Session Information
Date: Thursday, June 23, 2016
Session Title: Other
Session Time: 12:00pm-1:30pm
Location: Exhibit Hall located in Hall B, Level 2
Objective: Two case reports of Stiff person syndrome (SPS) and SPS variant- Stiff limb syndrome.
Background: Stiff person syndrome is a rare autoimmune disorder of central nervous system characterized by progressive rigidity of axial and limb muscles and episodic painful spasms. SPS can be classified into classic SPS, paraneoplastic SPS and its variants- e.g. Stiff limb syndrome. Diagnostic criteria are based on typical clinical signs and EMG findings showing contionuous motor unit activity at rest. In most cases antibodies against acid decarboxylase (GAD) are observed. Treatment is symptomatic using drugs that enhance GABA transimission and immnodomudulatory therapy.
Methods: Recommended guidelines for SPS therapy with use of symptomatic treatment and immunomodulatory therapy.
Results: Two case report: Patient 1 is a 41 year old patient with classis SPS. The paraneoplastic etiology in this patient was ruled out, also there was no evidence for any autoimmune disorder. Symptomatic therapy with baclofen (3x25mg) and diazepam (3x5mg), following with 5 sessions of plasma exchange showed no benefit. Afterwards he was treated with IVIG (0,4g/kg/day given over 5 days) with significant improvement lasting 7 months when treatment with IVIG was repeated. Eventually we started chronic immunosuppresive treatment with azathioprine (100mg/d) and methylprednisolone (16mg/d). Treatment is well tolerated, patient is with no significant disability. Patient 2 is a 52 year old woman treated for Hashimoto´s thyroiditis, diagnosed with SPS variant- Stiff limb syndrome affected her right upper limb. The symptomatic therapy with baclofen (30mg/d) and diazepam (3x5mg) was not tolerated, plasma exchange showed no benefit. We started treatment with IVIG (0,4g/kg/day given over 5 days) with significant benefit. Afterwards we started chronic treatment with azathioprine (100mg/d) and methylprednisolone (16mg/d). Treatment is well tolerated with good clinical benefit with no need to repeat IVIG therapy.
Conclusions: SPS is a rare disorder, often misdiagnosed, highly heterogeneous in its manifestation. If left untretaded it could lead to serious disability or eventually to death. Treatment is difficult and change from case to case.
To cite this abstract in AMA style:
P. Dosekova, G. Krastev, J. Haring, R. Lackovic. Stiff person syndrome [abstract]. Mov Disord. 2016; 31 (suppl 2). https://www.mdsabstracts.org/abstract/stiff-person-syndrome/. Accessed May 16, 2025.« Back to 2016 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/stiff-person-syndrome/