From the Research
The life expectancy for people with Stiff Person Syndrome (SPS) is generally normal for most patients, though some may have a slightly reduced lifespan depending on symptom severity and complications. Most SPS patients do not die directly from the condition itself. Common causes of mortality in SPS patients include:
- Complications from falls or injuries due to muscle rigidity and spasms
- Respiratory complications during severe spasm episodes affecting chest muscles
- Complications from associated autoimmune conditions that frequently co-occur with SPS (such as type 1 diabetes, thyroiditis, or pernicious anemia) Sudden death can rarely occur from autonomic dysfunction or during severe spasm episodes affecting breathing. The prognosis varies significantly between patients, with some experiencing mild symptoms that respond well to treatment while others face progressive disability. Early diagnosis and appropriate treatment with medications like diazepam, baclofen, gabapentin, or immunotherapies (IVIG, plasmapheresis, or rituximab) can significantly improve quality of life and potentially reduce complications, as shown in a study published in 2021 1. Regular monitoring by neurologists and management of associated autoimmune conditions are essential for optimizing outcomes and preventing complications that could impact life expectancy. According to a recent study published in 2020, treatment protocols for SPS may include GABAergic therapy and immunotherapy, with benzodiazepines as first-line treatment, and intravenous immunoglobulin (IVIG) or plasmapheresis for patients with refractory symptoms 2. Another study published in 2019 highlighted the effectiveness of therapeutic plasma exchange (TPE) in improving mobility and reducing disability in patients with severe SPS 3. Overall, while SPS can have a significant impact on quality of life, early diagnosis and appropriate treatment can help manage symptoms and reduce the risk of complications, as supported by the study published in 2016 4.