Evidence map›Paper›PMID 39019606›Full record

ArticleInternal medicine (Tokyo, Japan)2025

Histopathological Investigation of Progressive Encephalomyelitis with Rigidity and Myoclonus: An Autopsy Case Characterized by Oculomotor Dysfunction and Autonomic Failure.

Hiroyuki Yabata, Ryutaro Nakamura, Seiji Sugiyama, Yoshitaka Tamaki, Isamu Yamakawa, Shiori Onoda, Hirohito Ishigaki, Toshimasa Ikeda, Akio Akagi, Yasushi Itoh and 4 more

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PubMed Full text at PMC Publisher
In one paragraph

Article in Internal medicine (Tokyo, Japan), 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed, 1 pooled it
–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

2 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. Pooled it
  2. 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

14 authors.

Hiroyuki YabataDepartment of Neurology, Shiga University of Medical Science, Japan.
Ryutaro NakamuraDepartment of Neurology, Shiga University of Medical Science, Japan.
Seiji SugiyamaDepartment of Neurology, Shiga University of Medical Science, Japan.
Yoshitaka TamakiDepartment of Neurology, Shiga University of Medical Science, Japan.
Isamu YamakawaDepartment of Neurology, Shiga University of Medical Science, Japan.
Shiori OnodaDivision of Diagnostic Pathology, Shiga University of Medical Science Hospital, Japan.
Hirohito IshigakiDepartment of Pathology, Shiga University of Medical Science, Japan.
Toshimasa IkedaDepartment of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Japan.
Akio AkagiDepartment of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Japan.
Yasushi ItohDepartment of Pathology, Shiga University of Medical Science, Japan.
Ryoji KushimaDivision of Diagnostic Pathology, Shiga University of Medical Science Hospital, Japan.
Mari YoshidaDepartment of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Japan.
Yasushi IwasakiDepartment of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Japan.
Makoto UrushitaniDepartment of Neurology, Shiga University of Medical Science, Japan.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare disease associated with the presence of anti-glycine receptor (GlyR) antibodies. We herein report an autopsy case of an 80-year-old man diagnosed with anti-GlyR antibody-positive PERM who presented with symptoms of oculomotor dysfunction and autonomic failure. Despite intensive immunotherapy, the neurological symptoms showed almost no improvement, and the patient succumbed to aspiration pneumonia and bacterial translocation. Postmortem pathology revealed mild inflammatory changes and neuronal loss that were disproportionate to a severe clinical presentation. These results suggest that the clinical symptoms of PERM may result from antibody-mediated GlyR internalization, leading to neuronal disinhibition, rather than a neuroinflammatory signature.

Indexed as

EncephalomyelitisMuscle RigidityMyoclonusOcular Motility DisordersAged, 80 and overAutoantibodiesAutopsyFatal OutcomeHumansMaleReceptors, GlycineAutoantibodiesReceptors, Glycineanti-glycine receptor antibodyautopsypathologyprogressive encephalomyelitis with rigidity and myoclonus

Identifiers

PMID39019606
PMCPMC11904467

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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.