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Epilepsia de ausências juvenil
ORPHA:1941CID-10 · G40.3CID-11 · 8A61.31PCDT · SUSDOENÇA RARA

Uma epilepsia genética que começa por volta da puberdade. A epilepsia de ausência juvenil é caracterizada pela ocorrência esporádica de crises de ausência, frequentemente associada a uma presença que dura a vida toda de crises tônico-clônicas generalizadas (CTCGs) e espasmos mioclônicos esporádicos.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

Uma epilepsia genética que começa por volta da puberdade. A epilepsia de ausência juvenil é caracterizada pela ocorrência esporádica de crises de ausência, frequentemente associada a uma presença que dura a vida toda de crises tônico-clônicas generalizadas (CTCGs) e espasmos mioclônicos esporádicos.

Pesquisas ativas
3 ensaios
17 total registrados no ClinicalTrials.gov
Publicações científicas
251 artigos
Último publicado: 2026 Mar 20
Medicamentos
1 registrados
BRIVARACETAM

Tem tratamento?

1 medicamento registrado
Ver detalhes, fases e interações →
BRIVARACETAM

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 100 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Europe
Início
Adolescent
🏥
SUS: Cobertura parcialScore: 45%
PCDT disponívelCID-10: G40.3
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (2)
0202010694
Sequenciamento completo do exoma (WES)genetic_test
0301070040
Atendimento em reabilitação — doenças rarasrehabilitation
Você se identifica com essa condição?
O Raras está aqui pra te apoiar — com ou sem diagnóstico

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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

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

Partes do corpo afetadas

🧠
Neurológico
8 sintomas
🦴
Ossos e articulações
1 sintomas
👁️
Olhos
1 sintomas
👂
Ouvidos
1 sintomas
💪
Músculos
1 sintomas

+ 1 sintomas em outras categorias

Características mais comuns

90%prev.
Crise tônico-clônica bilateral
Muito frequente (99-80%)
90%prev.
EEG com complexos de poliespícula e onda
Muito frequente (99-80%)
90%prev.
Crise de início generalizado
Muito frequente (99-80%)
55%prev.
Anormalidade do movimento ocular
Frequente (79-30%)
55%prev.
Morfologia anormal da boca
Frequente (79-30%)
55%prev.
Mioclonias
Frequente (79-30%)
13sintomas
Muito frequente (3)
Frequente (4)
Ocasional (6)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 13 características clínicas mais associadas, ordenadas por frequência.

Crise tônico-clônica bilateralBilateral tonic-clonic seizure
Muito frequente (99-80%)90%
EEG com complexos de poliespícula e ondaEEG with polyspike wave complexes
Muito frequente (99-80%)90%
Crise de início generalizadoGeneralized-onset seizure
Muito frequente (99-80%)90%
Anormalidade do movimento ocularAbnormality of eye movement
Frequente (79-30%)55%
Morfologia anormal da bocaAbnormality of the mouth
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico251PubMed
Últimos 10 anos116publicações
Pico202318 papers
Linha do tempo
2026Hoje · 2026🧪 1998Primeiro ensaio clínico📈 2023Ano 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

1 gene identificado com associação a esta condição. Padrão de herança: Multigenic/multifactorial, Unknown.

EFHC1EF-hand domain-containing protein 1Major susceptibility factor inTolerante
FUNÇÃO

Microtubule inner protein (MIP) part of the dynein-decorated doublet microtubules (DMTs) in cilia axoneme, which is required for motile cilia beating (PubMed:36191189). Microtubule-associated protein which regulates cell division and neuronal migration during cortical development (PubMed:19734894, PubMed:28370826). Necessary for radial and tangential cell migration during brain development, possibly acting as a regulator of cell morphology and process formation during migration (PubMed:22926142)

LOCALIZAÇÃO

Cytoplasm, cytoskeleton, cilium axonemeCytoplasm, cytoskeleton, flagellum axonemeCytoplasm, cytoskeleton, microtubule organizing center, centrosomeCytoplasm, cytoskeleton, spindleCytoplasm, cytoskeleton, spindle pole

MECANISMO DE DOENÇA

Juvenile myoclonic epilepsy 1

A subtype of idiopathic generalized epilepsy. Patients have afebrile seizures only, with onset in adolescence (rather than in childhood) and myoclonic jerks which usually occur after awakening and are triggered by sleep deprivation and fatigue.

EXPRESSÃO TECIDUAL(Ubíquo)
Pituitária
17.6 TPM
Tireoide
13.0 TPM
Testículo
12.7 TPM
Cervix Endocervix
11.6 TPM
Fallopian Tube
10.2 TPM
OUTRAS DOENÇAS (3)
juvenile absence epilepsyjuvenile myoclonic epilepsyepilepsy, juvenile absence, susceptibility to, 1
HGNC:16406UniProt:Q5JVL4

Medicamentos e terapias

BRIVARACETAMPhase 3

Mecanismo: Synaptic vesicle glycoprotein 2A modulator

Ver mais no OpenTargets

Variantes genéticas (ClinVar)

46 variantes patogênicas registradas no ClinVar.

🧬 EFHC1: NM_018100.4(EFHC1):c.573+145T>A ()
🧬 EFHC1: NM_018100.4(EFHC1):c.64-260G>T ()
🧬 EFHC1: NM_018100.4(EFHC1):c.64-261A>T ()
🧬 EFHC1: NM_018100.4(EFHC1):c.917-1G>C ()
🧬 EFHC1: GRCh37/hg19 6p12.3-12.1(chr6:50181657-55538355)x1 ()
Ver todas no ClinVar

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
3Fase 36
2Fase 24
·Pré-clínico6
Medicamentos catalogadosEnsaios clínicos· 1 medicamento · 15 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Epilepsia de ausências juvenil

🗺️

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

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

Outros ensaios clínicos

17 ensaios clínicos encontrados, 3 ativos.

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

Timeline de publicações
120 papers (10 anos)
#1

Investigation of adolescent cases diagnosed with idiopathic/genetic generalized epilepsy in terms of self-esteem, aggression, body perception, and alexithymia.

European journal of pediatrics2026 Mar 19

This study examined self-esteem, body image, alexithymia, and aggression in adolescents with idiopathic/genetic generalized epilepsy (IGE). These domains were selected because they may be affected not only by epilepsy itself but also by stigma, difficulties in emotional regulation, or treatment-related effects. Assessing these domains together was intended to examine whether IGE might be associated with additional psychosocial strain in otherwise clinically stable adolescents. We carried out a case-control study including 45 adolescents with IGE and 45 controls matched for age and sex. Controls were selected from outpatient services and were required to have no neurological disorders. Psychiatric disorders were assessed using the K-SADS (Kiddie Schedule for Affective Disorders and Schizophrenia) interview; no clinically significant psychiatric diagnoses were identified in the control group. All participants completed standardized Turkish questionnaires on the same day.Within the epilepsy group, 20.0% were diagnosed with juvenile absence epilepsy (JAE), 22.2% with juvenile myoclonic epilepsy (JME), and 57.8% with epilepsy with generalized tonic-clonic seizures alone (EGTCSA). Across all four psychological measures, the epilepsy and control groups showed no statistically significant differences (all p > 0.05). No significant differences were found in subgroup analyses based on epilepsy syndrome or seizure frequency. Adolescents with well-controlled IGE and minimal psychiatric comorbidities demonstrated comparable levels of psychosocial functioning to their healthy peers. This finding is consistent with evidence suggesting that psychosocial difficulties in epilepsy are often linked to comorbidity and contextual factors rather than to the diagnosis alone. Studies conducted in broader settings and with larger, more varied samples will be needed to better understand how medical, emotional, and environmental factors jointly influence psychosocial outcomes in this population. • Adolescents with epilepsy are frequently considered at increased risk for psychosocial difficulties, including lower self-esteem, emotional instability, and body image concerns. • In this study, no significant differences were found in self-esteem, body image, alexithymia, or aggression among Turkish adolescents with idiopathic/genetic generalized epilepsy compared with healthy controls. These findings suggest that, when seizures are well controlled and psychiatric comorbidities are limited, epilepsy itself may not confer additional psychosocial vulnerability.

#2

Causal effect of Helicobacter pylori infection on brain health and potential mediation by immune cells.

European archives of psychiatry and clinical neuroscience2026 Feb

Increasing evidence suggests an association between Helicobacter pylori(HP) infection and brain health and its clinical manifestations. A growing body of evidence underscored the profound influence of immune cells on brain health. However, the existing evidence is equivocal, and the causal link remains uncertain. This study aims to determine if there is a causal connection between Hp infections and 42 brain-related diseases, as well as 1,325 distinct brain structures, and to explore the potential role of immune cells in mediating these associations. Both univariable and multivariable mendelian randomization (UVMR and MVMR) was applied to evaluate the causal relationships between Hp protein antibodies and 1325 brain structure, as well as various brain-related diseases. Finally, we assessed the relationship between immune cells and HP protein antibodies and brain health, and then calculated the mediating effect of immune cells on the significant causal associations. Hp antibodies exhibited disease-specific effects: CagA conferred protection against anorexia nervosa(OR = 0.94, p = 0.015) and schizophrenia(OR = 0.91, p = 0.038).Conversely, catalase antibodies increased risks for frontotemporal dementia(OR = 1.47, p = 0.002) and juvenile absence epilepsy(OR = 1.01, p = 0.007). MVMR confirmed persistent associations with 61 brain structural alterations, such as IgG's impact on anterior cingulate morphology(OR = 1.06, p = 6.09E-05).Immune cells mediated 8.6-47.4% of Hp-brain effects: CD27⁺IgD⁺CD24⁺ B cells linked IgG to depression(18.2% mediation), while HLA-DR⁺ dendritic cells mediated UreA-associated white matter changes (10.9%). Hp infection may exert dual neuroprotective and pathogenic effects via antibody-specific mechanisms, modulated by immune cell phenotypes.This study underscores the gut-brain-immune axis as a pivotal frontier in neuropsychiatric research, with implications for precision interventions targeting Hp-related immune dysregulation.

#3

Effects of lipid-lowering drugs on epilepsy and its subtypes: A drug-target Mendelian randomization study.

Medicine2026 Mar 20

Statins, commonly used lipid-lowering drugs to reduce cardiovascular risk, have also been suggested to have protective effects against epilepsy. However, whether this association is causal remains unclear. We conducted a drug-target Mendelian randomization study to examine the effects of genetically predicted inhibition of 3 established lipid-lowering targets (3-hydroxy-3-methylglutaryl coenzyme A reductase [statins], Niemann-Pick C1-Like 1 [ezetimibe], and proprotein convertase subtilisin/kexin type 9 [PCSK9 inhibitors]) on epilepsy and its subtypes. The inverse-variance weighted approach served as the primary analysis, supplemented with multiple sensitivity tests to ensure robustness. Genetically proxied 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibition was associated with decreased risks of epilepsy (odds ratio [OR] = 0.87; 95% confidence interval [CI]: 0.82-0.92; P = 1.4 × 10-6) and focal epilepsy (OR = 0.83; 95% CI: 0.76-0.92; P = 1.5 × 10-4). Inhibition of Niemann-Pick C1-Like 1 corresponded to a lower risk of generalized epilepsy with tonic-clonic seizures (OR = 0.98; 95% CI: 0.97-0.98; P = 1.2 × 10-34) but increased risks of focal epilepsy (lesion negative, OR = 1.08; 95% CI: 1.06-1.09; P = 5.9 × 10-31), childhood absence epilepsy (OR = 1.06; 95% CI: 1.05-1.07; P = 1.8 × 10-21), and juvenile absence epilepsy (OR = 1.03; 95% CI: 1.01-1.05; P = 9.7 × 10-3). PCSK9 inhibition was linked to reduced risks of generalized epilepsy with tonic-clonic seizures (OR = 0.99; 95% CI: 0.98-1.00; P = 1.4 × 10-2), juvenile absence epilepsy (OR = 0.96; 95% CI: 0.93-1.00; P = 2.7 × 10-2), and juvenile myoclonic epilepsy (OR = 0.96; 95% CI: 0.93-1.00; P = 2.9 × 10-2). The effects of lipid-lowering drug targets on epilepsy risk vary by target and exhibit pleiotropy. Statins and PCSK9 inhibitors appear protective against epilepsy and several subtypes, whereas ezetimibe may increase susceptibility to certain subtypes. These results underscore the importance of considering target-specific effects when choosing lipid-lowering therapies for patients with or at risk of epilepsy.

#4

Specialization challenges and improvement strategies in pediatric epilepsy diagnosis and treatment under the leadership of tertiary hospitals: A multi-center cross-sectional survey in Chongqing.

Epileptic disorders : international epilepsy journal with videotape2026 Feb 27

To analyze the current state of primary pediatric epilepsy care, providing evidence for regional capacity-building strategies. The cross-sectional survey design was adopted to assess the epilepsy diagnostic and therapeutic capabilities of pediatric physicians from 37 medical institutions in the nine main urban districts and surrounding counties of Chongqing. Tertiary (56.8%, 21/37) and general hospitals (75.7%, 28/37) dominated healthcare, yet pediatric neurology lagged: only 48.7% (18/37) had dedicated teams. Key technologies like Video-EEG (43.2%) and genetic testing (54.1%) were underutilized; novel therapies (ketogenic diet 13.5%, neuromodulation 8.1%) were rarely applied. The median annual outpatient volume was 50 cases, and 33.3% of hospitals achieved >50% seizure freedom rate, yet 20.8% had >15% treatment inefficacy. Evident deficiencies of physician training: 52.4% (87/166) lacked systematic epilepsy training, and only 1.2% (2/166) had passed advanced EEG certification, resulting in only 19.3% (32/166) fully mastering epilepsy classification systems, while 31.9% demonstrated medication selection errors. Significant discrepancies in clinical decision-making: 18.7% (31/166) of physicians inappropriately selected carbamazepine for juvenile absence epilepsy, while 3.6% (6/166) adopted a high-risk immediate medication switching strategy. Fragmented patient management: 8.4% of physicians did not provide health education, and 59.0% (98/166) cited poor family adherence due to insufficient disease awareness. The regional referral rates varied widely (median: 17.5%, IQR: 35-5%). Tertiary hospitals undertake the main clinical burden but lack the construction of specialized infrastructure; lagging staff training leads to undermining the standardization of diagnosis and management; and the problem of the lack of systematic solutions in patient management persists. To enhance the overall service delivery, there are some proposed solutions including establishing hierarchical diagnosis and treatment networks, enhancing specialist training, and implementing regional quality control systems.

#5

Syndrome-specific and familial imaging traits in juvenile absence epilepsy.

Epilepsia2026 Jan 13

Juvenile absence epilepsy (JAE) is an idiopathic generalized epilepsy characterized by absences, generalized tonic-clonic seizures, and cognitive difficulties. In contrast to juvenile myoclonic epilepsy (JME), where distinct functional and structural brain alterations are well established, it remains unclear whether comparable changes are identifiable in absence-predominant syndromes. We aimed to delineate functional and structural correlates of the cognitive profile in people with JAE and to explore potential familial imaging traits. We acquired working memory functional magnetic resonance imaging (MRI) and high-resolution T1-weighted MRI in 23 individuals with JAE, 18 unaffected siblings, and 28 controls. Compared with both siblings and controls, patients showed increased motor cortex activation during the attention-only condition, but relative suppression of motor activity and inadequate default mode network deactivation with increasing working memory demand. Gray matter volume was reduced in sensorimotor regions and in the left inferior and middle frontal gyri in patients. Larger volumes in these frontal regions correlated with better language function. In contrast, increased gray matter volume in the dorsal midcingulate cortex was present in both patients and their siblings relative to controls. Our findings in JAE differ from the patterns of functional reorganization reported in JME, indicating that each syndrome involves distinct motor-cognitive pathophysiological mechanisms aligned with its seizure profile. Inferior frontal structural abnormalities likely contribute to the well-recognized language difficulties in JAE, whereas increased midcingulate gray matter volume may serve as a familial marker linked to attentional vulnerability.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC44 artigos no totalmostrando 115

2026

Effects of lipid-lowering drugs on epilepsy and its subtypes: A drug-target Mendelian randomization study.

Medicine
2026

Investigation of adolescent cases diagnosed with idiopathic/genetic generalized epilepsy in terms of self-esteem, aggression, body perception, and alexithymia.

European journal of pediatrics
2026

Specialization challenges and improvement strategies in pediatric epilepsy diagnosis and treatment under the leadership of tertiary hospitals: A multi-center cross-sectional survey in Chongqing.

Epileptic disorders : international epilepsy journal with videotape
2026

Syndrome-specific and familial imaging traits in juvenile absence epilepsy.

Epilepsia
2026

Bidirectional causal relationships between epilepsy subtypes and psychiatric disorders: A two-sample mendelian randomization study.

Journal of affective disorders
2026

Epilepsy and stroke: A mendelian Randomization study.

Epilepsy research
2026

Clinical and EEG predictors of treatment response in pediatric absence epilepsy - a single-center experience.

Neurologia i neurochirurgia polska
2026

Causal effect of Helicobacter pylori infection on brain health and potential mediation by immune cells.

European archives of psychiatry and clinical neuroscience
2025

Long-term seizure outcomes and the likelihood of antiseizure medication withdrawal in patients with juvenile absence epilepsy: A 10-year follow-up study.

Seizure
2025

Increased volumes of the precuneus and the pallidum in idiopathic generalized epilepsy.

Scientific reports
2025

[Clinical response to brivaracetam in two cases of drug-resistant juvenile absence epilepsy].

Anales del sistema sanitario de Navarra
2025

Focus on epilepsy and epilepsy syndromes in children with autism spectrum disorders: a study of 74 patients.

Brain &amp; development
2025

Absence-to-bilateral-tonic-clonic seizure with focal features: A diagnostic challenge.

Epileptic disorders : international epilepsy journal with videotape
2025

Autobiographical memory impairment in genetic generalized epilepsies: neurocognitive and pathophysiological determinants.

Arquivos de neuro-psiquiatria
2024

Power Spectral Density and Default Mode Network Connectivity in Generalized Epilepsy Syndromes: What to Expect from Drug-Resistant Patients.

Biomedicines
2025

Unsupervised clustering of a deeply phenotyped cohort of adults with idiopathic generalized epilepsy.

Epilepsia
2025

Assessing the Need for Repeat EEG in Pediatric Patients with Idiopathic Generalized Epilepsy After Anti-Seizure Medication Withdrawal Following Seizure Freedom.

Journal of child neurology
2024

Metabolite Associations with Childhood and Juvenile Absence Epilepsy: A Bidirectional Mendelian Randomization Study.

Psychiatry and clinical psychopharmacology
2024

Genetic heterogeneity in familial forms of genetic generalized epilepsy: from mono- to oligogenism.

Human genomics
2024

Generalized spike-waves in idiopathic generalized epilepsies: Does their frequency matter?

Brain and behavior
2024

Lamotrigine vs levetiracetam in female patients of childbearing age with juvenile absence epilepsy: A Bayesian reanalysis.

Epilepsia
2024

Causal links between serum micronutrients and epilepsy: a Mendelian randomization analysis.

Frontiers in neurology
2024

Assessing the impact of circulating inflammatory cytokines and proteins as drivers and therapeutic targets in epilepsy: A Mendelian randomization study.

Epilepsy &amp; behavior : E&amp;B
2024

Challenges in the Management of Narcolepsy in a Resource Limited Setting: A Case Report.

Cureus
2024

Exploring brain network oscillations during seizures in drug-naïve patients with juvenile absence epilepsy.

Frontiers in neurology
2024

Analyzing the causal relationship between lipid-lowering drug target genes and epilepsy: a Mendelian randomization study.

Frontiers in neurology
2024

Long-term prognosis of patients with photosensitive idiopathic generalized epilepsy.

Seizure
2024

Drug-resistant generalized epilepsies: Revisiting the frontiers of idiopathic generalized epilepsies.

Revue neurologique
2024

Clearance for Driving in Genetic Generalized Epilepsy.

Journal of child neurology
2023

Thyroid function and epilepsy: a two-sample Mendelian randomization study.

Frontiers in human neuroscience
2024

Lack of causal association between epilepsy and dementia: A Mendelian randomization analysis.

Epilepsy &amp; behavior : E&amp;B
2024

Exploring causal correlations between systemic inflammatory cytokines and epilepsy: A bidirectional Mendelian randomization study.

Seizure
2024

Adolescent-Onset Epilepsy: Clinical Features and Predictive Factors for First-Year Seizure Freedom.

Neuropediatrics
2023

Dynamic alterations of striatal-related functional networks in juvenile absence epilepsy.

Epilepsy &amp; behavior : E&amp;B
2023

Nonconvulsive Status Epilepticus With Generalized Spike-and-Wave Discharges: Pathophysiological and Nosological Considerations.

Cureus
2023

[Idiopathic generalized epilepsies].

Medicina
2023

Relationship Between Electroencephalography and Seizure Outcome in Typical Absence Seizures in Children.

Pediatric neurology
2023

Cognitive phenotype of juvenile absence epilepsy: An investigation of patients and unaffected siblings.

Epilepsia
2023

Outcome of Absence Epilepsy With Onset at 8-11 Years of Age: Watershed Ages When Syndromes Overlap.

Journal of child neurology
2023

Changes in awake and sleep electroencephalography characteristics after 1-year treatment for childhood and juvenile absence epilepsy.

Seizure
2023

EEG phase synchronization during absence seizures.

Frontiers in neuroinformatics
2023

Antiseizure medications for idiopathic generalized epilepsies: a systematic review and network meta-analysis.

Journal of neurology
2023

Alzheimer Disease and Epilepsy: A Mendelian Randomization Study.

Neurology
2023

The causal effect of serum 25-hydroxyvitamin D levels on epilepsy: A two-sample Mendelian randomization study.

Epilepsia open
2023

Sub-region analysis of DMD gene in cases with idiopathic generalized epilepsy.

Neurogenetics
2024

Clinical application of trio-based whole-exome sequencing in idiopathic generalized epilepsy.

Seizure
2023

A cross-sectional investigation of cognition and epileptiform discharges in juvenile absence epilepsy.

Epilepsia
2023

Clinical and Electrophysiological Features Predicting Response to Antiseizure Medications in Juvenile Absence Epilepsy.

Neuropediatrics
2022

Severe Raynaud's phenomenon from ethosuximide raised concern over possible onset of systemic vasculitis: a case report.

Pediatric rheumatology online journal
2023

Juvenile absence epilepsy: integrating photosensitivity and autonomic focal epileptic symptoms.

Acta neurologica Belgica
2022

Childhood vs. juvenile absence epilepsy: How to make a diagnosis.

Seizure
2023

Idiopathic generalized epilepsies: Analysis of 101 patients.

Medicina clinica
2022

Efficacy and tolerability of brivaracetam monotherapy in childhood and juvenile absence epilepsy: An innovative adaptive trial design.

Epilepsia open
2022

School performance and psychiatric comorbidity in juvenile absence epilepsy and juvenile myoclonic epilepsy: a Danish population-based cohort study.

Journal of neurology
2022

ILAE definition of the Idiopathic Generalized Epilepsy Syndromes: Position statement by the ILAE Task Force on Nosology and Definitions.

Epilepsia
2022

Validation of Pediatric Idiopathic Generalized Epilepsy Diagnoses from the Danish National Patient Register During 1994‒2019.

Clinical epidemiology
2022

Recurrence rates and risk factors for seizure recurrence following antiseizure medication withdrawal in adolescent patients with genetic generalized epilepsy.

Epilepsia open
2023

Effect of Sleep on Epileptic Discharges in Patients with Idiopathic Generalized Epilepsy.

Clinical EEG and neuroscience
2021

Different circuitry dysfunction in drug-naive patients with juvenile myoclonic epilepsy and juvenile absence epilepsy.

Epilepsy &amp; behavior : E&amp;B
2022

Genetic generalized epilepsies in adults - challenging assumptions and dogmas.

Nature reviews. Neurology
2021

Involvement of ADGRV1 Gene in Familial Forms of Genetic Generalized Epilepsy.

Frontiers in neurology
2021

Neuropsychiatric comorbidities in genetic/idiopathic generalized epilepsies and their effects on psychosocial outcomes.

Epilepsy &amp; behavior : E&amp;B
2021

Association Analysis of Candidate Variants in Admixed Brazilian Patients With Genetic Generalized Epilepsies.

Frontiers in genetics
2021

Therapeutic approach to difficult-to-treat typical absences and related epilepsy syndromes.

Expert review of clinical pharmacology
2021

Portrait of My Father: Frida Kahlo's Intimate Relation to Epilepsy.

European neurology
2021

Age-related morphological differences in the spike-and-wave complexes of absence epilepsy.

Epilepsy research
2021

Social outcome and psychiatric comorbidity of generalized epilepsies - A case-control study.

Epilepsia
2021

Alternatives to valproate in girls and women of childbearing potential with Idiopathic Generalized Epilepsies: state of the art and guidance for the clinician proposed by the Epilepsy and Gender Commission of the Italian League Against Epilepsy (LICE).

Seizure
2021

Aberrant basal ganglia-thalamo-cortical network topology in juvenile absence epilepsy: A resting-state EEG-fMRI study.

Seizure
2020

Genetic Landscape of Common Epilepsies: Advancing towards Precision in Treatment.

International journal of molecular sciences
2020

Network characteristics of genetic generalized epilepsy: Are the syndromes distinct?

Seizure
2020

Computationally-Efficient Algorithm for Real-Time Absence Seizure Detection in Wearable Electroencephalography.

International journal of neural systems
2020

How to diagnose and classify idiopathic (genetic) generalized epilepsies.

Epileptic disorders : international epilepsy journal with videotape
2020

Trait impulsivity correlates with active myoclonic seizures in genetic generalized epilepsy.

Epilepsy &amp; behavior : E&amp;B
2020

Clinico-electrographic characteristics and classification of genetic generalized epilepsy in Oman.

Epilepsy research
2020

Genetic generalized epilepsies with frontal lesions mimicking migratory disorders on the epilepsy monitoring unit.

Epilepsia open
2020

Idiopathic (genetic) generalized epilepsies with absences: clinical and electrographic characteristics and seizure outcome.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2020

High-power, frontal-dominant ripples in absence status epilepticus during childhood.

Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology
2020

Cognitive Function in Genetic Generalized Epilepsies: Insights From Neuropsychology and Neuroimaging.

Frontiers in neurology
2020

Social impairment and stigma in genetic generalized epilepsies.

Epilepsy &amp; behavior : E&amp;B
2020

Default mode network dysfunction in idiopathic generalised epilepsy.

Epilepsy research
2020

Praxis induction and its relationship with cognition in genetic generalized epilepsy.

Epilepsy &amp; behavior : E&amp;B
2020

Single-subject manual independent component analysis and resting state fMRI connectivity outcomes in patients with juvenile absence epilepsy.

Magnetic resonance imaging
2019

Genomic and Personalized Medicine Perspective in Genetic Generalized Epilepsy.

Archives of Iranian medicine
2019

Synaptic clustering differences due to different GABRB3 mutations cause variable epilepsy syndromes.

Brain : a journal of neurology
2019

Peripapillary retinal nerve fibre layer thinning in genetic generalized epilepsy.

Seizure
2018

Juvenile absence epilepsy relapsing as recurrent absence status, mimicking transient global amnesia, in an elderly patient.

Epileptic disorders : international epilepsy journal with videotape
2018

Practice guideline update summary: Efficacy and tolerability of the new antiepileptic drugs I: Treatment of new-onset epilepsy: Report of the American Epilepsy Society and the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology.

Epilepsy currents
2018

Perampanel in routine clinical use in idiopathic generalized epilepsy: The 12-month GENERAL study.

Epilepsia
2018

Multi-gene panel testing in Korean patients with common genetic generalized epilepsy syndromes.

PloS one
2018

Practice guideline update summary: Efficacy and tolerability of the new antiepileptic drugs I: Treatment of new-onset epilepsy: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology and the American Epilepsy Society.

Neurology
2018

Relapse after treatment withdrawal of antiepileptic drugs for Juvenile Absence Epilepsy and Juvenile Myoclonic Epilepsy.

Seizure
2018

Electroclinical and prognostic characteristics of epilepsy patients with photosensitivity.

Ideggyogyaszati szemle
2018

Source analysis of epileptiform discharges in absence epilepsy using Magnetoencephalography (MEG).

Epilepsy research
2017

[Pathomorphosis of idiopathic generalized epilepsy. Juvenile forms].

Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova
2017

Lacosamide for refractory generalized tonic-clonic seizures of non-focal origin in clinical practice: A clinical and VEEG study.

Epilepsy &amp; behavior case reports
2017

Long-term outcome in adolescent-onset generalized genetic epilepsies.

Epilepsia
2017

Gene expression analysis in untreated absence epilepsy demonstrates an inconsistent pattern.

Epilepsy research
2017

Resistance to valproic acid as predictor of treatment resistance in genetic generalized epilepsies.

Epilepsia
2017

[Classification of idiopathic generalised epilepsies in patients over 16 years of age].

Revista de neurologia
2016

Impaired consciousness in patients with absence seizures investigated by functional MRI, EEG, and behavioural measures: a cross-sectional study.

The Lancet. Neurology
2017

Can EEG Differentiate Among Syndromes in Genetic Generalized Epilepsy?

Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society
2016

Cortical and subcortical brain alterations in Juvenile Absence Epilepsy.

NeuroImage. Clinical
2016

Analysis of rare copy number variation in absence epilepsies.

Neurology. Genetics
2016

Role for serotonin2A (5-HT2A) and 2C (5-HT2C) receptors in experimental absence seizures.

Neuropharmacology
2016

[Anticonvulsive Therapy after the First Unprovoked Seizure – Pros and Cons].

Praxis
2016

Focal EEG features and therapeutic response in patients with juvenile absence and myoclonic epilepsy.

Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology
2015

Celiac Disease and Juvenile Absence Epilepsy.

Pediatric emergency care
2016

Epileptiform K-Complexes and Sleep Spindles: An Underreported Phenomenon in Genetic Generalized Epilepsy.

Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society
2016

Seizure outcome in patients with juvenile absence epilepsy.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2015

Seizure and Psychosocial Outcomes of Childhood and Juvenile Onset Generalized Epilepsies: Wolf in Sheep's Clothing, or Well-Dressed Wolf?

Epilepsy currents
2015

Investigation of GRIN2A in common epilepsy phenotypes.

Epilepsy research
2015

Focal seizure symptoms in idiopathic generalized epilepsies.

Neurology
2015

Treatment of pediatric epilepsy in Poland.

European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society
2014

[Antiepileptic drugs and seizure aggravation].

Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

Ainda não temos associações cadastradas para Epilepsia de ausências juvenil.

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Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Epilepsia de ausências juvenil

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Ordenadas pelo número de sintomas em comum.

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. Investigation of adolescent cases diagnosed with idiopathic/genetic generalized epilepsy in terms of self-esteem, aggression, body perception, and alexithymia.
    European journal of pediatrics· 2026· PMID 41857384mais citado
  2. Causal effect of Helicobacter pylori infection on brain health and potential mediation by immune cells.
    European archives of psychiatry and clinical neuroscience· 2026· PMID 40991030mais citado
  3. Effects of lipid-lowering drugs on epilepsy and its subtypes: A drug-target Mendelian randomization study.
    Medicine· 2026· PMID 41861198mais citado
  4. Specialization challenges and improvement strategies in pediatric epilepsy diagnosis and treatment under the leadership of tertiary hospitals: A multi-center cross-sectional survey in Chongqing.
    Epileptic disorders : international epilepsy journal with videotape· 2026· PMID 41757792mais citado
  5. Syndrome-specific and familial imaging traits in juvenile absence epilepsy.
    Epilepsia· 2026· PMID 41531116mais citado
  6. Bidirectional causal relationships between epilepsy subtypes and psychiatric disorders: A two-sample mendelian randomization study.
    J Affect Disord· 2026· PMID 41443308recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:1941(Orphanet)
  2. MONDO:0800453(MONDO)
  3. Epilepsia(PCDT · Ministério da Saúde)
  4. GARD:2162(GARD (NIH))
  5. Variantes catalogadas(ClinVar)
  6. Busca completa no PubMed(PubMed)
  7. Q3726905(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

Epilepsia de ausências juvenil
Compêndio · Raras BR

Epilepsia de ausências juvenil

ORPHA:1941 · MONDO:0800453
🇧🇷 Brasil SUS
Geral
Prevalência
1-9 / 100 000
Herança
Multigenic/multifactorial, Unknown
CID-10
G40.3 · Epilepsia e síndromes epilépticas generalizadas idiopáticas
CID-11
Ensaios
3 ativos
Medicamentos
1 registrados
Início
Adolescent
Prevalência
0.0 (Europe)
MedGen
UMLS
C0014553
Repurposing
14 candidatos
aminohydroxybutyric-acidcarbonic anhydrase inhibitor
diclofenamidesuccinimide antiepileptic
ethosuximideglutamate receptor antagonist
+11 outros
EuropePMC
Wikidata
Papers 10a
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