ArticleBMC neurology2019
Quantitative clinical and autoimmune assessments in stiff person syndrome: evidence for a progressive disorder.
Article in BMC neurology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 45 papers, 1 of them a synthesis that pooled it.
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Who cites it
45 citing papers in PubMed, 1 synthesis or guideline pooled it, 105 citations in OpenAlex.
- Comorbid autoimmune disease in stiff-person syndrome spectrum disorder: a systematic review and meta-analysis.Journal of neurology · 2026Pooled it
- Long-Term Outcomes in Stiff Person Spectrum Disorder.European journal of neurology · 2026Article
- Article
- Review
- Status Spasticus in Stiff-Person Syndrome.Annals of Indian Academy of Neurology · 2026Article
- Promising Effects of CAR T-Cell Therapy in Refractory Stiff Person Syndrome and a Hopeful Future for All Neuroautoimmunities.Neurology(R) neuroimmunology & neuroinflammation · 2026Review
- Sequential efgartigimod and ofatumumab for stiff-person syndrome: a case report illustrating a pathophysiology-informed strategy and literature review.Frontiers in immunology · 2026Review
- Oculomotor Abnormalities in Anti-Glutamic Acid Decarboxylase-Positive Stiff Person Syndrome.Neurology international · 2025Article
- Anti-CD19 CAR T-Cell Therapy in Advanced Stiff-Person Syndrome and Concomitant Myasthenia Gravis.Neurology(R) neuroimmunology & neuroinflammation · 2025Article
- Core diagnostic features of stiff person syndrome: insights from a case-control study.Journal of neurology · 2025Article
- Clinical Features and Outcomes of Glutamic Acid Decarboxylase-65 Antibody-Associated Pure Cerebellar Ataxia and Stiff Person Syndrome Spectrum Disorders: A Single-Center Cohort Study.European journal of neurology · 2025Observational
- Botulinum toxin in stiff person syndrome: how useful is it in helping muscle stiffness and spasms?Therapeutic advances in neurological disorders · 2025Article
- Supranuclear Vertical Gaze Palsy in Movement Disorders.Neuro-ophthalmology (Aeolus Press) · 2025Review
- Case Report: Suspected "stiff dog syndrome" in a Maltese dog.Frontiers in veterinary science · 2025Article
- Recent advances in autoimmune encephalitis.Arquivos de neuro-psiquiatria · 2024Review
- Stiff-person syndrome and related disorders - diagnosis, mechanisms and therapies.Nature reviews. Neurology · 2024Review
- Stiff Person Syndrome With Positive Anti-glutamic Acid Decarboxylase (GAD) Autoantibodies.Cureus · 2024Article
- Expanding clinical profiles and prognostic markers in stiff person syndrome spectrum disorders.Journal of neurology · 2024Observational
- From Wheelchair Bound to Working: A Case Study of Intravenous Ketamine Infusions in Treating Stiff Person Syndrome.Cureus · 2024Article
- Successful Autologous Hematopoietic Stem Cell Transplant in Glycine Receptor Antibody-Positive Stiff Person Syndrome: A Case ReportNeurology(R) neuroimmunology & neuroinflammation · 2024Article
Corrections and comments
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Authors and funding
3 authors at 2 institutions in 2 countries.
Funding
Abstract
backgroundStiff Person Syndrome (SPS) is an under-diagnosed disorder that affects mobility and the quality of life of affected patients. The aim of the study is to describe the natural history of SPS, the extent of accumulated disability and the associated clinical and immunological features in patients followed for up to 8 years in a single center.
methodsOur collective cohort included 57 SPS patients. Additionally, 32 of these patients were examined every 6 months for a two-year period in a longitudinal study protocol, to assess disease progression using quantitative measures of stiffness and heightened sensitivity.
resultsThe most frequent initial symptom was leg stiffness, followed by paraspinal muscle rigidity and painful spasms in 95% of the patients. Although none of the patients required assistance for ambulation during the first 2 years of disease onset, 46 patients (80%) lost the ability to walk independently during our follow-up, despite symptomatic medications. In the longitudinal cohort, the number of stiff areas increased (p < 0.0001), consistent with worsening functional status and quality of life. High-titer anti-GAD antibodies were present in serum and CSF with elevated intrathecal GAD-specific IgG synthesis, but they did not correlate with clinical severity or progression.
conclusionsThis large study on SPS patients, combining an eight-year follow-up at a single center by the same leading neurologist and his team, is the first to provide longitudinal data in a large patient subgroup using objective clinical measures. One of the main findings is that SPS is a progressive disease leading to physical disability over time.
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Registered trials
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