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Presentation
Treatment of Spinocerebellar Ataxia with Intravenous Immune Globulin (IVIG)
American Academy of Neurology Annual Meeting (AAN) (2014)
  • Theresa A. Zesiewicz, University of South Florida
  • Israt Jahan, University of South Florida
  • Kelly L. Sullivan, Georgia Southern University
  • Clifton L. Gooch, University of South Florida
  • Tuan Vu, University of South Florida
Abstract
Objective: To assess the effect of intravenous immune globulin (IVIG) on symptoms of spinocerebellar ataxia (SCA) as measured by total Scale for the Assessment and Rating of Ataxia (SARA) score and gait parameters in an open-label pilot study.

Background: While the exact pathophysiology of SCA is unknown, inflammation may contribute to neuronal injury. Immunohistochemical studies of SCA3 cell cultures have reported extracellular A-immunoreactive deposits in the pons.

Methods: Eligible patients included those with genetic or sporadic SCA, without peripheral neuropathy, vitamin deficiencies, or autoimmune disorders. Patients received IVIG (2 grams/kilogram of body weight divided over five days) during weeks 1, 5, and 9 and were evaluated 14 days after each course of IVIG, and 28 days and 56 days after the final course using the SARA and the GAITRite ® Walkway System (CIR Systems, Inc.).

Results: Five SCA patients enrolled in the study. One patient withdrew due to an adverse event (eye irritation, not treatment related) and one patient withdrew for personal reasons. The three remaining patients had SCA3, SCA5, and sporadic SCA. Following IVIG treatment, there was approximately 40% reduction in SARA total score, and a 10-20% mean improvement in spatiotemporal gait parameters, including gait velocity and stride length during comfortable walking. The greatest improvement was generally noted after the third course. Improvements in gait (SARA subscore), oral motor action, and speech (PATA) were observed. Clinical improvements persisted for 28 days after the final infusion, but attenuated by 56 days after the last infusion. Treatment related adverse events were limited to mild headaches.

Conclusions: In this pilot study, IVIG was well tolerated without major adverse events. Improved ataxia symptoms were observed in SCA patients, as demonstrated by clinical scales and by quantitative temporospatial gait analysis. Further controlled studies of IVIG in the treatment of SCA are warranted.

Study Supported by: Drug was supplied by Baxter® Pharmaceuticals.
Keywords
  • Treatment,
  • Spinocerebellar Ataxia,
  • Intravenous Immune Globulin,
  • IVIG
Disciplines
Publication Date
May 1, 2014
Location
Philadelphia, PA
Citation Information
Theresa A. Zesiewicz, Israt Jahan, Kelly L. Sullivan, Clifton L. Gooch, et al.. "Treatment of Spinocerebellar Ataxia with Intravenous Immune Globulin (IVIG)" American Academy of Neurology Annual Meeting (AAN) (2014)
Available at: http://works.bepress.com/kelly_sullivan/51/