Diabetes also co-occurs in approximately 30% of people with SPSD, said Dr. Dalakas. “Endocrinologists should also be aware of this connection.”
Paraneoplastic stiff person syndrome is thought to be triggered by cancer, which might not have been diagnosed, making it important to work up patients for malignancy – particularly breast cancer, small cell lung cancer, lymphoma, and thymoma, Dr. Newsome advised.
Although most cases of SPSD are diagnosed in mid-life, the disorder can occur in teenagers and the elderly.
“It’s not the first thing you think of when a 70-year-old patient comes with neck pain, so it’s missed more often, and the prognosis is worse,” Dr. Dalakas warned.
What does the future hold?
Like Dr. Newsome, Dr. Dalakas is encouraged when SPSD hits the headlines because, generally, awareness facilitates diagnosis and research. (Both clinicians serve on the medical advisory board of The Stiff Person Syndrome Research Foundation.)
“We are looking for better therapies that target immune factors,” said Dr. Dalakas. “There are several of those that are relevant, so we need to select the best immune marker that we think plays a role in the antibody production,” he said.
“There’s a lot of hope – at least I have a lot of hope for what the future holds with SPSD,” added Dr. Newsome. “More research is needed and it starts with awareness of SPSD.”
Dr. Newsome discloses that he has received consulting fees for serving on scientific advisory boards of Biogen, Genentech, Bristol Myers Squibb, EMD Serono, Jazz Pharmaceuticals, Novartis, Horizon Therapeutics, TG Therapeutics; is the study lead principal investigator for a Roche clinical trial; and has received research funding (paid directly to his employing institution) from Biogen, Roche, Lundbeck, Genentech, The Stiff Person Syndrome Research Foundation, National Multiple Sclerosis Society, U.S. Department of Defense, and Patient-Centered Outcomes Research Institute. Dr. Dalakas reports nothing relevant to disclose.
References
1. Chia NH et al. Ann Clin Transl Neurol. 2023;10(7):1083-94. doi: 10.1002/acn3.51791.
2. Dalakas MC.. Curr Treat Options Neurol. 2009;11(2):102-10. doi: 10.1007/s11940-009-0013-9.
3. Newsome SD and Johnson T. J Neuroimmunol. 2022;369:577915. doi: 10.1016/j.jneuroim.2022.577915.
4. Ortiz JF et al. Cureus. 2020;12(12):e11995. doi: 10.7759/cureus.11995.
5. Dalakas CD. Neurol Neuroimmunol Neuroinflamm. 2023;10(3):e200109. doi: 10.1212/NXI.0000000000200109.