Preserved eye movements in adults with spinal muscular atrophy
| Autor: | Evangelos Anagnostou, Sophia Xirou, Evangelia Kararizou, Leonidas Stefanis, Constantinos Papadopoulos, George K. Papadimas |
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| Rok vydání: | 2021 |
| Předmět: |
Adult
Male 0301 basic medicine medicine.medical_specialty Eye Movements genetic structures Physiology Ocular motor 030105 genetics & heredity Extraocular muscles Muscular Atrophy Spinal Neuro-ophthalmology Young Adult 03 medical and health sciences Cellular and Molecular Neuroscience 0302 clinical medicine Physical medicine and rehabilitation Physiology (medical) Saccades medicine Humans Eye Movement Measurements Aged Muscle Weakness business.industry Eye movement Spinal muscular atrophy Middle Aged SMA medicine.disease Saccadic masking medicine.anatomical_structure Oculomotor Muscles Female Neurology (clinical) Neuron business 030217 neurology & neurosurgery |
| Zdroj: | Muscle & Nerve. 63:765-769 |
| ISSN: | 1097-4598 0148-639X |
| DOI: | 10.1002/mus.27204 |
| Popis: | Introduction Spinal muscular atrophy (SMA) most prominently affects proximal limb and bulbar muscles. Despite older case descriptions, ocular motor neuron palsies or other oculomotor abnormalities are not considered part of the phenotype. Methods We investigated oculomotor function by testing saccadic eye movements of 15 patients with SMA. Their performance was compared with that of age-matched healthy controls. Horizontal rightward and leftward saccades were recorded by means of video-oculography, whereas subjects looked at light-emitting diode targets placed at ±5°, ±10°, and ±15° eccentricities. Results No differences in saccade amplitude gains, peak velocities, peak velocity-to-amplitude ratios, or durations were observed between controls and patients. More specifically, for 5° target eccentricities, patients had a mean saccadic peak velocity of 153°/s, whereas for 10° and 15° these values were 268°/s and 298°/s, respectively. The corresponding mean peak velocities of the control group were 151°/s, 264°/s, and 291°/s. Discussion Our results indicate that patients with SMA perform fast and accurate horizontal saccades without evidence of extraocular muscle weakness. These quantitative oculomotor data corroborate clinical experience that neuro-ophthalmic symptoms in SMA are not common and, if present, should prompt suspicion for an alternative neuromuscular disorder. |
| Databáze: | OpenAIRE |
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