Evidence map›Paper›PMID 34106418›Full record

ArticleCerebellum (London, England)2022

Spinocerebellar Ataxia Type 1: One-Year Longitudinal Study to Identify Clinical and MRI Measures of Disease Progression in Patients and Presymptomatic Carriers.

Anna Nigri, Lidia Sarro, Alessia Mongelli, Anna Castaldo, Luca Porcu, Chiara Pinardi, Marina Grisoli, Stefania Ferraro, Laura Canafoglia, Elisa Visani and 4 more

Abstract read
PubMed Publisher
In one paragraph

Article in Cerebellum (London, England), 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.

0numbers the graph read from it
0cells of the map it votes in
21citing papers in PubMed
2.0field-weighted citation impact, top 14% 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

21 citing papers in PubMed, 23 citations in OpenAlex.

  1. Article
  2. Article
  3. Early and Progressive Spinal Cord Atrophy in Spinocerebellar Ataxia Type 1.Movement disorders : official journal of the Movement Disorder Society · 2026
    Article
  4. Review
  5. Article
  6. Article
  7. Article
  8. Article
  9. Review
  10. Article
  11. Sensitivity of Advanced Magnetic Resonance Imaging to Progression over Six Months in Early Spinocerebellar Ataxia.Movement disorders : official journal of the Movement Disorder Society · 2024
    Article
  12. Observational
  13. Article
  14. Article
  15. Article
  16. Article
  17. Article
  18. Article
  19. Electrophysiological and neuropsychological assessment of cognition in spinocerebellar ataxia type 1 patients: a pilot study.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2023
    Article
  20. Article
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

14 authors at 3 institutions in 1 country.

Anna NigriNeuroradiology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Lidia SarroMedical Genetics and Neurogenetics, Fondazione IRCCS Istituto Neurologico Carlo Besta, via Celoria 11, 20133, Milan, Italy.
Alessia MongelliMedical Genetics and Neurogenetics, Fondazione IRCCS Istituto Neurologico Carlo Besta, via Celoria 11, 20133, Milan, Italy.
Anna CastaldoMedical Genetics and Neurogenetics, Fondazione IRCCS Istituto Neurologico Carlo Besta, via Celoria 11, 20133, Milan, Italy.
Luca PorcuMethodology for Clinical Research Laboratory, Oncology Department, Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Milan, Italy.
Chiara PinardiNeuroradiology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Marina GrisoliNeuroradiology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Stefania FerraroNeuroradiology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Laura CanafogliaNeurophysiology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Elisa VisaniNeurophysiology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Maria Grazia BruzzoneNeuroradiology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Lorenzo NanettiMedical Genetics and Neurogenetics, Fondazione IRCCS Istituto Neurologico Carlo Besta, via Celoria 11, 20133, Milan, Italy.
Franco TaroniMedical Genetics and Neurogenetics, Fondazione IRCCS Istituto Neurologico Carlo Besta, via Celoria 11, 20133, Milan, Italy.
Caterina MariottiMedical Genetics and Neurogenetics, Fondazione IRCCS Istituto Neurologico Carlo Besta, via Celoria 11, 20133, Milan, Italy. caterina.mariotti@istituto-besta.it.ORCID http://orcid.org/0000-0003-2405-3564
Fondazione IRCCS Istituto Neurologico Carlo Besta · ITMario Negri Institute for Pharmacological Research · ITOspedale Martini · IT

Funding

Ministero della Salute RF-2011-02347420
6 · The paper itself

Abstract

Spinocerebellar ataxias type 1 (SCA1) is an autosomal dominant disease usually manifesting in adulthood. We performed a prospective 1-year longitudinal study in 14 presymptomatic mutation carriers (preSCA1), 11 ataxic patients, and 21 healthy controls. SCA1 patients had a median disease duration of 6 years (range 2-16) and SARA score of 7 points (range 3.5-20). PreSCA1 had an estimated time before disease onset of 9.7 years (range 4-30), and no signs of ataxia. At baseline, SCA1 patients significantly differed from controls in SARA score (Scale for Assessment and Rating of Ataxia), cognitive tests, and structural MRI measures. Significant volume loss was found in cerebellum, brainstem, basal ganglia, and cortical thinning in frontal, temporal, and occipital regions. PreSCA1 did not differ from controls. At 1-year follow-up, SCA1 patients showed significant increase in SARA score, and decreased volume of cerebellum (- 0.6%), pons (- 5.5%), superior cerebellar peduncles (- 10.7%), and midbrain (- 3.0%). Signs of disease progression were also observed in preSCA1 subjects, with increased SARA score and reduced total cerebellar volume. Our exploratory study suggests that clinical scores and MRI measures provide valuable data to monitor and quantify the earliest changes associated with the preclinical and the symptomatic phases of SCA1 disease.

Indexed as

Spinocerebellar AtaxiasAdultDisease ProgressionHumansLongitudinal StudiesMagnetic Resonance ImagingProspective StudiesHereditary ataxiasPreclinicalPresymptomatic phaseSCA1 mutation carrierStructural MRISymbol Digit Modality Test

Identifiers

PMID34106418
OpenAlexW3166942520

What OpenQuestion holds

Textmetadata
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.