[Abstract Title]. - Society for Neuroscience

Poster
248. Dystonia
Time: Sunday, November 16, 2008, 1:00 pm - 5:00 pm
Program#/Poster#: 248.5/U4
Topic: C.03.j. Dystonia
Support: NIH grant R01NS47151
Title: Quantification of gait in dystonic Gunn rats
Authors: K. D. CHANIARY 1 , *A. C. RICE 2 , M. S. BARON 2 , P. A. WETZEL 1 , S. M.
SHAPIRO 2 ;
1 Dept Biomed. Engin., 2 Dept Neurol., MCV - VCU, Richmond, VA
Abstract: The objective of this study was to develop a simple, non-invasive method to quantify
motor deficits in dystonic jaundiced Gunn rats congenitally deficient in UDP-glucuronosyl
transferase. These rats, when exposed to sulfadimethoxine, develop bilirubin encephalopathy
(kernicterus) with hearing loss and dystonia, closely resembling the human syndrome. We
recently characterized electromyograms (EMGs) in this model supporting our clinical impression
of dystonia. On day 16, Gunn rats were treated with 100 mg/kg of sulfadimethoxine and on day
31, gait analysis measurements were obtained. Systematic measurement of hindlimb spread, step
length ratio, stance/swing ratio and walking speed were carried out during free, spontaneous
locomotion in jaundiced dystonic, non-jaundiced non-dystonic and jaundiced non-dystonic
littermates. Data analysis demonstrated a significant increase in hindlimb spread
(67.72+0.88mm) in sulfa treated jaundiced animals as compared to controls (50.85+0.57mm)
including a significant decrease in step length ratio in sulfa treated jaundiced animals
(0.975+0.010) as compared to controls (1.002+0.011). This study helps us to develop a technique
to objectively characterize dystonia in rats. Systematic studies of motor control in this model
may lead to better understanding and treatment of movement disorders associated with
kernicterus in humans.
Disclosures: K.D. Chaniary, None; A.C. Rice , collaborator, B. Research Grant (principal
investigator, collaborator or consultant and pending grants as well as grants already received);
M.S. Baron, collaborator, B. Research Grant (principal investigator, collaborator or consultant
and pending grants as well as grants already received); P.A. Wetzel, None; S.M. Shapiro, PI, B.
Research Grant (principal investigator, collaborator or consultant and pending grants as well as
grants already received).