Evidence map›Paper›PMID 26989571›Full record

ReviewTremor and other hyperkinetic movements (New York, N.Y.)2016

Clinical Spectrum of Stiff Person Syndrome: A Review of Recent Reports.

Harini Sarva, Andres Deik, Aman Ullah, William L Severt

Open access · greenAbstract readReview
In one paragraph

Review in Tremor and other hyperkinetic movements (New York, N.Y.), 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 40 papers.

0numbers the graph read from it
0cells of the map it votes in
40citing papers in PubMed
3.8field-weighted citation impact, top 7% of its field
1 · What the graph read from 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.

2 · The registry

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.

3 · Its place in the literature

Who cites it

40 citing papers in PubMed, 66 citations in OpenAlex.

  1. Article
  2. Review
  3. Status Spasticus in Stiff-Person Syndrome.Annals of Indian Academy of Neurology · 2026
    Article
  4. Review
  5. Article
  6. Article
  7. Article
  8. Review
  9. Autoimmune diseases of the spine and spinal cord.The neuroradiology journal · 2024
    Review
  10. Article
  11. Review
  12. Paraneoplastic neurological syndrome: growing spectrum and relevance.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2022
    Review
  13. Article
  14. Article
  15. Review
  16. Clinical spectrum of high-titre GAD65 antibodies.Journal of neurology, neurosurgery, and psychiatry · 2021
    Article
  17. Article
  18. Article
  19. Article
  20. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

4 authors at 1 institution in 1 country.

Harini SarvaDepartment of Neurology, Maimonides Medical Center, Brooklyn, NY, USA.
Andres DeikParkinson Disease and Movement Disorders Center, Department of Neurology, University of Pennsylvania, PA, USA.
Aman UllahDepartment of Neurology, Maimonides Medical Center, Brooklyn, NY, USA.
William L SevertDepartment of Neurology, Division of Movement Disorders, Icahn School of Medicine at Mount Sinai, Mount Sinai Beth Israel Medical Center, New York, NY, USA.
Maimonides Medical Center · US

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

background"Classic" stiff person syndrome (SPS) features stiffness, anti-glutamic acid decarboxylase (anti-GAD) antibodies, and other findings. Anti-GAD antibodies are also detected in some neurological syndromes (such as ataxia) in which stiffness is inconsistently present. Patients with otherwise "classic" SPS may either lack anti-GAD antibodies or be seropositive for others. Hence, SPS cases appear to fall within a clinical spectrum that includes conditions such as progressive encephalomyelitis with rigidity and myoclonus (PERM), which exhibits brainstem and autonomic features. We have compiled herein SPS-spectrum cases reported since 2010, and have segregated them on the basis of likely disease mechanism (autoimmune, paraneoplastic, or cryptogenic) for analysis.

methodsThe phrases "stiff person syndrome", "PERM", "anti-GAD antibody syndrome", and "glycine receptor antibody neurological disorders" were searched for in PubMed in January 2015. The results were narrowed to 72 citations after excluding non-English and duplicate reports. Clinical descriptions, laboratory data, management, and outcomes were categorized, tabulated, and analyzed.

resultsSixty-nine autoimmune, 19 paraneoplastic, and 13 cryptogenic SPS-spectrum cases were identified. SPS was the predominant diagnosis among the groups. Roughly two-thirds of autoimmune and paraneoplastic cases were female. Anti-GAD antibodies were most frequently identified, followed by anti-amphiphysin among paraneoplastic cases and by anti-glycine receptor antibodies among autoimmune cases. Benzodiazepines were the most commonly used medications. Prognosis seemed best for cryptogenic cases; malignancy worsened that of paraneoplastic cases. DISCUSSION: Grouping SPS-spectrum cases by pathophysiology provided insights into work-up, treatment, and prognosis. Ample phenotypic and serologic variations are present within the categories. Ruling out malignancy and autoimmunity is appropriate for suspected SPS-spectrum cases.

Indexed as

anti-glutamic acid decarboxylase antibodiesanti-glycine receptor antibodiesprogressive encephalomyelitis with rigidity and myoclonusstiff limb syndromeStiff person syndromestiff trunk syndrome

Identifiers

PMID26989571
PMCPMC4790195
OpenAlexW2292622929

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC-ND
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Registered trials

None linked

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.