Spiral analysis in Niemann-Pick disease type C

Annie W. Hsu, Panida A. Piboolnurak, Alicia G. Floyd, Qiping P. Yu, James E. Wraith, Marc C. Patterson, Seth L. Pullman

Research output: Contribution to journalArticlepeer-review

22 Scopus citations

Abstract

Spiral analysis is a computerized method of analyzing upper limb motor physiology through the quantification of spiral drawing. The objective of this study was to determine whether spirals drawn by patients with Niemann-Pick disease type C (NPC) could be distinguished from those of controls, and to physiologically characterize movement abnormalities in NPC. Spiral data consisting of position, pressure, and time were collected from 14 NPC patients and 14 age-matched controls, and were analyzed by the Mann-Whitney U test. NPC spirals were characterized by: lower speed (2.67 vs. 9.56 cm/s, P < 0.001) and acceleration (0.10 vs. 2.04 cm/s2, P < 0.001), higher loop width variability (0.88 vs. 0.28, P < 0.001), tremor (5/10 vs. 0/10 trials in the dominant hand, P < 0.001), and poor overall spiral rating (2.53 vs. 0.70, P < 0.005). NPC spirals also exhibited sustained drawing pressure profiles that were abnormally invariant with time. Other features, such as the tightness of loop widths, were normal. Our findings reveal that differing aspects of tremor, Parkinsonism, ataxia, and dystonia are quantifiable in NPC patients.

Original languageEnglish (US)
Pages (from-to)1984-1990
Number of pages7
JournalMovement Disorders
Volume24
Issue number13
DOIs
StatePublished - Oct 15 2009

Keywords

  • Motor physiology
  • Niemann-Pick
  • Spiral analysis

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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