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Case Report/Case Series |

Autologous Stem Cell Transplantation for Stiff Person Syndrome:  Two Cases From the Ottawa Blood and Marrow Transplant Program

Sheilagh Sanders, MD1; Christopher Bredeson, MD2,3; C. Elizabeth Pringle, MD1; Lisa Martin4; David Allan, MD2,3; Isabelle Bence-Bruckler, MD2; Linda Hamelin, MSc2; Harry S. Hopkins2; Mitchell Sabloff, MD2; Dawn Sheppard, MD2; Jason Tay, MD2,3; Lothar Huebsch, MD2; Harold L. Atkins, MD2,3
[+] Author Affiliations
1Department of Medicine, University of Ottawa, Ottawa, Ontario, Canada
2Ottawa Hospital Blood and Marrow Transplant Program, Ottawa, Ontario, Canada
3Ottawa Hospital Research Institute, Ottawa, Ontario, Canada
4Canadian Blood Services, Ottawa, Ontario, Canada
JAMA Neurol. 2014;71(10):1296-1299. doi:10.1001/jamaneurol.2014.1297.
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Importance  Stiff person syndrome (SPS) is a rare neurological disease causing significant functional disability for patients and presenting a therapeutic challenge for clinicians. Autologous hematopoietic stem cell transplantation (auto-HSCT) has been used successfully to remit autoimmune-mediated neurological diseases. We report 2 cases of severe SPS treated with auto-HSCT, a novel therapy for this disease.

Observations  Two anti–glutamic acid decarboxylase antibody–positive patients with SPS had an autologous hematopoietic stem cell graft collected and stored. Subsequently, the patients underwent auto-HSCT. Both patients achieved clinical remission with sustained, marked improvement in symptoms and a return to premorbid functioning, now more than 2.5 and 4.5 years after the procedure.

Conclusions and Relevance  Stiff person syndrome represents a novel indication for auto-HSCT. The resolution of clinical manifestations of SPS despite the persistence of anti–glutamic acid decarboxylase antibodies following auto-HSCT suggests that the antibody does not play a direct role in pathogenesis of SPS.

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Immunoablative Regimens

Immunoablative regimens for case 1 (A) and case 2 (B). Auto-HSCT indicates autologous hematopoietic stem cell transplantation.

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