Saccadic abnormalities in nephropathic cystinosis

Barrett Katz, Ronald B. Melles, Doris A. Trauner, Jerry A. Schneider

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Abstract

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° 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.

Original languageEnglish (US)
Pages (from-to)115-119
Number of pages5
JournalJournal of Clinical Neuro-Ophthalmology
Volume8
Issue number2
StatePublished - Jun 1988

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Keywords

  • Cystinosis
  • Saccades
  • Saccadic duration

ASJC Scopus subject areas

  • Ophthalmology
  • Clinical Neurology

Cite this

Katz, B., Melles, R. B., Trauner, D. A., & Schneider, J. A. (1988). Saccadic abnormalities in nephropathic cystinosis. Journal of Clinical Neuro-Ophthalmology, 8(2), 115-119.