Title : Stiff-person syndrome-A review of case reports and new advancements in its management.
Abstract:
Stiffness-person syndrome (SPS) is a neurological disease causing symptoms of rigidity and painful muscle spasms. It is caused by increased serum anti-glutamic acid decarboxylase antibodies (anti-GAD). Persistent muscle stiffness can lead to difficulty walking and recurrent falls. This review discusses SPS, its pathophysiology, clinical features, diagnoses and the existing treatment strategies. . Published articles on the topic were collected by searching various databases that include PubMed, Sciencedirect, Hindawi, ResearchGate and Neurology-related journals in the period of 2003-2023. The keywords used for searching included ‘‘Stiff-person syndrome’’, ‘‘Case reports’’, and ‘‘Management’’. A total of 21 articles were extracted, out of which 9 articles were case reports, and around 5 articles focussed on the treatment methods currently used. The comparison narrative was divided into three groups according to the age of the cases. The presenting symptoms of all the patients were roughly the same, mainly comprising of painful muscle spasms, gait abnormalities, tremors and difficulty standing. Physical examination were consistent overall in all cases which include showed brisk symmetric reflexes and spastic walk, and rigid abdomen. Electromyography findings were consistent through out all the age groups- continuous motor unit discharge. Anti-GAD antibody titre levels were also elevated in all the cases except one. The treatment regimen for all age groups comprised of similar patterns- doses of Intravenous Immunoglobulin, Plasma exchange therapy,diazepam, baclofen, benzodiazepenes, and gabapentin. This review provides encouragement for additional investigation and clinical trials exploring advanced treatment modalities aimed at improving short-term clinical symptoms and fostering the long-term well-being of patients.