Chiras French Langerhans Cell Histiocytosis Study Group
Publish Date: 2006/04/20
Volume: 16, Issue: 9, Pages: 2074-2082
Abstract
CNS complications of LCH include “space occupying” lesions corresponding to histiocytic granulomas and “neurodegenerative” presentation NDLCH characterized by a progressive cerebellar ataxia Studies analyzing specifically the MRI presentation of NDLCH are scarce We present here the MRIs of 13 patients registered as isolated NDLCH 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
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