Neurophysiologic Studies of the Peripheral Nervous System in Nephropathic Cystinosis

Michael R. Swenson, Doris A. Trauner, Steve Rimmer, Ronald B. Melles, Jerry A. Schneider, Barrett Katz

Research output: Contribution to journalArticlepeer-review

5 Scopus citations


Cystinosis is an autosomal recessive metabolic disorder in which nonprotein cystine accumulates within most body tissues due to a defect in lysosomal cystine transport. Neurologic declarations are only recently being recognized. We studied 13 cystinotic subjects (aged 5 to 21 years old), determining median motor and sensory nerve conduction velocities, F waves, peroneal motor nerve conduction velocities, sural sensory nerve conduction velocities, median sympathetic skin response, electrocardiogram R-to-R variability, and blink reflex analysis. The results were normal. We conclude that neurophysiologic testing suggests relative sparing of the peripheral nervous system in nephropathic cystinosis.

Original languageEnglish (US)
Pages (from-to)528-529
Number of pages2
JournalArchives of Neurology
Issue number5
StatePublished - May 1991

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology


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