ArticleCurrent treatment options in neurology2009
Stiff person syndrome: advances in pathogenesis and therapeutic interventions.
Article in Current treatment options in neurology, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 92 papers, 3 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
92 citing papers in PubMed, 3 syntheses or guidelines pooled it, 218 citations in OpenAlex.
- Pediatric stiff-person syndrome and related disorders: A systematic review.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025Pooled it
- Rituximab in stiff-person syndrome with glutamic acid decarboxylase 65 autoantibody: a systematic review.Journal of neurology · 2025Pooled it
- Pooled it
- A double-blind, placebo-controlled study of rituximab in patients with stiff person syndrome.Annals of neurology · 2017Trial
- Stiff-Person Syndrome: A Comprehensive Review of a Rare Disease with Emphasis on Pathophysiology, Clinical Features, Diagnostic Challenges and Therapeutic Advances.The Yale journal of biology and medicine · 2026Review
- Graph-Theoretical Approach for Predicting Physicochemical Properties of Stiff-Person Syndrome Drugs.ChemistryOpen · 2026Article
- A Pediatric Case of Stiff-Person Syndrome: Presentation and Comparative Analysis.Journal of orthopaedic case reports · 2026Article
- Sequential efgartigimod and ofatumumab for stiff-person syndrome: a case report illustrating a pathophysiology-informed strategy and literature review.Frontiers in immunology · 2026Review
- Stiff Person Syndrome: A Case Report.Cureus · 2025Article
- Hung-up reflex: the history of a clinical sign and differential diagnosis.Acta neurologica Belgica · 2025Review
- Autologous hematopoietic stem cell transplantation in severe, refractory stiff person syndrome: a case series.Bone marrow transplantation · 2025Article
- Association of type 1 stiff-person syndrome and insulinoma: a case report and narrative review.Hormones (Athens, Greece) · 2025Review
- Exploring human plasma proteomic variations in mucolipidosis type IV.Molecular therapy. Methods & clinical development · 2025Article
- Core diagnostic features of stiff person syndrome: insights from a case-control study.Journal of neurology · 2025Article
- Neurophysiological Insights into the Pathophysiology of Stiff-Person Spectrum Disorders.Movement disorders clinical practice · 2025Review
- STIFF PERSON SYNDROME-MANAGING A RARE AUTOIMMUNE DISORDER IN A LOW RESOURCE SETTING: A CASE REPORT.Annals of Ibadan postgraduate medicine · 2025Article
- B-cell immune repertoire analysis of autoimmune neurological syndromes with anti-GAD65 antibodies.Frontiers in neurology · 2025Article
- Stiff-person syndrome and related disorders - diagnosis, mechanisms and therapies.Nature reviews. Neurology · 2024Review
- Atraumatic hip fracture due to stiff person syndrome: Case report and literature review.International journal of surgery case reports · 2024Article
- Machine learning characterization of a rare neurologic disease via electronic health records: a proof-of-principle study on stiff person syndrome.BMC neurology · 2024Article
32 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
1 author at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Stiff person syndrome (SPS) varies from mild to severe, but if untreated it can be progressive and disabling. Although progress has been made in understanding and treating SPS, the disease remains underdiagnosed, delaying treatment. Antibodies against glutamic acid decarboxylase provide an excellent diagnostic marker, but their role in disease pathogenesis is uncertain. Research focused on identifying new autoantigens has provided evidence that gamma-aminobutyric acid (GABA)(A) receptor-associated protein (GABARAP), a 14-kD protein localized at the postsynaptic regions of GABAergic synapses, is an antigenic target. Circulating anti-GABARAP antibodies that inhibit GABA(A) receptor expression on GABAergic neurons have been found in up to 65% of SPS patients. The impairment of GABAergic pathways and reduction of brain GABA results in clinical manifestations of stiffness, spasms, and phobias. Increased awareness of SPS among practicing physicians is necessary to recognize the disease early and prevent permanent disability. Most patients with SPS respond to GABA-enhancing drugs, but the high doses required cause unacceptable adverse effects. The disease clearly responds to intravenous immunoglobulin, but repeated infusions are needed to maintain response. New immunomodulating agents are being explored to treat difficult cases and to induce long-lasting remissions.
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.