Saccadic Abnormalities in Nephropathic Cystinosis
作者:
Barrett Katz,
Ronald Melles,
Doris Trauner,
Jerry Schneider,
期刊:
Journal of Clinical Neuro-Ophthalmology
(OVID Available online 1988)
卷期:
Volume 8,
issue 2
页码: 113-120
ISSN:0272-846X
年代: 1988
出版商: OVID
关键词: Cystinosis;Saccades;Saccadic duration.
数据来源: OVID
摘要:
Cystinosis is an autosomal recessive metabolic disorder in which nonprotein cystine accumulates within most body tissues due to a defect in lysosomal cystine transport. The pathognomonic manifestation of cystinosis is the presence of distinctive iridescent crystals within ocular tissues. Although these crystals have been detected within the extraocular muscles, no associated abnormality of eye movement has been described. We measured horizontal saccades of 5–30|Mo in six patients with infantile nephropathic cystinosis. Our data indicate that patients with cystinosis have slowed saccades. Saccadic duration, peak velocity, peak acceleration, and peak deceleration were all abnormal.
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