Objective: The aim of this study is to describe a Tunisian case series of patients with SPS and to determinate its particularity compared to other studies.

Background: Stiff Person Spectrum (SPS) disorder is a rare group of auto-immune disorders. Its physiopathology frequently associated with anti-GAD antibodies but also with paraneoplastic presentation.

Method: We conducted a retrospective mono-centric  study in the national institute of neurology Mongi Ben Hmida of Tunis, including all the patients diagnosed as SPS based on clinical, electrophysiological findings and serological testing, based on DALAKS criteria for SPS.

Data collected included demographic characteristics, clinical symptoms at onset, disease progression, associated autoimmune conditions, antibody status, and treatment response.

Results: Nine patients were included, with a sex ratio H/F of 0.8, a mean age at onset of 51 (between 29 and 75) and a mean delay to diagnosis of 8 months (between 2 months and 24).Most patients (7 out of 9) had the classical presentation of SPS typically starting with insidious onset of stiffness in the proximal lower limbs, followed by spasms and rigidity of the thoracolumbar, paraspinal and abdominal muscles and proximal upper limbs, only one patient had bulbar symptoms associated with the SPS and one patient had stiff limb syndrome presentation. Two patients had a family history of malignancy. Only one patient had a history of thyroid disorder and Parkinson disease respectively.

Regarding immunological studies, 2 patients had positive anti-GAD antibodies and one patient had positive anti-Ri antibodies with no malignancy detected.

All patients were treated with corticotherapy and benzodiazepine, while Five patients received intra-venous immunoglobulin. At follow-up, all patients exhibited partial amelioration and 8 were wheelchair bound.

Conclusion: This study provides precious insight into the SPS within a Tunisian case series, highlighting the clinical manifestations, the serological diagnosis and evolution after treatment, leading to further studies in the SPS field.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/stiff-person-syndrome-about-a-tunisian-case-series/