Evidence map›Paper›PMID 33042921›Full record

ArticleFrontiers in pediatrics2020

Fatal Progressive Meningoencephalitis Diagnosed in Two Members of a Family With X-Linked Agammaglobulinemia.

Yasushi Kasahara, Masaru Imamura, Chansu Shin, Hiroshi Shimizu, Jirou Utsumi, Ryosuke Hosokai, Haruko Iwabuchi, Takayuki Takachi, Akiyoshi Kakita, Hirokazu Kanegane and 2 more

Abstract readCase Reports
In one paragraph

Article in Frontiers in pediatrics, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.

0numbers the graph read from it
0cells of the map it votes in
5citing 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

5 citing papers in PubMed.

  1. Review
  2. Revisiting X-linked agammaglobulinemia.Journal of human immunity · 2025
    Review
  3. Article
  4. X-linked agammaglobulinaemic progressive encephalopathy mimicking mitochondrial encephalomyopathy-latic axidosis-stroke like episode.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2024
    Article
  5. 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

12 authors.

Yasushi KasaharaDepartment of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
Masaru ImamuraDepartment of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
Chansu ShinDepartment of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
Hiroshi ShimizuDepartment of Pathology, Brain Research Institute, Niigata University, Niigata, Japan.
Jirou UtsumiDepartment of Pediatrics, Niigata Cancer Center Hospital, Niigata, Japan.
Ryosuke HosokaiDepartment of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
Haruko IwabuchiDepartment of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
Takayuki TakachiDepartment of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
Akiyoshi KakitaDepartment of Pathology, Brain Research Institute, Niigata University, Niigata, Japan.
Hirokazu KaneganeDepartment of Pediatrics, Graduate School of Medicine, University of Toyama, Toyama, Japan.
Akihiko SaitohDepartment of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
Chihaya ImaiDepartment of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Chronic enteroviral meningoencephalitis is a well-known complication in patients with X-linked agammaglobulinemia (XLA). However, progressive neurodegenerative disorders or chronic neuroinflammatory diseases with no causative microorganisms have been recognized as rare central nervous system (CNS) complications in XLA. We herein report a family in which two of three members with XLA had developed progressive meningoencephalitis with an unknown etiology. A 15-month-old male infant presented with left-sided ptosis. Initially, the family denied any family history of inherited diseases, but later disclosed a family history of agammaglobulinemia previously diagnosed in two family members. In the early 1980s, one of the elder brothers of the index patient's mother who had been treated with intramuscular immunoglobulin [or later intravenous immunoglobulin (IVIG)] for agammaglobulinemia deceased at 10 years of age after showing progressive neurological deterioration during the last several years of his life. The index patient was diagnosed with XLA caused by Bruton tyrosine kinase deficiency (654delG; Val219Leufs

Indexed as

autoimmunityBruton tyrosine kinaseneurodegenerationprogressive meningoencephalitisX-linked agammaglobulinemia

Identifiers

PMID33042921
PMCPMC7530192

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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.