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MRI features of neurodegenerative Langerhans cell histiocytosis
N Martin-Duverneuil1, A Idbaih, K Hoang-Xuan
1Department of Neuroradiology, GH Pitié-Salpêtrière, 47 Bd de l'Hôpital, 75013, Paris, France. nadine.martin-duverneuil@psl.ap-hop-paris.fr
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.
Insights
Neurodegenerative-LCH (ND-LCH) presents with unique MRI findings. This study details characteristic brain imaging features to aid in diagnosing this rare form of Langerhans cell histiocytosis.
Area of Science:
- Neurology
- Radiology
- Pediatric Oncology
Background:
- Central nervous system (CNS) complications of Langerhans cell histiocytosis (LCH) include space-occupying lesions and neurodegenerative presentations (ND-LCH).
- Neurodegenerative-LCH (ND-LCH) is characterized by progressive cerebellar ataxia.
- Research specifically analyzing the MRI presentation of ND-LCH is limited.
Purpose of the Study:
- To describe the magnetic resonance imaging (MRI) findings in patients with isolated ND-LCH.
- To identify characteristic MRI semeiology for diagnosing pure ND-LCH.
Main Methods:
- Retrospective analysis of MRI scans from 13 patients diagnosed with isolated ND-LCH.
- Detailed evaluation of lesion location, signal characteristics (T1 and T2 hyperintensity), and presence of atrophy in both posterior fossa and supratentorial regions.
Main Results:
- Posterior fossa involvement (92%) included cerebellar white matter T2 hyperintensities, dentate nucleus T1 hyperintensities, and pontine tegmentum/pyramidal tract abnormalities.
- Supratentorial involvement (85%) showed cerebral white matter T2 lesions and globus pallidus T1 hyperintensities.
- Cerebellar atrophy was observed in 8 cases, and cerebral/corpus callosum atrophy in 3 cases each.
Conclusions:
- This series establishes a distinctive MRI presentation for ND-LCH.
- Recognizing these specific MRI features can help orient diagnosis toward the pure neurodegenerative form of LCH.
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