Two case reports of rare stiff person syndrome

August 25, 2014

Bottom Line: Two female patients achieved clinical remission from the rare, debilitating neurological disease called stiff person syndrome (SPS, which can be marked by a "tin soldier" gait) after an autologous (from your own body) stem cell transplant that eventually allowed them to return to work and regain their previous functioning.

Author: Sheilagh Sanders, M.D., of the University of Ottawa, Canada, and colleagues.

Background: SPS is a disease characterized by stiffness of the skeletal muscles, painful muscle spasms and, in severe cases, the disease can prevent movement and walking. Autologous hematopoietic stem cell transplantation (auto-HSCT) has been used to successfully treat patients with autoimmune diseases such as multiple sclerosis and scleroderma, which are resistant to more conventional treatment. A regimen of high-dose chemotherapy and antilymphocyte antibodies rid the body of diseased immune cells (immunoablation) before the immune system is regenerated with auto-HSCs.

About the Care Report: The Ottawa Hospital Blood and Marrow Transplant Program performed immunoablation and auto-HSCT on the two women with severe SPS based on a regimen used for multiple sclerosis.

Results: One of the women was diagnosed in 2005 at age 48 after having progressive leg stiffness, spasms, falls and walking with a "tin soldier" gait. The auto-HSC was performed in 2009. One month after the transplant, her SPS symptoms were resolved and she was fully mobile six months after the transplant and returned to work and playing sports. She remains asymptomatic nearly five years after transplantation.

The second woman was an otherwise healthy woman who had had periodic leg muscle stiffness that lasted several hours and she was eventually diagnosed with SPS in 2008 at age 30 years. She had stopped working, driving and moved back in with her parents before undergoing auto-HSCT in 2011. Her post-transplant course was complicated by four periods of severe muscle spasms within 18 months of transplantation. The woman has been able to return to work and her previous activities. She has not had SPS symptoms in more than a year.

Discussion: 'To our knowledge, this is the first report documenting that immunoablation followed by auto-HSCT can produce long-lasting and complete remission of SPS."
-end-
(JAMA Neurol. Published online August 25, 2014. doi:10.1001/.jamaneurol.2014.1297. Available pre-embargo to the media at http://media.jamanetwork.com.)

Editor's Note: Please see the article for additional information, including other authors, author contributions and affiliations, financial disclosures, funding and support, etc.

Media Advisory: To contact corresponding author Harold L. Atkins, M.D., call Paddy Moore at 613-737-8899 x73687 or email padmoore@ohri.ca.

To place an electronic embedded link to this study in your story A link for this case report will be live at the embargo time: http://archneur.jamanetwork.com/article.aspx?doi=10.1001/jamaneurol.2014.1297.

The JAMA Network Journals

Related Transplantation Articles from Brightsurf:

A revolutionary new treatment alternative to corneal transplantation
A new approach in ophthalmology that offers a revolutionary alternative to corneal transplantation has just been developed by researchers and clinicians in North America, Europe, and Oceania.

Fewer complications after organ transplantation
A large international study coordinated by University Hospital Regensburg and Charité - Universitätsmedizin Berlin has demonstrated the safety of new cell therapy approaches for use in kidney transplant recipients.

Elderly patients also benefit from kidney transplantation
So far, kidney transplantation has generally not been offered to elderly patients (>75 years) because of the perioperative risks.

New material will allow abandoning bone marrow transplantation
Scientists from the National University of Science and Technology 'MISIS' developed nanomaterial, which will be able to restore the internal structure of bones damaged due to osteoporosis and osteomyelitis.

Fewer medical tests -- timely listing for transplantation
Younger patients would benefit greatly from kidney transplantation. Their expected remaining lifetime may even be doubled by having a transplant.

Uterus transplantation -- ethically just as problematic as altruistic surrogacy
In 2014, the first child to have been gestated in a donated uterus was born.

Advancing transplantation: Hepatitis C-infected organs safe for transplantation when followed by antiviral treatment
Twenty patients at Penn Medicine have been cured of the hepatitis C virus (HCV) following lifesaving kidney transplants from deceased donors who were infected with the disease, according to a study published today in Annals of Internal Medicine.

Transplantation followed by antiviral therapy cured hepatitis C
Twenty patients who received kidneys transplanted from hepatitis C virus (HCV)-infected donors experienced HCV cure, good quality of life, and excellent renal function at one year.

The Journal of Heart and Lung Transplantation: 50 years of heart transplantation progress
This month marks the 50th anniversary of the world's first human heart transplant performed at Groote Schuur Hospital in Cape Town by South African surgeon, Christiaan Barnard.

Older donor lungs should be considered for transplantation
With a scarcity of lungs available for transplantation, the use of lungs from donors older than age 60 has been shown to achieve reasonable outcomes and should be considered as a viable option, according to research published online today in The Annals of Thoracic Surgery.

Read More: Transplantation News and Transplantation Current Events
Brightsurf.com is a participant in the Amazon Services LLC Associates Program, an affiliate advertising program designed to provide a means for sites to earn advertising fees by advertising and linking to Amazon.com.