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Open Access Highly Accessed Research article

Treatment of multiple system atrophy using intravenous immunoglobulin

Peter Novak1*, Arlene Williams2, Paula Ravin1, Omar Zurkiya3, Amir Abduljalil4 and Vera Novak5

Author affiliations

1 Department of Neurology, University of Massachusetts Medical School, D55 Lake Avenue North, Worcester, MA, 01655, USA

2 Clinical Trials Unit, University of Massachusetts Medical School, Worcester, MA, USA

3 Department of Radiology, Harvard Medical School, Boston, MA, USA

4 Department of Radiology, The Ohio State University, Columbus, OH, USA

5 Division of Gerontology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA

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Citation and License

BMC Neurology 2012, 12:131  doi:10.1186/1471-2377-12-131

Published: 1 November 2012

Abstract

Background

Multiple system atrophy (MSA) is a progressive neurodegenerative disorder of unknown etiology, manifesting as combination of parkinsonism, cerebellar syndrome and dysautonomia. Disease-modifying therapies are unavailable. Activation of microglia and production of toxic cytokines suggest a role of neuroinflammation in MSA pathogenesis. This pilot clinical trial evaluated safety and tolerability of intravenous immunoglobulin (IVIG) in MSA.

Methods

This was a single-arm interventional, single-center, open-label pilot study. Interventions included monthly infusions of the IVIG preparation Privigen®, dose 0.4 gram/kg, for 6 months. Primary outcome measures evaluated safety and secondary outcome measures evaluated preliminary efficacy of IVIG. Unified MSA Rating Scale (UMSARS) was measured monthly. Quantitative brain imaging using 3T MRI was performed before and after treatment.

Results

Nine subjects were enrolled, and seven (2 women and 5 men, age range 55–64 years) completed the protocol. There were no serious adverse events. Systolic blood pressure increased during IVIG infusions (p<0.05). Two participants dropped out from the study because of a non-threatening skin rash. The UMSARS-I (activities of daily living) and USMARS-II (motor functions) improved significantly post-treatment. UMSARS-I improved in all subjects (pre-treatment 23.9 ± 6.0 vs. post-treatment 19.0±5.9 (p=0.01). UMSARS-II improved in 5 subjects, was unchanged in 1 and worsened in 1 (pre-treatment 26.1±7.5 vs. post-treatment 23.3±7.3 (p=0.025). The MR imaging results were not different comparing pre- to post-treatment.

Conclusions

Treatment with IVIG appears to be safe, feasible and well tolerated and may improve functionality in MSA. A larger, placebo-controlled study is needed.