Case Report
Stiff person syndrome (SPS): Literature review and case report
Submitted: 17 January 2013 | Published: 30 November 2013
About the author(s)
Erna Pretorius, Department of Neurology, Faculty of Health Sciences, University of the Free State, Bloemfontein, South Africa, South AfricaWilmarie Struwig, Department of Internal Medicine, Faculty of Health Sciences, University of the Free State, Bloemfontein, South Africa, South Africa
Abstract
Stiff person syndrome (SPS) is a rare, debilitating condition which presents with progressive and inconsistent neurological features. The main symptoms are stiffness and intermittent, painful muscle spasms, triggered and exacerbated by stressful and emotional stimuli. The fluctuating clinical nature of SPS, and otherwise normal neurological examination, often lead to a misdiagnosis of conversion disorder. Psychiatric symptoms frequently accompany this disorder and patients are often first seen by psychiatrists. SPS is autoimmune-based: antibodies are directed against glutamate decarboxylase, resulting in dysregulation of gamma-aminobutyric acid (GABA) in the brain which is considered the cause of the neuropsychiatric symptomatology. SPS should be considered in the differential diagnosis of conversion disorder. Effective management requires early detection, a collaborative approach with GABA-ergic medication and intravenous immunoglobulins, and management of concomitant psychiatric disorders. We describe a patient with SPS. Only one other case has been reported in South Africa.
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Crossref Citations
1. Femur Neck Fracture in a Patient with Stiff Person Syndrome
Mujahid Jamil, Muhammad Saleem, Ahmed Habib, Sara Khan, Muhammad Qahir
JBJS Case Connector vol: 9 issue: 3 first page: e0049 year: 2019
doi: 10.2106/JBJS.CC.18.00049