ArticleOrphanet journal of rare diseases2026
Clinical spectrum, diagnosis, cognitive outcomes and therapeutic strategies in pediatric Rasmussen syndrome: a 10-year cohort from a tertiary pediatric center.
Article in Orphanet journal of rare diseases, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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.
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
0 citing papers in PubMed.
No citing paper in PubMed yet.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
10 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundRasmussen syndrome (RS) is a rare, immune-mediated, progressive epileptic encephalopathy. A comprehensive understanding of its heterogeneous clinical course, along with the identification of prognostic and early determinants, is crucial for timely intervention. To this end, we retrospectively analyzed 21 pediatric RS patients (2014-2024). Collected data encompassed epidemiology, clinical course, cognition, neuroimaging, immunology, histopathology, and treatment. Primary outcomes included the time from onset to the appearance of different clinical symptoms and cerebral atrophy. Cognitive decline was assessed through longitudinal Full-Scale IQ (FSIQ) measurements.
resultsMean age at onset was 7.3 years; 52.4% were < 8 years. All patients presented with epilepsy as their initial manifestation and age at onset correlated with time to cognitive decline (r = 0.578, p = 0.049), motor symptoms (r = 0.568, p = 0.034) and epilepsia partialis continua (EPC) (r = 0.765, p = 0.004), identifying an age of 8 years as a cut-off to predict a different disease course. EPC occurred in 57.1% (median: 6.5 months) and its early onset was associated with a precocious appearance of motor deficit (r = 0.863, p < 0.001) and cerebral atrophy (r = 0.753, p = 0.007). The severity of cognitive decline was not related to age at disease onset, rapidity of development of cognitive impairment or baseline cognitive status. Four patients displayed atypical, slow-progressing forms with minimal deficits. Immunotherapy achieved partial or transient seizure control in most cases; rituximab was effective in 50%, particularly when started 2-5 years post-onset. Hemispherectomy yielded seizure freedom in 69.2%, with better postoperative FSIQ linked to higher preoperative FSIQ (r = 0.640, p = 0.025) and younger surgical age (r = -0.621, p = 0.003).
conclusionsAge at onset is a key prognostic factor in RS. The development of epilepsy and EPC, cognitive and motor impairment show a strong temporal association. The degree of cognitive decline does not parallel other clinical markers. Post-surgical cognitive outcomes are better in patients operated at a younger age and with greater cognitive preservation. We propose a clinical framework integrating immunotherapy selection and surgical planning to optimize outcomes and guide patient counseling.
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
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.