Clinical features of epileptic seizures in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes.


Journal

Seizure
ISSN: 1532-2688
Titre abrégé: Seizure
Pays: England
ID NLM: 9306979

Informations de publication

Date de publication:
Mar 2023
Historique:
received: 24 10 2022
revised: 15 02 2023
accepted: 16 02 2023
medline: 4 4 2023
pubmed: 25 2 2023
entrez: 24 2 2023
Statut: ppublish

Résumé

This study aimed to characterize the clinical features of epilepsy in mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) and analyze the clinical determinants for drug-resistant epilepsy in MELAS. A single-center, retrospective study was conducted to investigate the clinical features of epilepsy in patients with MELAS. Collected variables included seizure semiology, electroencephalography (EEG), muscle biopsy, genetic testing, neuroimaging findings, resting serum lactic value and modified Rankin scale (mRS) of patients with MELAS. We also investigated the differences between the adult-onset group and the child-onset group and analyzed the risk factors for drug-resistant epilepsy in MELAS. We studied 97 patients (56 males: 41 females) with confirmed MELAS. Epileptic seizure occurred in 100.0% of patients and the initial symptom of 69.1% patients was epileptic seizure. The average age of disease onset was 21.0 years, ranging from 2 to 60 years. The seizure types of these patients with MELAS were variable, with generalized onset (51.5%) to be the most common type. The EEG changes in the patients with MELAS were mainly slow wave (90.9%) and epileptiform discharge (68.2%). The child-onset group with earlier seizure onset presented significantly higher resting serum lactic value (p = 0.0048) and lower incidence of stroke-like lesion in the brain (p = 0.003), especially in the temporal lobe (p < 0.001), compared with the adult-onset group. Importantly, drug-resistant epilepsy in MELAS was demonstrated to be closely related to the earlier age of seizure onset (p = 0.013), as well as the higher mRS score (p < 0.001) and higher resting serum lactic value (p = 0.009). Early identification of MELAS should be considered among individuals with recurrent epilepsy through clinical screening. Age of seizure onset and resting serum lactic value may predict the development of drug-resistant epilepsy in MELAS. Close observation and appropriate anti-epileptic treatment are indispensable for individuals with MELAS to improve the prognosis. Further studies with larger sample size are required to further evaluate the risk factors of drug-resistant epilepsy in MELAS and provide guidance on treatment of MELAS.

Sections du résumé

BACKGROUND AND PURPOSE OBJECTIVE
This study aimed to characterize the clinical features of epilepsy in mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) and analyze the clinical determinants for drug-resistant epilepsy in MELAS.
METHODS METHODS
A single-center, retrospective study was conducted to investigate the clinical features of epilepsy in patients with MELAS. Collected variables included seizure semiology, electroencephalography (EEG), muscle biopsy, genetic testing, neuroimaging findings, resting serum lactic value and modified Rankin scale (mRS) of patients with MELAS. We also investigated the differences between the adult-onset group and the child-onset group and analyzed the risk factors for drug-resistant epilepsy in MELAS.
RESULTS RESULTS
We studied 97 patients (56 males: 41 females) with confirmed MELAS. Epileptic seizure occurred in 100.0% of patients and the initial symptom of 69.1% patients was epileptic seizure. The average age of disease onset was 21.0 years, ranging from 2 to 60 years. The seizure types of these patients with MELAS were variable, with generalized onset (51.5%) to be the most common type. The EEG changes in the patients with MELAS were mainly slow wave (90.9%) and epileptiform discharge (68.2%). The child-onset group with earlier seizure onset presented significantly higher resting serum lactic value (p = 0.0048) and lower incidence of stroke-like lesion in the brain (p = 0.003), especially in the temporal lobe (p < 0.001), compared with the adult-onset group. Importantly, drug-resistant epilepsy in MELAS was demonstrated to be closely related to the earlier age of seizure onset (p = 0.013), as well as the higher mRS score (p < 0.001) and higher resting serum lactic value (p = 0.009).
CONCLUSION CONCLUSIONS
Early identification of MELAS should be considered among individuals with recurrent epilepsy through clinical screening. Age of seizure onset and resting serum lactic value may predict the development of drug-resistant epilepsy in MELAS. Close observation and appropriate anti-epileptic treatment are indispensable for individuals with MELAS to improve the prognosis. Further studies with larger sample size are required to further evaluate the risk factors of drug-resistant epilepsy in MELAS and provide guidance on treatment of MELAS.

Identifiants

pubmed: 36827862
pii: S1059-1311(23)00055-9
doi: 10.1016/j.seizure.2023.02.014
pii:
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

110-116

Informations de copyright

Copyright © 2023 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

Déclaration de conflit d'intérêts

Conflicts of interest The authors declared no conflicts of interest.

Auteurs

Xiaxin Yang (X)

Department of Neurology, Qilu Hospital of Shandong University, Jinan, Shandong 250012, PR. China.

Anqi Sun (A)

Department of Neurology, Qilu Hospital of Shandong University, Jinan, Shandong 250012, PR. China.

Kunqian Ji (K)

Research Institute of Neuromuscular and Neurodegenerative Diseases and Department of Neurology, Qilu Hospital of Shandong University, Jinan, Shandong, 250012, PR. China.

Xiaotang Wang (X)

Department of Neurology, Qilu Hospital of Shandong University, Jinan, Shandong 250012, PR. China.

Xue Yang (X)

Department of Neurology, Qilu Hospital of Shandong University, Jinan, Shandong 250012, PR. China. Electronic address: catecolamine@126.com.

Xiuhe Zhao (X)

Department of Neurology, Qilu Hospital of Shandong University, Jinan, Shandong 250012, PR. China. Electronic address: zhaoxiuhe@126.com.

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Classifications MeSH