Evidence map›Paper›PMID 31516623›Full record

ArticleJournal of pediatric neurosciences

Electroneurography and Advanced Neuroimaging Profile in Pediatric-onset Metachromatic Leukodystrophy.

Abhinav Raina, Sruthi S Nair, Chinmay Nagesh, Bejoy Thomas, Muralidharan Nair, Soumya Sundaram

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PubMed Full text at PMC Publisher
In one paragraph

Article in Journal of pediatric neurosciences. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
5citing papers in PubMed, 1 pooled it
0.8field-weighted citation impact, top 27% 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

5 citing papers in PubMed, 1 synthesis or guideline pooled it, 17 citations in OpenAlex.

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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

6 authors at 1 institution in 1 country.

Abhinav RainaDepartment of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.
Sruthi S NairDepartment of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.
Chinmay NageshDepartment of Imaging Sciences and Intervention Radiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.
Bejoy ThomasDepartment of Imaging Sciences and Intervention Radiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.
Muralidharan NairDepartment of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.
Soumya SundaramDepartment of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.
Sree Chitra Thirunal Institute for Medical Sciences and Technology · IN

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

contextMetachromatic leukodystrophy (MLD) is a rare autosomal-recessive disorder characterized by demyelination of central and peripheral nervous system. There is scarcity of literature on the electrophysiological aspects of peripheral nerves and the advanced neuroimaging findings in MLD.

aimThe aim was to study the nerve conduction parameters and advanced neuroimaging findings in patients with MLD. MATERIALS AND

methodsThis study is a retrospective analysis conducted, between 2005 and 2016, of 12 patients who had biochemical, histopathological, or genetic confirmation of MLD and disease onset before 18 years of age. The clinical, electroneurography, and the advanced neuroimaging findings were reviewed and analyzed. STATISTICAL ANALYSIS: The data were presented as percentages or mean ± standard deviation as defined appropriate for qualitative and quantitative variables.

resultsMean age of onset was 4.84 (±4.60) years and seven patients were males. Eight patients had juvenile MLD and four had late infantile MLD. Clinical presentation of psychomotor regression was more common in infantile MLD (75%), whereas gait difficulty (62.5%) and cognitive impairment (37.5%) were more frequent in juvenile MLD. Nerve conduction study (NCS) revealed diffuse demyelinating sensorimotor peripheral neuropathy in 9 (75%) patients. One patient had a rare presentation with conduction blocks in multiple nerves with contrast enhancement of cauda equina. Diffusion restriction involving periventricular and central white matter was seen in five patients and bilateral globus pallidi blooming was noted in three patients.

conclusionThis study highlights the utility of NCS and advanced magnetic resonance imaging sequences in the diagnosis of MLD.

Indexed as

Advanced neuroimagingconduction block• Conduction block and cauda equina enhancement are rare findings in metachromatic leukodystrophy mimicking acquired demyelinating polyradiculoneuropathies.demyelinating neuropathy• Diffusion restriction involving periventricular and central white matter and bilateral globus pallidi mineralization can be seen in metachromatic leukodystrophy.• Electroneurography can be a useful adjunct tool for the detection of demyelinating neuropathy in a case of suspected metachromatic leukodystrophy.metachromatic leukodystrophy

Identifiers

PMID31516623
PMCPMC6712919
OpenAlexW2969579796

What OpenQuestion holds

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LicenceCC BY-NC-SA
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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.