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Spiral analysis in Niemann-Pick disease type C
Movement Disorders, 08/19/09
Hsu AW et al. – Findings show that differing aspects of tremor, Parkinsonism, ataxia, and dystonia are quantifiable in pts with Niemann-Pick disease type C (NPC).
Methods- Spiral analysis: computerized method of analyzing upper limb motor physiology through quantification of spiral drawing
- Study of whether spirals drawn by NPC pts vs by controls are distinct
- Physiological characterization of movement abnormalities in NPC
- Assessment of spiral data: position, pressure, time
- Subjects: 14 NPC pts and 14 age-matched controls
- Analysis by Mann-Whitney U test
- NPC spiral characterization: lower speed (2.67 vs 9.56 cm/s) and acceleration (0.10 vs 2.04 cm/s2), higher loop width variability (0.88 vs 0.28), tremor (5/10 vs 0/10 trials in dominant hand), and poor overall spiral rating (2.53 vs 0.70)
- NPC spirals: sustained drawing pressure profiles abnormally invariant with time
- Normal other features: tightness of loop widths
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