Practical Neurology Podcast
Stiff-person syndrome
Feb 22, 2025
In this discussion, Professor Saiju Jacob, a leading consultant neurologist specializing in neuroimmunology, explores Stiff-Person Syndrome (SPS), a complex autoimmune disorder. He highlights the diagnostic dilemmas faced, particularly its prevalence in women and the role of specific antibody tests. The conversation delves into associated ocular symptoms and the importance of clinical history in accurate diagnosis. Professor Jacob also discusses treatment strategies, emphasizing the need for early intervention and the potential improvements in mental health with effective management.
42:06
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Quick takeaways
- Stiff-person syndrome is characterized by severe muscle stiffness and exaggerated startle responses, complicating timely diagnosis due to symptom overlap with other disorders.
- Management of SPS includes symptomatic treatments and immunotherapy options, emphasizing that early intervention is crucial for improving patient outcomes.
Deep dives
Understanding Stiff Person Syndrome
Stiff Person Syndrome (SPS) is a rare neurological disorder characterized by a spectrum of symptoms including axial stiffness and exaggerated startle reactions. The condition can manifest in various forms, including stiff limb syndrome, where stiffness may affect only one limb. A key feature distinguishing SPS from other disorders is the patient's continuous startle response to stimuli, which occurs repeatedly as opposed to the typical reflexive response seen in healthy individuals. This syndrome is not limited to humans, as variations have been identified in animals, reinforcing the need for a wider understanding of the condition beyond just human implications.
