Evidence map›Paper›PMID 29383515›Full record

ArticleJournal of neurology2018

Diffusion tensor imaging in metachromatic leukodystrophy.

Diane F van Rappard, Marsh Königs, Marjan E Steenweg, Jaap Jan Boelens, Jaap Oosterlaan, Marjo S van der Knaap, Nicole I Wolf, Petra J W Pouwels

Abstract read
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In one paragraph

Article in Journal of neurology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.

0numbers the graph read from it
0cells of the map it votes in
13citing papers in PubMed
–field-weighted citation impact
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

13 citing papers in PubMed.

  1. Article
  2. Review
  3. Observational
  4. Article
  5. Article
  6. Leukodystrophy Imaging: Insights for Diagnostic Dilemmas.Medical sciences (Basel, Switzerland) · 2024
    Review
  7. Article
  8. Article
  9. Article
  10. Article
  11. Article
  12. Review
  13. 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

8 authors.

Diane F van RappardDepartment of Pediatric Neurology, Center for Childhood White Matter Disorders, VU University Medical Center, Amsterdam, The Netherlands.
Marsh KönigsClinical Neuropsychology Section, FGB VU University, Amsterdam, The Netherlands.
Marjan E SteenwegDepartment of Pediatric Neurology, Center for Childhood White Matter Disorders, VU University Medical Center, Amsterdam, The Netherlands.
Jaap Jan BoelensDepartment of Pediatrics, Blood and Marrow Transplantation Program, University Medical Center Utrecht, Utrecht, The Netherlands.
Jaap OosterlaanClinical Neuropsychology Section, FGB VU University, Amsterdam, The Netherlands.
Marjo S van der KnaapDepartment of Pediatric Neurology, Center for Childhood White Matter Disorders, VU University Medical Center, Amsterdam, The Netherlands.
Nicole I WolfDepartment of Pediatric Neurology, Center for Childhood White Matter Disorders, VU University Medical Center, Amsterdam, The Netherlands.
Petra J W PouwelsAmsterdam Neuroscience, VU University Medical Center Amsterdam, Academic Medical Center, VU University Amsterdam and University of Amsterdam, Amsterdam, The Netherlands. pjw.pouwels@vumc.nl.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectiveWe aimed to gain more insight into the pathomechanisms of metachromatic leukodystrophy (MLD), by comparing magnitude and direction of diffusion between patients and controls at diagnosis and during follow-up.

methodsFour late-infantile, 16 juvenile and 8 adult onset MLD patients [of which 13 considered eligible for hematopoietic cell transplantation (HCT)] and 47 controls were examined using diffusion tensor imaging. Fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD) and radial diffusivity (RD) were quantified and compared between groups using tract-based spatial statistics (TBSS). Diffusion measures were determined for normal-appearing white matter (NAWM), corpus callosum, thalamus (all based on subject-wise segmentation), and pyramidal tracts, determined with probabilistic tractography. Measures were compared between HCT-eligible patients, non-eligible patients and controls using general linear model and nonparametric permutation analyses (randomise) for TBSS data, considering family-wise error corrected p < 0.05 significant.

resultsThroughout white matter (WM), FA was decreased and MD and RD increased in both patient groups compared to controls, while AD was decreased in NAWM and corpus callosum. In the thalamus, no differences in FA were observed, but all diffusivities were increased in both patient groups. Differences were most pronounced between controls and patients non-eligible for HCT. Longitudinally (median follow-up 3.9 years), diffusion measures remained relatively stable for HCT-treated patients, but were progressively abnormal for non-eligible patients.

interpretationThe observed diffusion measures confirm that brain microstructure is changed in MLD, reflecting different pathological processes including loss of myelin and sulfatide accumulation. The observation of both increased and decreased AD probably reflects a balance between myelin and axonal loss vs. intracellular sulfatide storage in macrophages, depending on region and disease stage.

Indexed as

Diffusion Magnetic Resonance ImagingDiffusion Tensor ImagingAdolescentAdultBrainChildChild, PreschoolDisease ProgressionFemaleFollow-Up StudiesHumansImage Interpretation, Computer-AssistedLeukodystrophy, MetachromaticLinear ModelsLongitudinal StudiesMaleAxial diffusivityDiffusion weighted imagingRadial diffusivitySulfatide storageWhite matter disorder

Identifiers

PMID29383515
PMCPMC5834549

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Registered trials

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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.