Details

Autor(en) / Beteiligte

• Autier, Lila
• Gaymard, Bertrand
• Bayen, Eléonore
• Del Cul, Antoine
• Cohen-Aubart, Fleur
• Martin-Duverneuil, Nadine
• Haroche, Julien
• Mokhtari, Karima
• Héritier, Sébastien
• Donadieu, Jean
• Hoang-Xuan, Khê
• Idbaih, Ahmed

Titel

Eye movement abnormalities in neurodegenerative langerhans cell histiocytosis

Ist Teil von

• Neurological sciences, 2022-11, Vol.43 (11), p.6539-6546

Ort / Verlag

Cham: Springer International Publishing

Erscheinungsjahr

2022

Quelle

Springer Online Journals【Remote access available】

Beschreibungen/Notizen

• 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.