Two case reports of rare stiff person syndrome

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.

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 (auto-HSCT) has been used to successfully treat patients with autoimmune diseases such as 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.

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.

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 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.

"To our knowledge, this is the first report documenting that immunoablation followed by auto-HSCT can produce long-lasting and complete remission of SPS." Sheilagh Sanders, M.D., of the University of Ottawa, Canada, and colleagues said in their JAMA Neurology article.

More information: JAMA Neurol. Published online August 25, 2014. DOI: 10.1001/.jamaneurol.2014.1297

add to favorites email to friend print save as pdf

Related Stories

Ocular complications common after pediatric HSCT

Mar 01, 2013

(HealthDay)—For pediatric patients undergoing hematopoietic stem cell transplantation (HSCT), complications are common, but are generally mild and self-limiting, according to a study published online Feb. ...

Recommended for you

New ALS associated gene identified using innovative strategy

13 hours ago

Using an innovative exome sequencing strategy, a team of international scientists led by John Landers, PhD, at the University of Massachusetts Medical School has shown that TUBA4A, the gene encoding the Tubulin Alpha 4A protein, ...

Can bariatric surgery lead to severe headache?

13 hours ago

Bariatric surgery may be a risk factor for a condition that causes severe headaches, according to a study published in the October 22, 2014, online issue of Neurology, the medical journal of the American Academy of Neurol ...

Bipolar disorder discovery at the nano level

13 hours ago

A nano-sized discovery by Northwestern Medicine scientists helps explain how bipolar disorder affects the brain and could one day lead to new drug therapies to treat the mental illness.

Brain simulation raises questions

17 hours ago

What does it mean to simulate the human brain? Why is it important to do so? And is it even possible to simulate the brain separately from the body it exists in? These questions are discussed in a new paper ...

Human skin cells reprogrammed directly into brain cells

17 hours ago

Scientists have described a way to convert human skin cells directly into a specific type of brain cell affected by Huntington's disease, an ultimately fatal neurodegenerative disorder. Unlike other techniques ...

User comments