Abstract

Stiff Person syndrome (SPS) is a chronic autoimmune neurological disorder causing muscle stiffness, excessive startle and pain. Patients often have anti-GAD and occasionally anti-amphiphysin antibodies (especially in the paraneoplastic forms). There is no clear age, sex or race predilection clearly identified. Immunomodulatroy therapy with plasma exchange and IVIg is often beneficial in most patients.

We systematically looked at patients with a diagnosis of SPS with positive antibodies. 12 of these patients were evaluated in detail for their clinical features, concomitant illness and immunological status. We also evaluated the treatment response of these patients using a locally developed rating scale combining the functional activity and quality of life scores (BRIT scale– submitted as another abstract). Many patients had a long delay between the onset of symptoms and the final diagnosis. It was also noted that a good proportion of patients had certain behavioural and personality changes which could well be attributed to the disease, rather than by chance. More than 80% of patients had significant response to immunomodulatory therapy, although the duration of remission/response was variable.

This is one of the largest cohorts of SPS patients looked after in a single centre, which gives an insight into the clinical characterstics and immunological response of this rare neuroimmunological disorder.