SynthesisEpilepsia2026
Epilepsy in dentatorubral-pallidoluysian atrophy: A systematic review and meta-analysis.
Synthesis in Epilepsia, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper, 1 of them a synthesis that pooled it.
What it found
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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.
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.
Who cites it
1 citing paper in PubMed, 1 synthesis or guideline pooled it.
- Epilepsy in dentatorubral-pallidoluysian atrophy: A systematic review and meta-analysis.Epilepsia · 2026Pooled it
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
11 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveDentatorubral-pallidoluysian atrophy (DRPLA) is a rare autosomal dominant neurodegenerative disease caused by a CAG repeat expansion in the ATN1 gene. The juvenile onset type often presents with epilepsy, including progressive myoclonic epilepsy (PME). However, evidence on epilepsy in DRPLA remains limited. This systematic review and meta-analysis aimed to summarize clinical characteristics of DRPLA-related epilepsy.
methodsWe systematically searched MEDLINE (PubMed), CENTRAL, Embase, Ichushi, and ClinicalTrials.gov for studies on DRPLA-related epilepsy, following PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. The review protocol was registered with the Open Science Framework. Any study design reporting at least one case of DRPLA-related epilepsy was eligible, including case reports, case series, cohort studies, and clinical trials. Eligible studies underwent screening and full-text assessment, followed by inclusion in descriptive and meta-analytic syntheses. Meta-analyses included only studies reporting ≥5 DRPLA patients.
resultsA total of 181 studies encompassing 1191 patients met the eligibility criteria. DRPLA patients with epilepsy had a younger onset age (16.9 [95% confidence interval (CI) = 13.76-20.76] vs. 45.5 years [95% CI = 42.77-48.47]) and more CAG repeats (66.7 [95% CI = 63.63-69.84] vs. 59.2 [95% CI = 55.67-62.92]) than those without epilepsy. DRPLA patients with epilepsy showed a higher likelihood of paternal versus maternal inheritance (odds ratio = 2.47 [95% CI = .97-6.27]). Focal seizures were frequently observed (40.0%-76.5%) alongside myoclonic and generalized tonic-clonic seizures. Electroencephalographic findings included slow bursts (38.0%), photoparoxysmal responses (36.6%), and interictal epileptiform discharges (77.5%). Giant somatosensory-evoked potentials, typically seen in PME, were observed in only two patients and absent in 27. Among antiseizure medications, perampanel and levetiracetam were more frequently reported as effective than sodium channel blockers. SIGNIFICANCE: This review synthesizes fragmented evidence on DRPLA-related epilepsy and highlights key clinical and electrophysiological patterns. Despite limitations from small-scale studies, these findings support more informed clinical care and underscore the need for larger cohort studies.
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