Stiff-person syndrome (SPS) is a rare and complex neurologic disorder characterized by progressive muscle stiffness, painful spasms, and gait difficulties. In this report, we describe a case of SPS who presented with a relapse while on maintenance immunosuppressive treatment. In addition, we review the literature of 16 previously reported cases of SPS from India, highlighting the diverse clinical features, comorbidities, treatment response, and relapse. The occurrence of paraneoplastic SPS emphasizes the need for early recognition and diagnosis.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11418777PMC
http://dx.doi.org/10.4103/aian.aian_92_24DOI Listing

Publication Analysis

Top Keywords

stiff-person syndrome
8
expanding understanding
4
understanding stiff-person
4
syndrome insights
4
insights cases
4
cases india
4
india stiff-person
4
sps
4
syndrome sps
4
sps rare
4

Similar Publications

We describe a woman with stiff-person syndrome (SPS), whose muscle spasms resulted in sequential bilateral femoral neck fractures. Orthopaedic fixation of the first fracture was complicated by increased muscle spasm, fracture nonunion and ultimately metalwork fracture. SPS was diagnosed following the fracture of the contralateral femoral neck, neurology assessment and detection of high-titre antibodies to glutamic acid decarboxylase.

View Article and Find Full Text PDF

A patient with reactivated varicella zoster virus (VZV) manifesting in the left-sided dermatome L3 and S2-S4 developed tonic spasms which morphed into myoclonic jerks, paresis, rigidity and hypoesthesia of the left leg. Later, stimuli-sensitive myoclonus progressed to affect the upper body and was accompanied by fever surges with high-frequency myoclonus, hypertensive derailment, dysphagia and other features of the brainstem with autonomic dysfunction. Cerebrospinal fluid tested positive for VZV, MRI showed no signs of myelitis and EEG was negative for epilepsy.

View Article and Find Full Text PDF

Stiff person spectrum disorder is a disease that involves a host of conditions that are associated with glutamic acid decarboxylase 65-kilodalton isoform autoantibodies. These conditions may include diabetes mellitus type 1, pernicious anemia, autoimmune leukoencephalopathy, cerebellar ataxia, and stiff-person syndrome. Clinical recognition and diagnosis of stiff person spectrum disorder are important early, as immunologic treatment options showing reliable efficacy in slowing disease progression are available.

View Article and Find Full Text PDF
Article Synopsis
  • * The presence of anti-GAD antibodies is linked to type 1 diabetes and various neurological disorders collectively referred to as GAD antibody-spectrum disorders (GAD-SD).
  • * A case study of a 17-year-old male who developed GAD-SD and type 1 diabetes after a stem cell transplant is presented, showing symptoms like memory issues and abnormal brain scans, leading to treatment with steroids and insulin.
View Article and Find Full Text PDF

Population-Based Study of the Epidemiology of Stiff Person Syndrome in a Large Colorado-Based Health System.

Neurology

December 2024

From the Department of Neurology (P.D.C., S.S.), and Department of Biostatistics & Informatics (S.S.), University of Colorado Anschutz Medical Campus; University of Colorado School of Medicine Anschutz Medical Campus (R.D., P.W., E.E., B.V., E.M., K.V.N., A.M.C., A.L.P.); University of Colorado School of Medicine (R.F.), Aurora; School of Osteopathic Medicine (R.F.), Kanas City University, MO; University of Colorado School of Medicine Anschutz Medical Campus (R.V.C.); School of Medicine (R.V.C.), Aurora, CO; Renown Health (R.V.C.), Reno, NV; Rocky Mountain MS Center (K.V.N., A.L.P.), University of Colorado School of Medicine; and Skaggs School of Pharmacy and Pharmaceutical Sciences (K.V.N.), University of Colorado Anschutz Medical Campus, Aurora.

Article Synopsis
  • Stiff person syndrome spectrum disorder (SPSD) is a rare autoimmune condition with estimated prevalence of 1-2 cases per million, marked by muscle stiffness and painful spasms; this study aims to clarify its incidence and prevalence using data from the University of Colorado Health system.
  • A total of 273 patients were identified with potential SPSD diagnosis codes, but only 59 were confirmed to have the disorder, leading to a prevalence estimate of 2.11 cases per 100,000 persons.
  • Different clinical diagnostic criteria were assessed, revealing varying estimated prevalence rates for SPSD, indicating inconsistencies in diagnosis and classification within the population studied.
View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!