Evidence map›Paper›PMID 38078976›Full record

Observational studyJournal of neurology2024

Expanding clinical profiles and prognostic markers in stiff person syndrome spectrum disorders.

Yujie Wang, Chen Hu, Salman Aljarallah, Maria Reyes Mantilla, Loulwah Mukharesh, Alexandra Simpson, Shuvro Roy, Kimystian Harrison, Thomas Shoemaker, Michael Comisac and 7 more

Open access · hybridAbstract readObservational Study
PubMed Full text at PMC Publisher
In one paragraph

Observational study in Journal of neurology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
17citing papers in PubMed, 2 pooled it
3.3field-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

17 citing papers in PubMed, 2 syntheses or guidelines pooled it, 16 citations in OpenAlex.

  1. Pooled it
  2. Pooled it
  3. Article
  4. Article
  5. Review
  6. Article
  7. Long-Term Outcomes in Stiff Person Spectrum Disorder.European journal of neurology · 2026
    Article
  8. Article
  9. Article
  10. Observational
  11. Article
  12. Observational
  13. Article
  14. Article
  15. Article
  16. Article
  17. 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

17 authors at 1 institution in 1 country.

Yujie WangDivision of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, 600 N Wolfe St, Pathology 627, Baltimore, MD, 21287, USA.
Chen HuDivision of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, 600 N Wolfe St, Pathology 627, Baltimore, MD, 21287, USA.
Salman AljarallahDivision of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, 600 N Wolfe St, Pathology 627, Baltimore, MD, 21287, USA.
Maria Reyes MantillaDivision of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, 600 N Wolfe St, Pathology 627, Baltimore, MD, 21287, USA.
Loulwah MukhareshDivision of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, 600 N Wolfe St, Pathology 627, Baltimore, MD, 21287, USA.
Alexandra SimpsonDivision of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, 600 N Wolfe St, Pathology 627, Baltimore, MD, 21287, USA.
Shuvro RoyDivision of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, 600 N Wolfe St, Pathology 627, Baltimore, MD, 21287, USA.
Kimystian HarrisonDivision of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, 600 N Wolfe St, Pathology 627, Baltimore, MD, 21287, USA.
Thomas ShoemakerDivision of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, 600 N Wolfe St, Pathology 627, Baltimore, MD, 21287, USA.
Michael ComisacDivision of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, 600 N Wolfe St, Pathology 627, Baltimore, MD, 21287, USA.
Alexandra BalshiDivision of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, 600 N Wolfe St, Pathology 627, Baltimore, MD, 21287, USA.
Danielle ObandoDivision of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, 600 N Wolfe St, Pathology 627, Baltimore, MD, 21287, USA.
Daniela A Pimentel MaldonadoDivision of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, 600 N Wolfe St, Pathology 627, Baltimore, MD, 21287, USA.
Jacqueline KoshorekDivision of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, 600 N Wolfe St, Pathology 627, Baltimore, MD, 21287, USA.
Sarah SnoopsDivision of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, 600 N Wolfe St, Pathology 627, Baltimore, MD, 21287, USA.
Kathryn C FitzgeraldDivision of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, 600 N Wolfe St, Pathology 627, Baltimore, MD, 21287, USA.
Scott D NewsomeDivision of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, 600 N Wolfe St, Pathology 627, Baltimore, MD, 21287, USA. snewsom2@jhmi.edu.ORCID http://orcid.org/0000-0002-5284-4681
Johns Hopkins University · US

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectiveTo describe the clinical features of a cohort of individuals with stiff person syndrome spectrum disorders (SPSD) and identify potential early predictors of future disability.

backgroundThere is a need to better understand the full spectrum of clinical and paraclinical features and long-term impact of SPSD. DESIGN/

methodsObservational study from 1997 to 2022 at Johns Hopkins. Clinical phenotypes included classic SPS, partial SPS (limb or trunk limited), SPS-plus (classic features plus cerebellar/brainstem involvement), and progressive encephalomyelitis with rigidity and myoclonus (PERM). Outcome measures were modified Rankin scale (mRS) and use of assistive device for ambulation. Multivariate logistic regression was used to assess significant predictors of outcomes.

resultsCohort included 227 individuals with SPSD with mean follow-up of 10 years; 154 classic, 48 SPS-plus, 16 PERM, and 9 partial. Mean age at symptom onset was 42.9 ± 14.1 years, majority were white (69.2%) and female (75.8%). Median time to diagnosis was 36.2 months (longest for SPS-plus and PERM) and 61.2% were initially misdiagnosed. Most had systemic co-morbidities and required assistive devices for ambulation. Female sex (OR 2.08; CI 1.06-4.11) and initial brainstem/cerebellar involvement (OR 4.41; CI 1.63-14.33) predicted worse outcome by mRS. Older age at symptom onset (OR 1.04; CI 1.01-1.06), female sex (OR 1.99; CI 1.01-4.01), Black race (OR 4.14; CI 1.79-10.63), and initial brainstem/cerebellar involvement (OR 2.44; CI 1.04-7.19) predicted worse outcome by use of assistive device. Early implementation of immunotherapy was associated with better outcomes by either mRS (OR 0.45; CI 0.22-0.92) or use of assistive device (OR 0.79; CI 0.66-0.94).

conclusionsWe present the expanding phenotypic variability of this rare spectrum of disorders and highlight potential predictors of future disability.

Indexed as

MyoclonusStiff-Person SyndromeComorbidityFemaleHumansOutcome Assessment, Health CarePrognosisAnti-GAD65Progressive encephalomyelitis with rigidity and myoclonusStiff limb syndromeStiff person syndrome

Identifiers

PMID38078976
PMCPMC10973082
OpenAlexW4389564218

What OpenQuestion holds

Textmetadata
LicenceCC BY
Read underepoch 390

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.