Abstract
Langerhans cell histiocytosis (LCH) is a rare inflammatory myeloid neoplasm characterized by proliferation of tumor histiocytes that involves multiple organs including central nervous system. The physiopathologic process underlying degenerative neuro-LCH (i.e., DN-LCH) remains imperfectly settled. Since the main clinical features of DN-LCH are cerebellar ataxia and dysexecutive syndrome, eye movements might be disrupted and may help in disease diagnosis and monitoring. We retrospectively analyzed the medical records of twenty DN-LCH patients investigated using eye movement recording (EMR) in our hospital between 2015 and 2018. DN-LCH patients exhibited (i) abnormal gain in visually guided saccades including hypermetric saccades and excessive gain variability -45.0%-, (ii) increased mean antisaccade error rates -66.7%-, (iii) altered smooth pursuit -50.0%-, and (iv) excessive number of square wave jerks-25%- and gaze-evoked nystagmus. Our study suggests that DN-LCH patients present a peculiar pattern of eye movement impairments supporting cerebellar and prefrontal dysfunctions. As a non-invasive method, EMR could therefore be a useful tool for quantitative monitoring of DN-LCH patients. Further studies are warranted to support our findings.
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LA: acquisition and interpretation of data, drafting manuscript, and responsibility for the integrity of the study. AI and BG: study concept and design, acquisition and interpretation of data, and study supervision. EB, ADC, FC-A, NM-D, JH, SH, JD, and KH-X: critical revision of manuscript for intellectual content. All read and approved the manuscript.
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Ahmed Idbaih reports Research grants from Carthera, Transgene, Sanofi, Air liquide, Servier, Nutritheragene, advisory board for Leo Pharma, Novocure, Boehringer Ingelheim Int, travel funding from Novocure Carthera and Leo Pharma outside the submitted work .
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Autier, L., Gaymard, B., Bayen, E. et al. Eye movement abnormalities in neurodegenerative langerhans cell histiocytosis. Neurol Sci 43, 6539–6546 (2022). https://doi.org/10.1007/s10072-022-06180-y
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DOI: https://doi.org/10.1007/s10072-022-06180-y