Videoocular assessment of eye movement activity in an ataxia-telangiectasia: a case study.

Autor: Mironets SA; Neurocognitive Laboratory, Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology, Moscow, Russia, 117997., Shurupova MA; Neurocognitive Laboratory, Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology, Moscow, Russia, 117997. shurupova@fccps.ru.; Department of Neurobiology, Faculty of Biology, Lomonosov Moscow State, Moscow, Russia, 119234. shurupova@fccps.ru.; Department of Rehabilitation, Federal Center of Brain Research and Neurotechnologies of the Federal Medical Biological Agency, Moscow, Russia, 117513. shurupova@fccps.ru., Karelin AF; Neurocognitive Laboratory, Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology, Moscow, Russia, 117997.
Jazyk: angličtina
Zdroj: Documenta ophthalmologica. Advances in ophthalmology [Doc Ophthalmol] 2024 Apr; Vol. 148 (2), pp. 107-114. Date of Electronic Publication: 2024 Feb 14.
DOI: 10.1007/s10633-024-09964-z
Abstrakt: Purpose: Ataxia-telangiectasia (A-T) is an autosomal recessive disorder characterized by progressive neurological deficits, including prominent oculomotor dysfunction. We report 5 cases of eye movement assessment in children 9-15 years old with A-T.
Methods: Three different oculomotor tasks (gaze holding, visually guided saccades and visual search) were used, and video-oculography was performed. Additionally, the scale for the assessment and rating of ataxia (SARA) score was used to assess severity of the cerebellar ataxia.
Results: Unstable gaze holding, nystagmus and saccadic intrusions were found. In addition to psychophysiological assessment results, we provide quantitative analysis of oculomotor activity, revealing a specific abnormal oculomotor pattern, consisting of (i) marked saccade hypermetria, (ii) unstable gaze holding, and (iii) gaze-evoked nystagmus.
Conclusion: Our study opens the prospect to evaluate efficacy and safety of alternative methods for supporting the patient and improving his/her life quality.
(© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
Databáze: MEDLINE
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