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Published on: June 9, 2018
Quantitative Brain MRI Analysis in Neurodegenerative Langerhans Cell Histiocytosis
Chooyoung Baek1, Lucas Rincon de la Rosa2, Ilyes Aliouat3
1Sorbonne Université, AP-HP, Institut du Cerveau - Paris Brain Institute - ICM, Inserm, CNRS, Hôpitaux Universitaires La Pitié Salpêtrière - Charles Foix, DMU Neurosciences, Service de Neuro-Oncologie-Institut de Neurologie, Paris, France.
Neurodegenerative Langerhans Cell Histiocytosis (ND-LCH) causes progressive cerebellar atrophy. This study quantifies cerebellar volume loss over time in ND-LCH patients, revealing significant atrophy rates.
Area of Science:
- Neuroscience
- Radiology
- Neuropathology
Background:
- Neurodegenerative Langerhans Cell Histiocytosis (ND-LCH) involves severe central nervous system complications.
- Characterized by ataxia, pyramidal signs, cognitive impairment, and behavioral changes.
- Cerebellar atrophy is a common MRI finding, but its natural history is unknown.
Purpose of the Study:
- To quantitatively analyze cerebellar atrophy progression in ND-LCH patients.
- To compare volumetric changes in the cerebellum between ND-LCH patients and controls.
- To establish the rate of cerebellar atrophy in ND-LCH.
Main Methods:
- Retrospective analysis of follow-up MRI scans from 16 adult ND-LCH patients and 22 controls.
- Automated cerebellar analysis using Volbrain software (CERES).
- Linear regression analysis to assess atrophy rate over a median follow-up of six years.
Main Results:
- All ND-LCH patients showed global and cortical cerebellar atrophy over time.
- The average atrophy rate was -1.86 cm³/year, with significant patient heterogeneity.
- Atrophy affected all cerebellar lobules.
Conclusions:
- Longitudinal quantitative MRI analysis of the cerebellum in ND-LCH is feasible.
- Confirms significant, progressive cerebellar atrophy in ND-LCH patients.
- The estimated atrophy rate may serve as a therapeutic endpoint for this slow-progressing disease.
