Therapeutic plasma exchange shows promise in treating Stiff Person Syndrome

Therapeutic plasma exchange (TPE) can provide immediate symptomatic relief for patients with Stiff Person Syndrome (SPS) when used as an adjunct to other treatments, though the beneficial effects may not be sustained long-term, according to a study. This highlights TPE’s potential role in the management of refractory SPS cases, warranting further investigation into methods for maintaining its efficacy.

The first line of treatment for SPS typically includes gamma-aminobutyric acid (GABA)ergic agonists, benzodiazepines, and baclofen. However, in cases where these treatments prove insufficient, TPE has been employed either alone or in combination with other immunomodulatory therapies.

In this observational study, 5 patients with SPS underwent TPE as part of their treatment regimen. Notably, 80% of these patients tested positive for anti-glutamic acid decarboxylase 65-kilodalton isoform (anti-GAD65) antibodies, which are commonly associated with the disorder. All participants received immunosuppressive drugs in conjunction with TPE.

The clinical outcomes were promising, with all 5 patients experiencing significant symptom relief immediately following TPE. However, despite this initial success, the improvements were not sustained over the long term.

Reference
Kumawat V, Tripathi PP, Mahadevan A, et al. Therapeutic role of plasma exchange in the management of stiff person syndrome: experience from a tertiary care centre. Hematol Transfus Cell Ther. 2024;S2531-1379(24)00008-7. doi: 10.1016/j.htct.2023.11.014. Epub ahead of print. PMID: 38614933.