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Stiff Person Syndrome: A Rare Neurological Disorder, Heterogeneous in Clinical Presentation and Not Easy to Treat
Joint Authors
Capone, Loredana
Buechner, Susanne
Florio, Igor
Source
Case Reports in Neurological Medicine
Issue
Vol. 2015, Issue 2015 (31 Dec. 2015), pp.1-5, 5 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2015-05-27
Country of Publication
Egypt
No. of Pages
5
Main Subjects
Abstract EN
Background.
Stiff person syndrome (SPS) is a rare neurological disorder characterized by progressive rigidity of axial and limb muscles associated with painful spasms.
SPS can be classified into classic SPS, paraneoplastic SPS, and SPS variants.
Its underlying pathogenesis is probably autoimmune, as in most cases antibodies against glutamic acid decarboxylase (GAD) are observed.
Similarly, paraneoplastic SPS is usually linked to anti-amphiphysin antibodies.
Treatment is based on drugs enhancing gamma-aminobutyric acid (GABA) transmission and immunomodulatory agents.
Case Series.
Patient 1 is a 45-year-old male affected by the classic SPS, Patient 2 is a 73-year-old male affected by paraneoplastic SPS, and Patient 3 is a 68-year-old male affected by the stiff limb syndrome, a SPS variant where symptoms are confined to the limbs.
Symptoms, diagnostic findings, and clinical course were extremely variable in the three patients, and treatment was often unsatisfactory and not well tolerated, thus reducing patient compliance.
Clinical manifestations also included some unusual features such as recurrent vomiting and progressive dysarthria.
Conclusions.
SPS is a rare disorder that causes significant disability.
Because of its extensive clinical variability, a multitask and personalized treatment is indicated.
A clearer understanding of uncommon clinical features and better-tolerated therapeutic strategies are still needed.
American Psychological Association (APA)
Buechner, Susanne& Florio, Igor& Capone, Loredana. 2015. Stiff Person Syndrome: A Rare Neurological Disorder, Heterogeneous in Clinical Presentation and Not Easy to Treat. Case Reports in Neurological Medicine،Vol. 2015, no. 2015, pp.1-5.
https://search.emarefa.net/detail/BIM-1059007
Modern Language Association (MLA)
Buechner, Susanne…[et al.]. Stiff Person Syndrome: A Rare Neurological Disorder, Heterogeneous in Clinical Presentation and Not Easy to Treat. Case Reports in Neurological Medicine No. 2015 (2015), pp.1-5.
https://search.emarefa.net/detail/BIM-1059007
American Medical Association (AMA)
Buechner, Susanne& Florio, Igor& Capone, Loredana. Stiff Person Syndrome: A Rare Neurological Disorder, Heterogeneous in Clinical Presentation and Not Easy to Treat. Case Reports in Neurological Medicine. 2015. Vol. 2015, no. 2015, pp.1-5.
https://search.emarefa.net/detail/BIM-1059007
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1059007