制度・支援
指定難病 — No.24

亜急性硬化性全脳炎

検索語 Subacute Sclerosing Panencephalitis ・ 最終更新 2026-09-17 15:45 ・ 最新に更新

Data Sheet
指定 No.24
Src PubMed · CT.gov · jRCT

これは医療アドバイスではありません。診断・治療の判断は必ず主治医にご相談ください。論文や治験は「今わかっている研究の状況」を示すもので、効果を保証するものではありません。

( 01 )EVIDENCE / PUBMED · 5件

世界の論文

直近の研究を、やさしい日本語で

各論文の見出しにある「確からしさ」は、その研究がどれくらい信頼できるかの目安です。「理論段階」はまだ仮説に近く、下にいくほど多くの患者で検証されていて、「メタ解析」がもっとも信頼できます。

不明
MK-01 · PMID 42720737

Reproducibility and validity failures in systematic review deduplication tools: evaluation on a paediatric neuroradiology corpus

Abstract / 原文

BACKGROUND: Deduplication is usually treated as a routine preprocessing step in systematic reviews, yet errors introduced at this stage are not visible in Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) flow diagrams and can propagate through evidence synthesis. OBJECTIVE: To evaluate the validity and reproducibility of commonly used deduplication tools in a paediatric neuroradiology systematic review. MATERIALS AND METHODS: PubMed and Embase were searched for peer-reviewed literature on paediatric subacute sclerosing panencephalitis neuroimaging, yielding 603 records. Two reviewers independently deduplicated the combined corpus using Zotero (version 8.0.4) and Rayyan (updated 21 January 2026). A manual reference standard was established by dual-reviewer adjudication of potential duplicate pairs using title, publication year, author list, and Digital Object Identifier (DOI). Tool outputs were compared against this reference standard for false-positive and false-negative decisions and for reproducibility across runs. RESULTS: Manual adjudication identified 120 true duplicate records, leaving 483 unique records. Zotero produced identical outputs across reviewers, indicating deterministic behaviour, but generated 16 false-positive duplicate decisions and 5 false negatives, predominantly related to DOI collision. Rayyan identified more true duplicates but produced non-identical outputs across independent runs, with 16-17 false positives and 1 false negative per run. CONCLUSION: Commonly used deduplication tools can introduce either deterministic bias or non-deterministic record loss before screening begins. In paediatric radiology evidence synthesis, the deduplication software version and verification strategy should be explicitly reported. Auditable secondary checks should be considered before records are removed.

Journal
Pediatric radiology(2026 Sep)
Authors
5名
Type
Journal Article
PubMedで原文を見る
不明
MK-02 · PMID 42685800

Eyelid myoclonus in Subacute Sclerosing Panencephalitis - A newly recognised slow myoclonic pattern?

Journal
Journal of movement disorders(2026 Sep)
Authors
8名
Type
Journal Article
PubMedで原文を見る
不明
MK-03 · PMID 42673760

When the voice follows the jerk: Periodic vocalizations in subacute sclerosing panencephalitis

Abstract / 原文

Subacute sclerosing panencephalitis (SSPE) is a progressive neurological complication of measles infection and movement disorders are commonly seen in this condition. We describe an 11-year-old boy with SSPE, with mixed movement phenomenology, including dystonia, slow as well as brief myoclonus, repetitive behaviours, and peculiar periodic vocalizations, which followed the slow myoclonic jerks, and discuss possible mechanisms.

Journal
Parkinsonism & related disorders(2026 Aug)
Authors
9名
Type
Letter
PubMedで原文を見る
観察研究
MK-04 · PMID 42655637

Measles: A Narrative Review of Current Therapeutic Options and Implications for the Future

Abstract / 原文

Despite the availability of a safe and effective measles vaccine for several decades, measles remains a significant cause of morbidity and mortality worldwide, causing 95,000 deaths in 2024 alone. Currently, there is no licensed therapeutic agent against measles, and treatment is only supportive. This is a review of articles published from 2015 to 3 June 2025, on therapeutic agents used or under investigation against measles. Ribavirin has been used in severe measles cases; nevertheless, there are no randomized clinical trials so far. New delivery systems, such as polymer-based formulations and nanoparticles, have markedly increased ribavirin's efficacy, while cytotoxicity remained low. Viral membrane fusion (F) protein inhibitors and viral RNA polymerase inhibitors are promising next-generation therapeutics against measles and have been tested in animal models with good results. Lastly, interferons (IFNs) are key mediators of the innate immune response against measles, particularly for controlling brain infection. There are few reports indicating that repeated remdesivir courses, as well as intraventricular or intrathecal ribavirin or IFN, may temporarily stabilize the neurologic status of patients with subacute sclerosing panencephalitis. Intracranial administration of protein F inhibitors also demonstrated promising results in mice. Although several antiviral agents and natural products exhibit in vitro efficacy against measles virus, further research is required to determine their safety, pharmacological properties, and clinical efficacy before they can be considered therapeutic options. Overall, the development of antiviral agents against measles has been extremely slow. Therapeutic agents against measles are urgently needed.

Journal
Viruses(2026 Jul)
Authors
3名
Type
Journal Article, Review
PubMedで原文を見る
不明
MK-05 · PMID 42622797

Barking Tics in Subacute Sclerosing Panencephalitis: A Rare Case Report

Journal
Annals of Indian Academy of Neurology(2026 May)
Authors
4名
Type
Journal Article
PubMedで原文を見る
( 02 )TRIALS / JAPAN · 0件

日本で参加できる治験

現在 募集中のもの

日本で現在募集中の治験は見つかりませんでした。下の公式レジストリで条件を変えると見つかる場合があります。
( 03 )REGISTRY / jRCT

治験をもっと探す

日本の公式レジストリで全件を確認

上の一覧は ClinicalTrials.gov の一部です。日本国内の治験の多くは、日本の公式レジストリ jRCT にのみ登録されています。下記から最新の全件を確認できます。

jRCT で検索日本の臨床研究実施計画 公開システム「対象疾患名」に 亜急性硬化性全脳炎 を入力し、「募集状況」で 募集中 にチェックして検索します。ClinicalTrials.gov で全件を見る世界最大の治験データベース(英語)「亜急性硬化性全脳炎・日本・募集中」の条件で一覧が開きます。

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