Raras
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MELAS
ORPHA:550CID-10 · G71.3CID-11 · 8C73.YOMIM 540000DOENÇA RARA

A síndrome MELAS (miopatia mitocondrial, encefalopatia, acidose láctica e acidente vascular cerebral) é uma doença multissistêmica progressiva rara caracterizada por encefalomiopatia, acidose láctica e episódios semelhantes a acidente vascular cerebral. Outras características incluem endocrinopatia, doenças cardíacas, diabetes, perda auditiva e manifestações neurológicas e psiquiátricas.

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Introdução

O que você precisa saber de cara

📋

A síndrome MELAS (miopatia mitocondrial, encefalopatia, acidose láctica e acidente vascular cerebral) é uma doença multissistêmica progressiva rara caracterizada por encefalomiopatia, acidose láctica e episódios semelhantes a acidente vascular cerebral. Outras características incluem endocrinopatia, doenças cardíacas, diabetes, perda auditiva e manifestações neurológicas e psiquiátricas.

Pesquisas ativas
10 ensaios
41 total registrados no ClinicalTrials.gov
Publicações científicas
2.630 artigos
Último publicado: 2026 Apr 17

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.18
Japan
Início
Adolescent
+ adult, childhood
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: G71.3
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (2)
0202010694
Sequenciamento completo do exoma (WES)genetic_test
0301070040
Atendimento em reabilitação — doenças rarasrehabilitation
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Entender a doença

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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🧠
Neurológico
26 sintomas
❤️
Coração
9 sintomas
📏
Crescimento
8 sintomas
🫃
Digestivo
7 sintomas
💪
Músculos
6 sintomas
👂
Ouvidos
4 sintomas

+ 34 sintomas em outras categorias

Características mais comuns

90%prev.
Fraqueza muscular
Muito frequente (99-80%)
90%prev.
Afasia
Muito frequente (99-80%)
90%prev.
Enxaqueca
Muito frequente (99-80%)
90%prev.
Acidose láctica
Muito frequente (99-80%)
90%prev.
Espaço subaracnóideo cerebral alargado
Muito frequente (99-80%)
90%prev.
Aumento da concentração circulante de lactato
Muito frequente (99-80%)
103sintomas
Muito frequente (13)
Frequente (26)
Ocasional (45)
Muito raro (3)
Sem dados (16)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 103 características clínicas mais associadas, ordenadas por frequência.

Fraqueza muscularMuscle weakness
Muito frequente (99-80%)90%
AfasiaAphasia
Muito frequente (99-80%)90%
EnxaquecaMigraine
Muito frequente (99-80%)90%
Acidose lácticaLactic acidosis
Muito frequente (99-80%)90%
Espaço subaracnóideo cerebral alargadoWidened cerebral subarachnoid space
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico2.630PubMed
Últimos 10 anos200publicações
Pico2025115 papers
Linha do tempo
2026Hoje · 2026🧪 1978Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

19 genes identificados com associação a esta condição. Padrão de herança: Mitochondrial inheritance, Not applicable.

MT-TS2Candidate gene tested inDesconhecido
LOCALIZAÇÃO

VIAS BIOLÓGICAS (3)
G alpha (i) signalling eventsFormyl peptide receptors bind formyl peptides and many other ligandsG alpha (q) signalling events
OUTRAS DOENÇAS (4)
mitochondrial diseaseMERRF syndromeUsher syndrome type 3MELAS syndrome
HGNC:7498
MT-TL2MENDELIANDesconhecido
LOCALIZAÇÃO

VIAS BIOLÓGICAS (3)
G alpha (i) signalling eventsFormyl peptide receptors bind formyl peptides and many other ligandsG alpha (q) signalling events
OUTRAS DOENÇAS (3)
mitochondrial diseasematernally-inherited progressive external ophthalmoplegiaalternating hemiplegia of childhood
HGNC:7491
MT-TVMENDELIANDesconhecido
LOCALIZAÇÃO

VIAS BIOLÓGICAS (3)
G alpha (i) signalling eventsFormyl peptide receptors bind formyl peptides and many other ligandsG alpha (q) signalling events
OUTRAS DOENÇAS (2)
mitochondrial diseasematernally-inherited Leigh syndrome
HGNC:7500
MT-CYBCytochrome bMENDELIANDesconhecido
FUNÇÃO

Component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex) that is part of the mitochondrial respiratory chain. The b-c1 complex mediates electron transfer from ubiquinol to cytochrome c. Contributes to the generation of a proton gradient across the mitochondrial membrane that is then used for ATP synthesis

LOCALIZAÇÃO

Mitochondrion inner membrane

VIAS BIOLÓGICAS (3)
Respiratory electron transportComplex III assemblyMitochondrial translation termination
OUTRAS DOENÇAS (4)
mitochondrial diseaseLeber hereditary optic neuropathyhistiocytoid cardiomyopathymitochondrial complex III deficiency
HGNC:7427UniProt:P00156
MT-TKMENDELIANDesconhecido
LOCALIZAÇÃO

VIAS BIOLÓGICAS (3)
G alpha (i) signalling eventsFormyl peptide receptors bind formyl peptides and many other ligandsG alpha (q) signalling events
OUTRAS DOENÇAS (4)
mitochondrial diseaseMERRF syndromematernally-inherited cardiomyopathy and hearing lossmaternally-inherited Leigh syndrome
HGNC:7489
MT-ND1NADH-ubiquinone oxidoreductase chain 1Disease-causing germline mutation(s) inDesconhecido
FUNÇÃO

Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor (PubMed:1959619). Essential for the catalytic activity and assembly of complex I (PubMed:1959619, PubMed:26929434)

LOCALIZAÇÃO

Mitochondrion inner membrane

VIAS BIOLÓGICAS (4)
Respiratory electron transportComplex I biogenesisMitochondrial translation terminationMitochondrial protein degradation
MECANISMO DE DOENÇA

Leber hereditary optic neuropathy

A maternally inherited form of Leber hereditary optic neuropathy, a mitochondrial disease resulting in bilateral painless loss of central vision due to selective degeneration of the retinal ganglion cells and their axons. The disorder shows incomplete penetrance and male predominance. Cardiac conduction defects and neurological defects have also been described in some LHON patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.

OUTRAS DOENÇAS (5)
mitochondrial diseaseMELAS syndromemitochondrial complex I deficiencymaternally-inherited Leigh syndrome
HGNC:7455UniProt:P03886
MT-ND6NADH-ubiquinone oxidoreductase chain 6Disease-causing germline mutation(s) inDesconhecido
FUNÇÃO

Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor (PubMed:14595656, PubMed:8644732). Essential for the catalytic activity and assembly of complex I (PubMed:14595656, PubMed:8644732)

LOCALIZAÇÃO

Mitochondrion inner membrane

VIAS BIOLÓGICAS (4)
Respiratory electron transportComplex I biogenesisMitochondrial translation terminationMitochondrial protein degradation
MECANISMO DE DOENÇA

Leber hereditary optic neuropathy

A maternally inherited form of Leber hereditary optic neuropathy, a mitochondrial disease resulting in bilateral painless loss of central vision due to selective degeneration of the retinal ganglion cells and their axons. The disorder shows incomplete penetrance and male predominance. Cardiac conduction defects and neurological defects have also been described in some LHON patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.

OUTRAS DOENÇAS (5)
mitochondrial diseaseLeber plus diseasematernally-inherited Leigh syndromeMELAS syndrome
HGNC:7462UniProt:P03923
MT-TS1Disease-causing germline mutation(s) inDesconhecido
LOCALIZAÇÃO

VIAS BIOLÓGICAS (3)
G alpha (i) signalling eventsFormyl peptide receptors bind formyl peptides and many other ligandsG alpha (q) signalling events
OUTRAS DOENÇAS (6)
mitochondrial diseaseMERRF syndromepalmoplantar keratoderma-deafness syndromematernally-inherited progressive external ophthalmoplegia
HGNC:7497
MT-CO2Cytochrome c oxidase subunit 2Disease-causing germline mutation(s) inDesconhecido
FUNÇÃO

Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over t

LOCALIZAÇÃO

Mitochondrion inner membrane

VIAS BIOLÓGICAS (6)
Cytoprotection by HMOX1Respiratory electron transportTP53 Regulates Metabolic GenesComplex IV assemblyMitochondrial translation termination
MECANISMO DE DOENÇA

Mitochondrial complex IV deficiency

A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, exercise intolerance, developmental delay, delayed motor development and intellectual disability. Some affected individuals manifest a fatal hypertrophic cardiomyopathy resulting in neonatal death. A subset of patients manifest Leigh syndrome.

OUTRAS DOENÇAS (3)
mitochondrial diseaseMELAS syndromemitochondrial complex IV deficiency, nuclear-type
HGNC:7421UniProt:P00403
MT-TFDisease-causing germline mutation(s) inDesconhecido
LOCALIZAÇÃO

VIAS BIOLÓGICAS (2)
Post-translational protein phosphorylationRegulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs)
OUTRAS DOENÇAS (4)
mitochondrial diseaseMELAS syndromeGitelman-like kidney tubulopathy due to mitochondrial DNA mutationMERRF syndrome
HGNC:7481
MT-CO1Cytochrome c oxidase subunit 1Disease-causing germline mutation(s) inDesconhecido
FUNÇÃO

Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over t

LOCALIZAÇÃO

Mitochondrion inner membrane

VIAS BIOLÓGICAS (6)
Cytoprotection by HMOX1Respiratory electron transportTP53 Regulates Metabolic GenesComplex IV assemblyMitochondrial translation termination
MECANISMO DE DOENÇA

Leber hereditary optic neuropathy

A maternally inherited form of Leber hereditary optic neuropathy, a mitochondrial disease resulting in bilateral painless loss of central vision due to selective degeneration of the retinal ganglion cells and their axons. The disorder shows incomplete penetrance and male predominance. Cardiac conduction defects and neurological defects have also been described in some LHON patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.

OUTRAS DOENÇAS (6)
mitochondrial diseaseMELAS syndromemitochondrial complex IV deficiency, nuclear-typemitochondrial non-syndromic sensorineural hearing loss
HGNC:7419UniProt:P00395
MT-THCandidate gene tested inDesconhecido
LOCALIZAÇÃO

VIAS BIOLÓGICAS (1)
Catecholamine biosynthesis
OUTRAS DOENÇAS (4)
mitochondrial diseaseMERRF syndromeMELAS syndromemitochondrial non-syndromic sensorineural hearing loss
HGNC:7487
MT-ND4NADH-ubiquinone oxidoreductase chain 4Disease-causing germline mutation(s) inDesconhecido
FUNÇÃO

Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor (PubMed:15250827, PubMed:8344246, PubMed:8644732). Essential for the catalytic activity and assembly of complex I (PubMed:15250827, PubMed:8344246, PubMed:8644732)

LOCALIZAÇÃO

Mitochondrion inner membrane

VIAS BIOLÓGICAS (3)
Respiratory electron transportComplex I biogenesisMitochondrial translation termination
MECANISMO DE DOENÇA

Leber hereditary optic neuropathy

A maternally inherited form of Leber hereditary optic neuropathy, a mitochondrial disease resulting in bilateral painless loss of central vision due to selective degeneration of the retinal ganglion cells and their axons. The disorder shows incomplete penetrance and male predominance. Cardiac conduction defects and neurological defects have also been described in some LHON patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.

OUTRAS DOENÇAS (6)
mitochondrial diseasematernally-inherited Leigh syndromeMELAS syndromeLeber plus disease
HGNC:7459UniProt:P03905
MT-TL1Disease-causing germline mutation(s) inDesconhecido
LOCALIZAÇÃO

VIAS BIOLÓGICAS (3)
G alpha (i) signalling eventsFormyl peptide receptors bind formyl peptides and many other ligandsG alpha (q) signalling events
OUTRAS DOENÇAS (7)
mitochondrial diseasematernally-inherited Leigh syndromeMELAS syndromeMERRF syndrome
HGNC:7490
MT-ND5NADH-ubiquinone oxidoreductase chain 5Disease-causing germline mutation(s) inDesconhecido
FUNÇÃO

Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor (PubMed:15250827). Essential for the catalytic activity and assembly of complex I (PubMed:15250827)

LOCALIZAÇÃO

Mitochondrion inner membrane

VIAS BIOLÓGICAS (4)
Respiratory electron transportComplex I biogenesisMitochondrial translation terminationMitochondrial protein degradation
MECANISMO DE DOENÇA

Leber hereditary optic neuropathy

A maternally inherited form of Leber hereditary optic neuropathy, a mitochondrial disease resulting in bilateral painless loss of central vision due to selective degeneration of the retinal ganglion cells and their axons. The disorder shows incomplete penetrance and male predominance. Cardiac conduction defects and neurological defects have also been described in some LHON patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.

OUTRAS DOENÇAS (5)
mitochondrial diseaseMELAS syndromematernally-inherited Leigh syndromeMERRF syndrome
HGNC:7461UniProt:P03915
MT-CO3Cytochrome c oxidase subunit 3Candidate gene tested inDesconhecido
FUNÇÃO

Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over t

LOCALIZAÇÃO

Mitochondrion inner membrane

VIAS BIOLÓGICAS (5)
Cytoprotection by HMOX1Respiratory electron transportTP53 Regulates Metabolic GenesComplex IV assemblyMitochondrial translation termination
MECANISMO DE DOENÇA

Leber hereditary optic neuropathy

A maternally inherited form of Leber hereditary optic neuropathy, a mitochondrial disease resulting in bilateral painless loss of central vision due to selective degeneration of the retinal ganglion cells and their axons. The disorder shows incomplete penetrance and male predominance. Cardiac conduction defects and neurological defects have also been described in some LHON patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.

OUTRAS DOENÇAS (5)
mitochondrial diseasemitochondrial complex IV deficiency, nuclear-typeMELAS syndromehereditary recurrent myoglobinuria
HGNC:7422UniProt:P00414
MT-TQCandidate gene tested inDesconhecido
LOCALIZAÇÃO

VIAS BIOLÓGICAS (3)
G alpha (i) signalling eventsFormyl peptide receptors bind formyl peptides and many other ligandsG alpha (q) signalling events
OUTRAS DOENÇAS (2)
MELAS syndromeMERRF syndrome
HGNC:7495
MT-TWDisease-causing germline mutation(s) inDesconhecido
LOCALIZAÇÃO

VIAS BIOLÓGICAS (3)
G alpha (i) signalling eventsFormyl peptide receptors bind formyl peptides and many other ligandsG alpha (q) signalling events
OUTRAS DOENÇAS (3)
mitochondrial diseasematernally-inherited Leigh syndromeMELAS syndrome
HGNC:7501

Variantes genéticas (ClinVar)

91 variantes patogênicas registradas no ClinVar.

🧬 MT-TS2: NC_012920.1:m.8478_13589del ()
🧬 MT-TS2: NC_012920.1:m.8944_15057del ()
🧬 MT-TS2: Single allele ()
🧬 MT-TS2: NC_012920.1(MT-CYB):m.10950_15540del ()
🧬 MT-TS2: NC_012920.1(MT-TS2):m.12264C>T ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 485 variantes classificadas pelo ClinVar.

121
303
61
Patogênica (24.9%)
VUS (62.5%)
Benigna (12.6%)
VARIANTES MAIS SIGNIFICATIVAS
MT-ND5: NC_012920.1(MT-RNR1):m.758T>C [Pathogenic]
MT-ND3: NC_012920.1(MT-ND3):m.10176G>A [Conflicting classifications of pathogenicity]
MT-CYB: NC_012920.1(MT-CYB):m.15215G>A [Likely pathogenic]
MT-ND5: NC_012920.1(MT-ND5):m.12923G>A [Likely pathogenic]
MT-TL1: NC_012920.1(MT-TL1):m.3276A>T [Uncertain significance]

Vias biológicas (Reactome)

68 vias biológicas associadas aos genes desta condição.

Regulation of CDH11 Expression and Function Regulation of CDH11 gene transcription Intracellular oxygen transport Mitochondrial unfolded protein response (UPRmt) NADE modulates death signalling Activation of BIM and translocation to mitochondria Activation of caspases through apoptosome-mediated cleavage Ubiquinol biosynthesis Export of Viral Ribonucleoproteins from Nucleus NEP/NS2 Interacts with the Cellular Export Machinery Dual incision in TC-NER Formation of TC-NER Pre-Incision Complex Transcription-Coupled Nucleotide Excision Repair (TC-NER) Gap-filling DNA repair synthesis and ligation in TC-NER AXIN missense mutants destabilize the destruction complex Defective MUTYH substrate processing Defective MUTYH substrate binding Citric acid cycle (TCA cycle) Transcriptional regulation by RUNX3 Binding of TCF/LEF:CTNNB1 to target gene promoters Aryl hydrocarbon receptor signalling Ribosomal scanning and start codon recognition Translation initiation complex formation Activation of the mRNA upon binding of the cap-binding complex and eIFs, and subsequent binding to 43S GTP hydrolysis and joining of the 60S ribosomal subunit Regulation of NPAS4 gene expression Mechanical load activates signaling by PIEZO1 and integrins in osteocytes Mitochondrial translation termination Respiratory electron transport Complex III assembly TP53 Regulates Transcription of Caspase Activators and Caspases TP53 Regulates Transcription of Cell Death Genes Defective homologous recombination repair (HRR) due to PALB2 loss of function Defective HDR through Homologous Recombination Repair (HRR) due to PALB2 loss of BRCA1 binding function NPAS4 regulates expression of target genes Recruitment and ATM-mediated phosphorylation of repair and signaling proteins at DNA double strand breaks Complex I biogenesis Mitochondrial protein degradation TP53 Regulates Metabolic Genes Cytoprotection by HMOX1 Complex IV assembly Transcriptional regulation by RUNX2 NOTCH2 Activation and Transmission of Signal to the Nucleus Signaling by NOTCH2 Response of endothelial cells to shear stress Developmental Cell Lineages SARS-CoV-2 Infection Downregulation of ERBB2 signaling Regulation of CDH1 Expression and Function Regulation of RUNX2 expression and activity Defective Base Excision Repair Associated with NEIL1 Signaling by MET Virion Assembly and Release Virion Assembly and Release IKK complex recruitment mediated by RIP1 Transcriptional regulation by RUNX1 WNT mediated activation of DVL ABC transporter disorders Defective ABCA3 causes SMDP3 Strand-asynchronous mitochondrial DNA replication Signaling by NOTCH4 Regulation of CDH1 Gene Transcription Negative Regulation of CDH1 Gene Transcription NOTCH4 Activation and Transmission of Signal to the Nucleus Signaling by WNT rRNA modification in the nucleus and cytosol PTEN Regulation Regulation of PTEN gene transcription

Diagnóstico

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
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1Fase 11
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Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 20 ensaios
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Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — MELAS

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Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

7 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

41 ensaios clínicos encontrados, 10 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

📖Melhor nível de evidência: Revisão
Timeline de publicações
1.120 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 1.120

#1

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

Journal of neurology2026 Feb 23

This study aimed to identify specific EEG features of status epilepticus (SE) in patients with mitochondrial diseases and correlate them with clinical and neuroimaging findings. Clinical, EEG, and brain MRI data from adult patients with mitochondrial-related SE treated at Pitié-Salpêtrière hospital between 2010 and 2021 were reviewed. Thirteen 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). SE 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.

#2

A novel tRNASer(AGY) 12244G > a variant impairs mitochondrial function and presents with classical MELAS phenotype.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology2026 Feb 16

Mitochondrial disorders are a group of heterogeneous diseases marked by deficiencies in oxidative phosphorylation (OXPHOS). A common subtype, MELAS (mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes [SLEs]), is primarily linked to variants in mitochondrial transfer RNA (mt-tRNA) genes, yet the molecular mechanisms underlying many of these variants remain poorly understood. We performed a comprehensive assessment of a 14-year-old male patient, including clinical evaluation, genetic testing, histopathology, and functional biochemical analyses of muscle tissue. A systematic literature review was conducted to compare previously reported MT-TS2 variants and their associated phenotypes. We identified a rare m.12244G > A variant in the tRNASer(AGY) gene associated with classical MELAS phenotype. Functional analysis demonstrated impaired mitochondrial translation and OXPHOS dysfunction. Histological findings revealed COX-negative and ragged red fibers, while western blotting indicated downregulation of key mitochondrial proteins. Literature review showed that MT-TS2 variants are associated with variable phenotypes including encephalopathy, myopathy, deafness, diabetes, and retinopathy. Our study provides the first experimental validation of the pathogenicity of the m.12244G > A variant, confirming its deleterious impact on mitochondrial function. This finding expands the genotype spectrum of MELAS and highlights the importance of functional validation for rare mtDNA variants.

#3

Neuronal intranuclear inclusion disease: a diagnostic pitfall for MELAS.

BMC neurology2026 Feb 13

Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disease characterized by eosinophilic hyaluronan inclusions in the nervous system and internal organs. Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) is the most common neurological mitochondrial disease involving multiple organs. The complex and overlapping clinical manifestations of both diseases pose a significant risk for misdiagnosis. We present a case of NIID that closely mimicked the phenotype of MELAS. A 60-year-old Chinese man presented with recurrent headaches and cognitive impairment. Upon the first clinical presentation, brain magnetic resonance imaging (MRI) revealed multiple lacunar infarction foci. During the second presentation, the cranial MRI indicated lesions in the left occipital and parietal cortex. Cerebrospinal fluid (CSF) analysis ruled out common infectious, autoimmune, and paraneoplastic etiologies. Screening for Alzheimer’s disease (AD) biomarkers was also unremarkable. Muscle biopsy pathology revealed ragged-red fibers (RRFs), a finding consistent with MELAS. Based on the muscle biopsy findings, a provisional diagnosis of MELAS was made, and the patient received a course of intravenous arginine therapy. However, genetic testing for the NOTCH2NLC gene via capillary electrophoresis identified a heterozygous CGG repeat expansion (15 and 97 repeats), confirming the diagnosis of NIID. This case highlights the diagnostic challenge in distinguishing NIID from MELAS and underscores the necessity of genetic testing for NOTCH2NLC in patients with compatible phenotypes, even in the presence of MELAS-suggestive features like RRFs.

#4

Loss of Myofilaments in Gastrointestinal Smooth Muscle: A Novel Pathological Finding in MELAS-Associated Chronic Intestinal Pseudo-Obstruction.

Neurogastroenterology and motility2026 Feb

Chronic intestinal pseudo-obstruction (CIPO) occurs in up to 40% of patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). While enteric nervous system abnormalities have been documented, 15%-38% of CIPO cases show normal histology, suggesting alternative pathogenic mechanisms. We aimed to investigate smooth muscle pathology in MELAS-associated CIPO. Comprehensive pathological examination including light and electron microscopy was performed on autopsy material from a 52-year-old male with MELAS (m.3243A > G mutation) and recurrent intestinal obstruction symptoms. Gastrointestinal tissue from the entire digestive tract was analyzed and compared with age-matched control tissue. Histological examination revealed widespread vacuolization and pallor of gastrointestinal smooth muscle throughout the digestive tract (esophagus, stomach, duodenum, and colon). Electron microscopy demonstrated abundant abnormal mitochondria in smooth muscle cells and, notably, marked loss of myofilaments in the colonic muscle. The ultrastructural preservation was limited by postmortem changes (autolysis, occurring approximately 4 h after death) and the re-embedding technique from formalin-fixed paraffin-embedded tissue; however, the striking difference was evident compared to age-matched controls. Similar abnormalities were observed in the Auerbach plexus. This represents the first report of myofilament loss in MELAS-associated CIPO, suggesting that mitochondrial dysfunction may directly impair smooth muscle contractile apparatus beyond previously described neuronal abnormalities. These findings provide novel insights into CIPO pathogenesis and may inform therapeutic strategies emphasizing early enteral nutrition interventions that bypass affected gastrointestinal segments.

#5

Super-Refractory Status Epilepticus (SRSE) in a Patient With Compound Heterozygous OPA1 Variants: Case Report and Literature Review.

Annals of clinical and translational neurology2026 Feb 11

Super-Refractory Status Epilepticus (SRSE) is a rare, life-threatening neurological emergency with unclear etiology in many cases. Mitochondrial dysfunction, often due to disease-causing genetic variants, is increasingly recognized as a cause, with each gene producing distinct pathophysiological mechanisms. We describe the detailed clinical, neurophysiological, neuroimaging, and molecular findings of a 19-year-old female with SRSE associated with compound heterozygous variants in OPA1, a key gene for mitochondrial inner membrane fusion and cristae maintenance. In addition, a literature review was performed, identifying 16 previously published cases reporting one or both of the variants observed in the present case. Despite a longstanding history of generalized hypotonia, celiac disease, optic atrophy, cerebellar ataxia, and progressive motor decline, the proband had no prior history of seizures. She developed super-refractory status epilepticus with occipital-predominant epileptiform activity and MRI showing transient diffusion restriction in the right parieto-occipital cortex and cerebellum. Genetic testing revealed a frameshift variant (p.Val903GlyfsTer3) and a missense variant (p.Ile382Met) in the GTPase domain, known to impair mitochondrial fusion. Unlike POLG or MELAS-associated seizures, typically driven by severe mtDNA depletion and respiratory chain failure, OPA1 dysfunction usually spares mtDNA copy number but disrupts mitochondrial dynamics. In severe biallelic loss-of-function, a "second-hit" stressor may trigger a diffuse energy crisis and catastrophic seizures. This case of mitochondrial SRSE in a patient with no known infectious, autoimmune, or structural cause emphasizes the possible role of genetic background and mitochondrial disorders in the development of the disease. This case highlights a rare mitochondrial subtype of RSE, emphasizing the need to consider energy metabolism defects in unexplained refractory status epilepticus.

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2026

Sleep performance in MELAS is related not only to the syndrome, but also to several other endogenous and exogenous determinants.

Sleep &amp; breathing = Schlaf &amp; Atmung
2026

Integrated molecular and clinical profiling of primary mitochondrial oxidative phosphorylation disorders in an Indian cohort: Insights from genetics, neuroimaging, and machine learning.

Mitochondrion
2026

The Loss and Dysfunction of Smooth Muscle Cells in MELAS Are Not the Only Cause for Gastrointestinal Dysmotility.

Neurogastroenterology and motility
2026

MELAS syndrome complicated by anti-GFAP autoantibody positivity: a case report and literature review.

BMC neurology
2026

Olfactory Bulb Volume Reflects Olfactory Dysfunction and Network Organization: Insights From the Population-Based Rhineland Study.

International forum of allergy &amp; rhinology
2026

Progressive Myopathy and Respiratory Failure in a 7-Year-Old Boy With m.3251A>G MT-TL1 Mutation.

Journal of clinical neuromuscular disease
2026

Review Article: Overview of Clinical Genetics of Diabetes Mellitus.

Genes
2026

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

Journal of neurology
2026

Migratory vasodilatation of cerebral arteries in MELAS episodes: a case report and literature review.

Frontiers in immunology
2026

4,5-dihydroxyhexanoic acid is a robust circulating and urine marker of mitochondrial disease and its severity.

bioRxiv : the preprint server for biology
2026

A novel tRNASer(AGY) 12244G > a variant impairs mitochondrial function and presents with classical MELAS phenotype.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2026

Neuronal intranuclear inclusion disease: a diagnostic pitfall for MELAS.

BMC neurology
2026

Dysregulated iron homeostasis Drives mitochondrial Injury and ferroptosis susceptibility in MELAS fibroblasts.

Mitochondrion
2026

MELAS Syndrome Presenting with Hypertrophic Cardiomyopathy and Advanced Heart Failure: A Multisystem Diagnostic Challenge.

Journal of clinical medicine
2026

Loss of Myofilaments in Gastrointestinal Smooth Muscle: A Novel Pathological Finding in MELAS-Associated Chronic Intestinal Pseudo-Obstruction.

Neurogastroenterology and motility
2026

Super-Refractory Status Epilepticus (SRSE) in a Patient With Compound Heterozygous OPA1 Variants: Case Report and Literature Review.

Annals of clinical and translational neurology
2026

Multidimensional MRI is only one piece of the puzzle in the diagnosis of stroke-like episodes in MELAS.

Quantitative imaging in medicine and surgery
2026

Dynamic functional connectivity changes in the triple networks in patients with mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes.

Quantitative imaging in medicine and surgery
2026

Case Report: Simultaneous pancreas-kidney transplantation in MELAS: first reported case with 5-year follow-up.

Frontiers in transplantation
2026

A case of recurrent cerebellitis leading to the diagnosis of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS).

Rinsho shinkeigaku = Clinical neurology
2026

Before diagnosing immunological cerebellitis in an m.3243A>G carrier, a cerebellar stroke-like lesion should be ruled out.

Rinsho shinkeigaku = Clinical neurology
2026

Suppression of interferon signaling via small-molecule modulation of TFAM.

eLife
2026

Fahr Syndrome, Hypoparathyroidism and Mitochondrial Encephalomyopathy With Lactic Acidosis and Stroke-Like Episodes (MELAS) Syndrome.

AACE endocrinology and diabetes
2026

Biomarking MELAS with neurofilament light chain and circulating cell free mitochondrial DNA.

Molecular genetics and metabolism
2026

A case of delayed acute intestinal pseudo-obstruction after MELAS crisis in mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes.

Frontiers in medicine
2026

Taurine intake ameliorates lactic acidosis and hyperferritinemia occurring after mRNA SARS-CoV-2 vaccination in a patient with β-thalassemia trait: a case report and review of literature.

Journal of medical case reports
2026

Aversive behavioural responses of killer whales to sounds of long-finned pilot whales.

Scientific reports
2026

Transcriptional activation by MNRR1 is effected by recruiting p300 and can be induced by minimal peptides.

Mitochondrion
2026

Mitochondrial Myopathy, Lactic Acidosis, and Stroke-Like Episodes Combined with Diabetes: A Case Report.

Endocrine, metabolic &amp; immune disorders drug targets
2026

Society for Cardiovascular Magnetic Resonance 2024 Cases of SCMR Case Series.

Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance
2026

HLA Heterozygosity Influences Colorectal Cancer Risk and Survival Outcome.

Gastroenterology
2025

Descriptions of three hundred new species of Hesperiidae (Lepidoptera: Papilionoidea).

Insecta mundi
2026

Assessing the impact of ship emissions on the atmospheric chemical composition in the Eastern Mediterranean and the Piraeus port (Greece).

Scientific reports
2026

The symbiotic Wolbachia in Anopheles and its role in reducing the transmission of Plasmodium: updates and prospects.

Archives of microbiology
2025

Low-Frequency Photoparoxysmal Responses in a Patient With MELAS.

Revista de neurologia
2026

Altered gut microbiome function in ADHD: More Prevotella, less vitamin B12 biosynthesis, and beneficial modulation by synbiotic treatment.

Brain, behavior, and immunity
2026

Approach to the Patient: Mitochondrial Diabetes: Contemporary Cases and a Precision Medicine Approach.

The Journal of clinical endocrinology and metabolism
2025

Altered connectome gradient and its association with gene expression profiles in MELAS patients with stroke-like episodes.

AJNR. American journal of neuroradiology
2025

Mitochondrial tRNA-Derived Diseases.

International journal of molecular sciences
2025

Environmental DNA as a Tool for the Preliminary Assessment of Vertebrate Biodiversity: A Case Study from Sicilian Freshwater Ecosystems.

Biology
2025

Maternal lineage diversity and health-related haplogroups in the Gilgiti and Kohistani populations of northern Pakistan.

BMC genomics
2026

Episodic encephalopathy in NOTCH2NLC-related neuronal intranuclear inclusion disease: Clinical spectrum and a proposed classification framework.

Journal of the neurological sciences
2025

Sequential development of three syndromes in a patient with m.3243A>G mutation: a case report.

Frontiers of medicine
2025

Profiles of paediatric patients experiencing stroke-like episodes associated with mitochondrial disease.

Frontiers in neurology
2026

MELAS should only be diagnosed if the Hirano or Japanese criteria are met.

Radiology case reports
2025

Deep breath out: molecular survey of selected pathogens in blow and skin biopsies from North Atlantic cetaceans.

BMC veterinary research
2026

Counseling and Prognostic Challenges in Survivorship and Mortality in Primary Mitochondrial Disease: Reshaping a Once Bleak Landscape.

Pediatric neurology
2025

Clinical insights into mitochondrial retinopathy: A case report on m.3243A>G mutation and macular dystrophy.

Saudi journal of ophthalmology : official journal of the Saudi Ophthalmological Society
2025

Mitochondrial DNA A3243G variant: Current perspectives and clinical implications.

Intractable &amp; rare diseases research
2025

Fatal Methaemoglobin Intoxication Following Ketamine Infusion in a Depressed MELAS Patient: A Possible Association?

Case reports in critical care
2025

Human Breast Milk miRNAs: Investigation of Association Between Breastfeeding Children and Maternal Obesity in Obesity Development in Offspring.

Genes
2025

The daily rhythms of temperature preference are conserved in nocturnal and blind fish.

Journal of thermal biology
2025

Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like Episodes: A Masquerader of Young-onset Stroke.

The Journal of the Association of Physicians of India
2025

Monitoring the complexity and dynamics of mitochondrial translation.

Molecular cell
2026

Atypical clinical manifestation of MT-TL1 mutation in 6 months old patient.

Acta neurologica Belgica
2025

Migraine in monogenic disorders: Shedding light on new therapeutic targets.

Cephalalgia : an international journal of headache
2025

Modeling Mitochondrial Disease Using Brain Organoids: A Focus on Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like Episodes.

Journal of visualized experiments : JoVE
2025

Signs, symptoms, and health-related quality of life in MELAS: measuring what's important from the patient and clinician perspectives.

Journal of patient-reported outcomes
2026

Clinical characteristics and hearing impairment in carriers of the m.3243 A > G variant.

Journal of human genetics
2025

Retinopathy associated with MELAS syndrome. A case report.

Archivos de la Sociedad Espanola de Oftalmologia
2025

Multiparametric MRI detection of cerebral metabolism: a tool for early differentiation between MELAS and ischemic cerebral infarction.

Quantitative imaging in medicine and surgery
2025

From stroke workup to mitochondrial disease: A case report of MELAS.

Radiology case reports
2026

Personalized Anesthesia Strategies for Cochlear Implantation: Insights on Local Anesthesia From a Single-institution Experience.

Otology &amp; neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology
2025

Dynamic reconfiguration and transition of whole-brain networks in patients with MELAS revealed by a hidden Markov model.

Frontiers in neurology
2025

Safe Use of Metformin in Mitochondrial Diabetes in Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-Like Episodes: Insights From a Patient With m.3243A>G Mutation.

AACE endocrinology and diabetes
2025

Outcomes misaligned in mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS): implications for trial design.

Brain communications
2025

Genome-wide association study meta-analysis uncovers novel genetic variants associated with olfactory dysfunction.

BMC genomic data
2025

Effectiveness and Safety of Insulin Glargine 300 U/ml in High-Risk Subgroups (Renal Impairment and Older Age ≥ 70 years) of Insulin-Naïve People with Type 2 Diabetes: A Post hoc Analysis of Real-World ATOS Study.

Diabetes therapy : research, treatment and education of diabetes and related disorders
2025

Case Report: Abnormal pupils caused by the mitochondrial MT-TL1 gene m.3243A>G mutation.

Frontiers in pediatrics
2025

Autosomal recessive Alport syndrome should be diagnosed in an m.3243A>G carrier only if a pathogenic COL4A3 variant has been detected.

Internal medicine (Tokyo, Japan)
2025

Retinal multimodal-imaging and functional tests in a mitochondrial disease with focal and segmental glomerulosclerosis.

International journal of ophthalmology
2025

Neuroimaging in Mitochondrial Encephalomyopathy with Lactic Acidosis and Stroke-Like Episodes (MELAS): Authors' Reply.

Indian journal of pediatrics
2025

Epigenetic Profiling of Cell-Free DNA in Cerebrospinal Fluid: A Novel Biomarker Approach for Metabolic Brain Diseases.

Life (Basel, Switzerland)
2025

Comments on: "Stroke-Like Episode, Aphasia, and Hearing Loss in MELAS".

Journal of clinical neurology (Seoul, Korea)
2025

Re: Comments on "Stroke-Like Episode, Aphasia, and Hearing Loss in MELAS".

Journal of clinical neurology (Seoul, Korea)
2025

Effect of Cilostazol in the Expression of Biomarkers and Neurological Outcome Following Experimentally Induced Cerebrovascular Accident-Experimental Protocol.

Neurology international
2025

Neuroimaging in Mitochondrial Encephalomyopathy with Lactic Acidosis with Stroke-Like Episodes (MELAS): Correspondence.

Indian journal of pediatrics
2025

Diagnosing Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-Like Episodes (MELAS) Syndrome in a Young Adult Female Patient With Seizures and Lactic Acidosis.

Cureus
2025

A review of the link between the lactate-GPR81 axis and mitochondrial angiopathy in MELAS based on imaging characteristics.

Journal of neurology
2025

Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-Like Episodes (MELAS) Patients with m.3243A > G have High Prevalence of Wolff-Parkinson-White Syndrome.

Pediatric cardiology
2025

Peptide-mimetics derived from leucyl-tRNA synthetase are potential agents for the therapy of mt-tRNA related diseases.

Frontiers in pharmacology
2025

Severe clinical manifestation of mitochondrial disease due to the m.3243A>T variant: a case report of early-onset, multi-organ involvement and premature death.

Journal of rare diseases (Berlin, Germany)
2025

Zagociguat prevented stressor-induced neuromuscular dysfunction, improved mitochondrial physiology, and increased exercise capacity in diverse mitochondrial respiratory chain disease zebrafish models.

Frontiers in pharmacology
2025

Mercury exposure of six Greenland societies from toothed whale harvest over three decades spanning 1993-2020.

Environmental research
2025

Health-Related Education for New Immigrants to Prevent Chronic Diseases: A Systematic Review.

Journal of immigrant and minority health
2025

The spectrum of ophthalmologic abnormalities in MELAS is broader than expected.

Archivos de la Sociedad Espanola de Oftalmologia
2024

Noninvasive Assessments of Mitochondrial Capacity in People with Mitochondrial Myopathies.

Muscles (Basel, Switzerland)
2025

Clinical features, disease burden and impact on quality of life in participants with mitochondrial encephalomyopathy.

Frontiers in neurology
2025

Cognitive impairment profile in patients with the m.3243A> G variant in mitochondrial DNA.

BMC neurology
2025

Phosphodiesterase type 5 inhibition as a therapeutic strategy in primary mitochondrial disease: Evidence from patient fibroblasts and clinical observations.

Molecular genetics and metabolism
2025

Hypoparathyroidism in a Child with MELAS Syndrome: A Case Report of Severe Lactic Acidosis and Symmetrical Bilateral Basal Ganglia Calcification.

International journal of endocrinology and metabolism
2025

Pseudohypoxia-Stabilized HIF2α Transcriptionally Inhibits MNRR1, a Druggable Target in MELAS.

Cells
2025

[A case of recurrent cerebellitis leading to the diagnosis of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS)].

Rinsho shinkeigaku = Clinical neurology
2025

Anesthetic Management With Remimazolam for Adolescent Mitochondrial Encephalomyopathy With Lactic Acidosis and Stroke-like Episodes (MELAS): A Case Report.

Anesthesia progress
2025

Mitochondrial DNA Pathogenic Variant Prevalence in Primary Mitochondrial Disease Patients With African (L) Mitochondrial Genome Haplogroups.

JIMD reports
2025

Mitochondrial Hearing Loss: Genetic Variants and Clinical Progression.

Cureus
2025

Progressive encephalopathy in m.3243A > G/MT-TL1 mutation carriers: a quantitative EEG analysis.

Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology
2026

Combined Alport Syndrome Type 3A and Mitochondrial Disease Presenting with a Thin Base Membrane and Overt Albuminuria.

Internal medicine (Tokyo, Japan)
2025

Genome-wide association meta-analysis of human olfactory identification discovers sex-specific and sex-differential genetic variants.

Nature communications
2025

Neuroimaging in Mitochondrial Encephalomyopathy with Lactic Acidosis with Stroke-Like Episodes (MELAS).

Indian journal of pediatrics
2025

Psychiatric manifestations in a patient with a pathogenic variant in the MT-TL1 gene associated with MELAS.

BMJ case reports
2025

A survey of indoor and outdoor Anopheles density, species composition and circumsporozoite rate of malaria vectors on the Bijagós Archipelago, Guinea-Bissau.

Malaria journal
2025

Modelling mitochondrial diseases in neurons In Vitro: A systematic review.

Journal of neuromuscular diseases
2025

The m.3243A>G variant affects not only islet, hair, or retinal ganglion cells, but all cells.

GMS ophthalmology cases
2025

The m.3290T > C variant might be a protective factor against the pathogenic m.3243 A > G variant: a case study.

Orphanet journal of rare diseases
2025

Acute Management of Neurological Events in Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-Like Episodes (MELAS) Syndrome: A Case Report.

Cureus
2025

Open-label pilot study using hydroxytyrosol as dietary supplements in patients with mitochondrial diseases.

Orphanet journal of rare diseases
2025

Ischemic Stroke After Cochlear Implantation in a Patient With an Undiagnosed Mitochondrial Disorder.

Cureus
2025

CircRNA hypomethylation in the human amygdala implicates FKBP5 in alcohol use disorder.

bioRxiv : the preprint server for biology
2025

Delineating the mechanisms of cerebellar degeneration in paediatric and adult primary mitochondrial disease.

Acta neuropathologica
2025

Energy-Dependent Changes in Ion Mobility Peak Width Reveal Stability Shifts in Ribonucleic Acid Stem Loops.

Journal of the American Society for Mass Spectrometry
2025

Exploring the Phenotypic Heterogeneity and Bioenergetic Profile of the m.13513G>A mtDNA Substitution: A Heteroplasmy Perspective.

International journal of molecular sciences
2025

In vitro modelling of the neuropathophysiological features of mitochondrial epilepsy.

Seizure
2025

Unraveling Ribonucleic Acid Unfolding: A Quantitative Comparison of Solution and Gas-Phase Unfolding.

Analytical chemistry
2025

Endocrine manifestations and long-term outcomes of patients with mitochondrial diseases.

Orphanet journal of rare diseases
2025

Genetic migraine disorders and the response to calcitonin gene-related peptide antagonist treatment.

Headache
2025

Diplostomum-Induced Sac Formation in Lenses of Ameiurus Bullheads: A Host Defence Response?

Journal of fish diseases
2025

Stroke-Like Episode, Aphasia, and Hearing Loss in MELAS.

Journal of clinical neurology (Seoul, Korea)
2025

Crossed Cerebellar Diaschisis in a Patient with MELAS Syndrome: A Case Report.

Cerebellum (London, England)
2025

The copper ionophore disulfiram improves mitochondrial function in various yeast and human cellular models of mitochondrial diseases.

Human molecular genetics
2025

Interactions Between Killer Whales (Orcinus orca) and Neonate Long-Finned Pilot Whales (Globicephala melas) off South Iceland.

Ecology and evolution
2025

The genome sequence of long-finned pilot whale, Globicephala melas (Traill, 1809).

Wellcome open research
2025

Optimization of mtDNA-targeted platinum TALENs for bi-directionally modifying heteroplasmy levels in patient-derived m.3243A>G-iPSCs.

Molecular therapy. Nucleic acids
2025

Trio Exome Sequencing in VACTERL Association.

Kidney international reports
2025

Comparative modeling approaches for predicting Olea and Quercus pollen seasons in Thessaloniki, Greece.

Scientific reports
2025

Efficacy and Safety of Photobiomodulation in MELAS: Protocol for a Series of N-of-1 Trials.

Journal of clinical medicine
2025

A rare case of MELAS syndrome caused by mitochondrial DNA m.3256C>T mutation in China.

World journal of emergency medicine
2025

Clinical characteristics and long-term prognosis of 150 children with MELAS syndrome in China.

Journal of neurology
2025

A rare de novo mutation, m.1630A>G, in the mitochondrial tRNAVal (MT-TV) gene in a child with epilepsy: case report and review of the literature.

Translational pediatrics
2025

Molecular evolution of gustatory receptors in the Anopheles gambiae complex.

BMC ecology and evolution
2025

Segmental and Focal Glomerulosclerosis Secondary to MELAS Syndrome and Long-Term Outcomes After Kidney Transplant: Case Report and Literature Review.

Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation
2025

PHEMI-Phenylbutyrate in Patients With Lactic Acidosis: A Pilot, Single Arm, Phase I/II, Open-Label Trial.

Clinical therapeutics
2025

Metabolic remodeling in hiPSC-derived myofibers carrying the m.3243A>G mutation.

Stem cell reports
2025

Concentrations of persistent organic pollutants in long-finned pilot whales (Globicephala melas) sampled in a mass stranding event (MSE) on the Irish coast.

Marine pollution bulletin
2025

Genetic liability for anxiety and treatment response to the monoamine stabilizer OSU6162 in alcohol dependence: a retrospective secondary analysis.

Pharmacological reports : PR
2025

SUCCOR 10 years: a decade's perspective on radical hysterectomy outcomes in cervical cancer.

International journal of gynecological cancer : official journal of the International Gynecological Cancer Society
2025

Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) with high-frequency oscillations on scalp EEG: A case report.

Epilepsy &amp; behavior reports
2025

Stress, Anxiety and Depressive Symptoms, Burnout and Insomnia Among Greek Nurses One Year After the End of the Pandemic: A Moderated Chain Mediation Model.

Journal of clinical medicine
2025

Comprehensive predictors of drug-resistant epilepsy in MELAS: clinical, EEG, imaging, and biochemical factors.

BMC neurology
2025

Cardiac manifestations in adult MELAS syndrome (mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome)- a cross-sectional study.

Orphanet journal of rare diseases
2025

Causes of phenotypic heterogeneity in monozygotic MELAS twins.

Clinical neurology and neurosurgery
2025

A Retrospective Study Using a Novel Body-Shift Implant Design with a Novel Alloplastic Particulate Grafting Material in Immediate Extraction Sockets.

European journal of dentistry
2025

Spatial and Temporal Distribution of White Matter Lesions in NOTCH2NLC-Related Neuronal Intranuclear Inclusion Disease.

Neurology
2025

Serum chitotriosidase-1 (CHIT1) as candidate biomarker for mitochondriopathies.

Journal of neurology
2025

Serological Diagnosis of Brucella Infection in Cetaceans by Rapid Serum Agglutination Test and Competitive ELISA with Brucella abortus and Brucella ceti as Antigens.

Pathogens (Basel, Switzerland)
2025

Lateralized periodic discharges and photic sensitivity in adult onset MELAS syndrome in twin sisters.

Clinical neurology and neurosurgery
2025

Incidental finding of MELAS in a young woman with decompensated heart failure and end stage kidney disease: a case report.

European heart journal. Case reports
2024

Radiologic Findings in a Patient With Mitochondrial Encephalomyopathy With Lactic Acidosis and Stroke-Like Episodes Syndrome: A Case Report.

Cureus
2024

Aliens Among Us: Sensitivity of the Invasive Alien Fish Black Bullhead Ameiurus melas as a Bioindicator of Pollution and Its Safety for Human Consumption.

Toxics
2024

Diagnosis and Management of Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like Episodes Syndrome.

Biomolecules
2025

Perfusion and Electrophysiological Changes in MELAS.

Annals of neurology
2024

Multisystem clinicopathologic and genetic analysis of MELAS.

Orphanet journal of rare diseases
2025

Mitochondrial tRNA modifications: functions, diseases caused by their loss, and treatment strategies.

RNA (New York, N.Y.)
2024

Long-term prognostic factors and outcomes in mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes: a clinical and biochemical marker analysis.

Frontiers in neurology
2024

"Strokes" in MELAS are Actually Stroke-like Episodes Due To Metabolic Pathophysiology.

Neurology India
2025

Molecular Detection of a Novel Poxvirus in Black Bullhead (Ameiurus melas): Emerging Pathogens in a Natural Freshwater in Hungary?

Journal of fish diseases
2024

New Insights on the Diversity, Ecology and Genetic Population Structure of Anisakis spp. from Fish and Cetacean Hosts from Northeast Atlantic Waters.

Animals : an open access journal from MDPI
2025

Inhibition of the PI3K-AKT-MTORC1 axis reduces the burden of the m.3243A>G mtDNA mutation by promoting mitophagy and improving mitochondrial function.

Autophagy
2025

Disease registries and rare disorders: The virtuous example of mitochondrial medicine.

Experimental neurology
2024

LNC-ing Genetics in Mitochondrial Disease.

Non-coding RNA
2024

Unveiling the Mitochondrial Mystery: A Case of Mitochondrial Encephalopathy With Lactic Acidosis and Stroke-Like Episodes.

Cureus
2024

To prevent sudden death in m.3243A>G carriers, comprehensive neurologic, cardiac, and pulmological examinations are required.

Cardiology in the young
2024

Fabry Disease: A Rare Mutation With Common Clinical Presentation.

Cureus
2024

Effective Management of Chronic Intestinal Pseudo-Obstruction in MELAS Using Acotiamide: A Case Report.

Case reports in neurology
2024

Neuronal Intranuclear Inclusion Disease Presenting with Acute-Onset Dementia and Cortical Edema: A Case Report.

Frontiers in neurology
2024

A first vocal repertoire characterization of long-finned pilot whales (Globicephala melas) in the Mediterranean Sea: a machine learning approach.

Royal Society open science
2025

Phase 2b program with sonlicromanol in patients with mitochondrial disease due to m.3243A>G mutation.

Brain : a journal of neurology
2024

A novel m.5906G > a variant in MT-CO1 causes MELAS/Leigh overlap syndrome.

Molecular genetics and genomics : MGG
2024

Evaluation of Two Particle Number (PN) Counters with Different Test Protocols for the Periodic Technical Inspection (PTI) of Gasoline Vehicles.

Sensors (Basel, Switzerland)
2025

MELAS Presenting as Bilateral Symmetric Occipital and Temporal Cortices Lesions: A Case Report and Literature Review.

The neurologist
2024

Targeted nanopore sequencing using the Flongle device to identify mitochondrial DNA variants.

Scientific reports
2025

Cardiac and Renal Transplantation in Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like Symptoms: Anesthetic Challenges and Considerations.

Journal of cardiothoracic and vascular anesthesia
2024

Post-transcriptional methylation of mitochondrial-tRNA differentially contributes to mitochondrial pathology.

Nature communications
2024

[Hyperferritinemia - investigation, diagnosis and treatment].

Lakartidningen
2024

Multifocal vitelliform lesions associated with mitochondrial retinopathy.

American journal of ophthalmology case reports
2024

Moyamoya syndrome secondary to MELAS syndrome in a child: A case report and literature revue.

Radiology case reports
2024

[Taurine for Mitochondrial Diseases].

Brain and nerve = Shinkei kenkyu no shinpo
2024

Stroke-like episodes in patients with adult-onset neuronal intranuclear inclusion disease and patients with late-onset MELAS: A comparative study.

Annals of clinical and translational neurology
2024

Successful Simultaneous Heart-Kidney Transplant in a Patient With MT-TL1 MELAS Cardiomyopathy.

JACC. Case reports
2024

A post-hoc pooled analysis to evaluate efficacy and safety of insulin glargine 300 U/mL in insulin-naïve people with type 2 diabetes with/without prior use of glucagon-like peptide-1 receptor agonist therapy.

Diabetes research and clinical practice
2024

Anterior Cruciate Ligament Reconstruction Utilizing Double Adjustable-Loop Suspensory Fixation Devices Provides Good Clinical Outcomes in Patients under the Age of 40 Years at Two-Year Follow-Up.

Journal of clinical medicine
2024

Neurological manifestations in adult patients with the m.3243A>G variant in mitochondrial DNA.

BMJ neurology open
2024

Molecular investigation of endoparasites of marine mammals (Cetacea: Mysticeti, Odontoceti) in the Western Mediterranean.

Frontiers in veterinary science
2024

Molecular characterization of gliomas and glioneuronal tumors amid Noonan syndrome: cancer predisposition examined.

Frontiers in oncology
2024

Whole genome sequence analysis of population structure and insecticide resistance markers in Anopheles melas from the Bijagós Archipelago, Guinea-Bissau.

Parasites &amp; vectors
2024

Novel Mitochondrial Cytopathy Causing Mitochondrial Encephalomyopathy With Lactic Acidosis and Stroke-Like Episodes Syndrome and Tubulointerstitial Nephropathy.

Cureus
2024

The researcher's guide to selecting biomarkers in mental health studies.

BioEssays : news and reviews in molecular, cellular and developmental biology
2024

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes with coexisting nemaline myopathy: a case report.

Journal of medical case reports
2024

Safety and Effectiveness of Concomitant iGlarLixi and SGLT-2i Use in People with T2D During Ramadan Fasting: A SoliRam Study Sub-analysis.

Diabetes therapy : research, treatment and education of diabetes and related disorders
2024

Regulated and unregulated emissions from Euro VI Diesel and CNG heavy-duty vehicles.

Transportation research. Part D, Transport and environment
2024

Mitochondrial Variation in Anopheles gambiae and Anopheles coluzzii: Phylogeographic Legacy and Mitonuclear Associations With Metabolic Resistance to Pathogens and Insecticides.

Genome biology and evolution
2024

Blood-derived microRNAs are related to cognitive domains in the general population.

Alzheimer's &amp; dementia : the journal of the Alzheimer's Association
2024

Frailty Assessment and Its Impact on Loneliness among Older Adults Receiving Home-Based Healthcare during the COVID-19 Pandemic.

Healthcare (Basel, Switzerland)
2024

Serological Investigation for Brucella ceti in Cetaceans from the Northwestern Mediterranean Sea.

Animals : an open access journal from MDPI
2024

Cortical atrophy is a common phenotypic feature in MELAS patients.

Asian journal of surgery
2024

Fahr's syndrome as the initial imaging characteristics of MELAS syndrome with a possible seizure activity and cardiac arrest: a case report.

Frontiers in genetics
Ver todos os 1.289 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

Ainda não temos associações cadastradas para MELAS.

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Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para MELAS

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. EEG, clinical, and MRI features of status epilepticus associated with mitochondrial diseases.
    Journal of neurology· 2026· PMID 41729327mais citado
  2. A novel tRNASer(AGY) 12244G&#x2009;&gt;&#x2009;a variant impairs mitochondrial function and presents with classical MELAS phenotype.
    Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology· 2026· PMID 41692888mais citado
  3. Neuronal intranuclear inclusion disease: a diagnostic pitfall for MELAS.
    BMC neurology· 2026· PMID 41688968mais citado
  4. Loss of Myofilaments in Gastrointestinal Smooth Muscle: A Novel Pathological Finding in MELAS-Associated Chronic Intestinal Pseudo-Obstruction.
    Neurogastroenterology and motility· 2026· PMID 41681036mais citado
  5. Super-Refractory Status Epilepticus (SRSE) in a Patient With Compound Heterozygous OPA1 Variants: Case Report and Literature Review.
    Annals of clinical and translational neurology· 2026· PMID 41670169mais citado
  6. COQ8A-related Primary Coenzyme Q10 Deficiency Mimicking Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like Syndrome: A Pediatric Case Report and Review of Mitochondrial Mimics.
    Ann Afr Med· 2026· PMID 41992454recente
  7. Whether Metformin Causes Lactic Acidosis in m.3243A>G Carriers Depends on Several Factors.
    AACE Endocrinol Diabetes· 2026· PMID 41938317recente
  8. From confusion to diagnosis: a rare case of melas syndrome in a patient with familial consanguinity, recurrent stroke-like episodes, and concurrent FSGS.
    BMC Neurol· 2026· PMID 41928125recente
  9. Response to: "The Loss and Dysfunction of Smooth Muscle Cells in MELAS Are Not the Only Cause for Gastrointestinal Dysmotility".
    Neurogastroenterol Motil· 2026· PMID 41923532recente
  10. MERRF/MELAS overlap syndrome mimicking paradoxical cerebral embolism due to patent foramen ovale.
    Neurol Sci· 2026· PMID 41917332recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:550(Orphanet)
  2. OMIM OMIM:540000(OMIM)
  3. MONDO:0010789(MONDO)
  4. GARD:7009(GARD (NIH))
  5. Variantes catalogadas(ClinVar)
  6. Busca completa no PubMed(PubMed)
  7. Q2666433(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

MELAS
Compêndio · Raras BR

MELAS

ORPHA:550 · MONDO:0010789
Prevalência
Unknown
Herança
Mitochondrial inheritance, Not applicable
CID-10
G71.3 · Miopatia mitocondrial não classificada em outra parte
CID-11
Ensaios
10 ativos
Início
Adolescent, Adult, Childhood
Prevalência
0.18 (Japan)
MedGen
UMLS
C0162671
EuropePMC
Wikidata
Papers 10a
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