Evidence map›Paper›PMID 41729327›Full record

ArticleJournal of neurology2026

EEG, clinical, and MRI features of status epilepticus associated with mitochondrial diseases.

Maria Damiano, Virginie Lambrecq, Vi-Huong Nguyen-Michel, Clémence Marois, Benoit Rucheton, Fanny Mochel, Isabelle Gourfinkel-An, Sophie Demeret, Vincent Navarro

Abstract read
PubMed Publisher
In one paragraph

Article in Journal of neurology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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

0 citing papers in PubMed.

No citing paper in PubMed yet.

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

9 authors.

Maria Damiano *Clinical Neurophysiology Department and Neurology Department, Pitié-Salpêtrière Hospital, DMU Neurosciences, AP-HP, Sorbonne Université, Paris, France.
Virginie Lambrecq *Clinical Neurophysiology Department and Neurology Department, Pitié-Salpêtrière Hospital, DMU Neurosciences, AP-HP, Sorbonne Université, Paris, France.
Vi-Huong Nguyen-MichelClinical Neurophysiology Department and Neurology Department, Pitié-Salpêtrière Hospital, DMU Neurosciences, AP-HP, Sorbonne Université, Paris, France.
Clémence MaroisNeuro-Intensive Care Department, Pitié-Salpêtrière Hospital, AP-HP, Sorbonne Université, Paris, France.
Benoit RuchetonMetabolic Biochemistry Department, Pitié-Salpêtrière Hospital, AP-HP, Paris, France.
Fanny MochelDepartment of Medical Genetics, Reference Centers for Adult Neurometabolic Diseases and Adult Leukodystrophies, Pitié-Salpêtrière Hospital, AP-HP, Paris, France.
Isabelle Gourfinkel-AnClinical Neurophysiology Department and Neurology Department, Pitié-Salpêtrière Hospital, DMU Neurosciences, AP-HP, Sorbonne Université, Paris, France.
Sophie DemeretNeuro-Intensive Care Department, Pitié-Salpêtrière Hospital, AP-HP, Sorbonne Université, Paris, France.
Vincent NavarroClinical Neurophysiology Department and Neurology Department, Pitié-Salpêtrière Hospital, DMU Neurosciences, AP-HP, Sorbonne Université, Paris, France. vincent.navarro@aphp.fr.ORCID http://orcid.org/0000-0003-0077-8114

Funding

Agence Nationale de la Recherche ANR-10-IAIHU-06Assistance Publique - Hôpitaux de Paris DMU Neurosciences
6 · The paper itself

Abstract

backgroundThis study aimed to identify specific EEG features of status epilepticus (SE) in patients with mitochondrial diseases and correlate them with clinical and neuroimaging findings.

methodsClinical, EEG, and brain MRI data from adult patients with mitochondrial-related SE treated at Pitié-Salpêtrière hospital between 2010 and 2021 were reviewed.

resultsThirteen patients were included, with MELAS (n = 5), POLG (n = 3), MERRF (n = 2), COQ8A (n = 2), and MT-ND1 variant (n = 1). One patient had two distinct SE episodes, totaling 14 episodes. Three main EEG patterns were identified: Type 1 (rhythmic sequences with definite evolution, n = 6), Type 2 (Lateralized Periodic Discharges with plus-modifiers (LPDs-plus) and clinical ictal manifestations, n = 12), and Type 3 (LPD-plus evolving into rhythmic activities, n = 11). MRI findings revealed FLAIR hyperintensities indicative of stroke-like lesions in 12 patients (onset: 4-28 days). In three cases, initial MRI was normal, with delayed hyperintensities (5-35 days). In 11/12 cases, stroke-like lesions were confirmed using perfusion or ASL. The localization of these lesions corresponded to electroclinical presentation of SE in most cases. In two patients, the spatial distribution of FLAIR hyperintensities did not align with EEG findings. Evolution of EEG patterns was seen during SE in 12 out of 14 episodes: Type 1 to Type 2 (n = 3), Type 2 to Type 3 (n = 6), and Type 1 to Type 2, then Type 3 (n = 3).

conclusionSE is a severe complication of mitochondrial diseases. Three main EEG patterns were identified across episodes. Correlation with MRI suggests two mechanisms: (i) neuronal dysfunction generate rhythmic SE patterns that subsequently induce stroke-like lesions, due to increased local energy demands, and (ii) in more severe cases, acute bioenergetic failure and stroke-like lesions may directly trigger a specific EEG pattern, prolonging SE duration.

Indexed as

Mitochondrial DiseasesStatus EpilepticusAdultAgedBrainElectroencephalographyFemaleHumansMagnetic Resonance ImagingMaleMiddle AgedRetrospective StudiesYoung AdultClinical outcomeEEG patternsMitochondrial encephalopathyStatus epilepticus

Identifiers

PMID41729327

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

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.