Raras
Buscar doenças, sintomas, genes...
Síndrome Foix-Chavany-Marie
ORPHA:2048CID-10 · G12.2DOENÇA RARA

A Síndrome de Foix-Chavany-Marie (SFCM) é um tipo de paralisia cerebral que atinge os nervos que controlam a parte inferior do rosto, garganta e língua. Ela se caracteriza por dificuldade grave para falar (disartria) e para engolir (disfagia), associadas a uma paralisia que afeta os dois lados dos músculos da face, faringe (garganta), língua e mastigação. Uma característica importante é que há uma diferença notável entre os movimentos voluntários e involuntários: a pessoa tem dificuldade de mover esses músculos por vontade própria, mas os movimentos automáticos ou involuntários desses músculos são mantidos.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

A Síndrome de Foix-Chavany-Marie (SFCM) é um tipo de paralisia cerebral que atinge os nervos que controlam a parte inferior do rosto, garganta e língua. Ela se caracteriza por dificuldade grave para falar (disartria) e para engolir (disfagia), associadas a uma paralisia que afeta os dois lados dos músculos da face, faringe (garganta), língua e mastigação. Uma característica importante é que há uma diferença notável entre os movimentos voluntários e involuntários: a pessoa tem dificuldade de mover esses músculos por vontade própria, mas os movimentos automáticos ou involuntários desses músculos são mantidos.

Publicações científicas
98 artigos
Último publicado: 2025

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
<1 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Casos conhecidos
150
pacientes catalogados
Início
All ages
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: G12.2
🇧🇷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

Encontrou um erro ou informação desatualizada? Sugira uma correção →

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
3 sintomas
📏
Crescimento
1 sintomas
🦴
Ossos e articulações
1 sintomas

+ 3 sintomas em outras categorias

Características mais comuns

90%prev.
Anormalidade da voz
Muito frequente (99-80%)
90%prev.
Diabetes materna
90%prev.
Fala ausente
Muito frequente (99-80%)
90%prev.
Convulsão
Muito frequente (99-80%)
90%prev.
Atraso global do desenvolvimento
Muito frequente (99-80%)
90%prev.
Morfologia anormal do polegar
Muito frequente (99-80%)
8sintomas
Muito frequente (8)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 8 características clínicas mais associadas, ordenadas por frequência.

Anormalidade da vozAbnormality of the voice
Muito frequente (99-80%)90%
Diabetes maternaMaternal diabetes
Muito frequente90%
Fala ausenteAbsent speech
Muito frequente (99-80%)90%
ConvulsãoSeizure
Muito frequente (99-80%)90%
Atraso global do desenvolvimentoGlobal developmental delay
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2025
Total histórico98PubMed
Últimos 10 anos49publicações
Pico20219 papers
Linha do tempo
2025Hoje · 2026📈 2021Ano 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

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Síndrome Foix-Chavany-Marie

🗺️

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

Pesquisa e ensaios clínicos

Nenhum ensaio clínico registrado para esta condição.

🧪 Está conduzindo uma pesquisa?
Divulgue para pacientes e familiares que acompanham esta doença.
Divulgar pesquisa →

Publicações mais relevantes

Timeline de publicações
42 papers (10 anos)
#1

A Case of Foix-Chavany-Marie Syndrome with History of Glioblastoma Showing Partial Recovery.

European journal of case reports in internal medicine2025

Foix-Chavany-Marie syndrome (FCMS) or bilateral opercular syndrome (OPS) is a rare pseudobulbar palsy characterized by facial, lingual, pharyngeal, and masticatory voluntary muscle paralysis resulting in anarthria with preservation of autonomic, involuntary, and reflexive functions. Damage to the posterior part of the inferior frontal gyrus and inferior part of precentral gyrus play a role in the pathogenesis of FCMS. We report a rare case of bilateral OPS following an acute right middle cerebral artery (MCA) infarct in a patient with history of glioblastoma, and resection in the left MCA territory within the cingulate gyrus, showing recovery of speech and swallowing despite intensive bilateral opercular lesions owing to extensive multidisciplinary team support. However, the patient was discharged with a percutaneous endoscopic gastrostomy tube for long-term enteral feeding support due to partial recovery. The patient's history of glioblastoma, left MCA cingulate gyrus resection and right MCA infarction with automatic-voluntary dissociation led to the diagnosis of FCMS. Rehabilitation surprisingly showed mild improvement in speech and swallowing despite extensive bilateral opercular lesions proving that there are still chances of improvement in speech and swallowing in OPS with the right multidisciplinary approach. Patients with a history of brain tumours like glioblastoma can develop bilateral OPS later in life in case of other vascular events that cause lesions in a previously unaffected operculum, triggering symptoms. Extensive involvement of the speech and language team is of significant in the management of FCMS cases as above where some recovery might still be seen. Foix-Chavany-Marie syndrome (FCMS) can be caused by bilateral opercular lesions (new or old or a combination of both) in patients with history of high-grade brain tumours.Patients with bilateral opercular syndrome experience full or partial or no recovery at all of speech and swallowing, hence proper rehabilitation with a Speech and Language Team is significant.Understanding automatic-voluntary dissociation in FCMS and being able to differentiate it from bulbar palsy and other similar phenomena is crucial in making a diagnosis of FCMS.

#2

Partial Anterior Opercular Syndrome as Surgical Complication: Case Presentation and Brief Review of Literature.

Case reports in neurological medicine2025

Anterior opercular syndrome (a.k.a. Foix-Chavany-Marie syndrome) is a rare neurological condition, described as a paralysis of the mouth and tongue usually caused by a bilateral lesion of the frontal opercular area. The patient presents with speaking, chewing, and swallowing impairment, but autonomic and emotional functions-like smiling and yawning-are typically preserved. We present our patient's clinical data after critical analysis, together with a brief literature review about anterior opercular syndrome caused by unilateral opercular lesions. To our knowledge, less than 20 cases of anterior opercular syndrome caused by unilateral lesions are described in the literature. In some patients, a contralateral lesion can be detected on brain imaging in regions different from the anterior opercular cortex. This syndrome can rarely occur as a consequence of unilateral opercular cortex damage. The possible role of contralateral lesions located in neuronal pathways functionally related to the anterior operculum requires further investigation.

#3

Speech motor rehabilitation in opercular syndrome: a case study.

European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery2025 Oct

Anterior Opercular Syndrome, or Foix-Chavany-Marie Syndrome (FCMS), is a rare pseudobulbar palsy that severely impacts speech and swallowing due to orofacial muscle weakness. Limited research exists on therapeutic approaches targeting motor speech deficits in this condition. This case study aimed to evaluate the efficacy of speech based oromotor exercises in improving speech intelligibility and oromotor control in a 20-year-old male with FCMS. A personalized therapy regimen emphasizing the strength and mobility of jaw, lip, and tongue and exercises to improve intraoral pressure was implemented thrice weekly over three weeks. Progress was systematically recorded using the domains of Frenchay Dysarthria Assessment. Steady improvements in articulatory precision, speech clarity, and specific oromotor functions were observed, with significant gains in intelligibility was observed. However, slow progress was noted in tongue lateralization and intraoral pressure. This study demonstrates the potential of structured speech-based oromotor therapy to enhance speech outcomes in FCMS, offering a foundation for further research into targeted therapeutic models for this rare condition.

#4

Foix-Chavany-Marie syndrome: A rare presentation with arachnoid cyst involvement.

Radiology case reports2025 Sep

Foix-Chavany-Marie syndrome, also known as opercular syndrome, is a rare neurological condition typically caused by bilateral lesions of the opercular cortex. Involvement of arachnoid cysts in the pathogenesis is extremely uncommon. We report the case of a 66-year-old male who presented with sudden-onset dysarthria, dysphagia, and bilateral lower facial paralysis, but retained the ability to yawn and smile, demonstrating the characteristic dissociation between voluntary and involuntary orofacial movements. Magnetic resonance imaging revealed an acute infarction in the left corona radiata and a right-sided arachnoid cyst, leading to a diagnosis of Foix-Chavany-Marie syndrome. This case highlights a classic presentation of Foix-Chavany-Marie syndrome with a potentially novel pathogenic contribution from an arachnoid cyst.

#5

Opercular Syndrome without Involvement of Opercular Area-An Uncommon Presentation of Stroke: A Case Report.

The Journal of the Association of Physicians of India2025 Jan

Foix Chavany Marie syndrome (FCMS), or opercular syndrome, is a rare type of pseudobulbar palsy characterized by paralysis of bilateral facio-linguovelo-masticatory and pharyngeal muscles with automatic-voluntary dissociation. This syndrome was first described by Magnus in 1837 and further defined by two French neurologists, Charles Foix and Jean Alfred Emile Chavany, along with one French pediatrician, Julie Marie, who reported it first in 1926. Since then, a few cases have been reported across the world. We hereby report a case of FCMS in a 61-year-old male patient who presented to us with two different cerebrovascular events.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC74 artigos no totalmostrando 49

2025

A Case of Foix-Chavany-Marie Syndrome with History of Glioblastoma Showing Partial Recovery.

European journal of case reports in internal medicine
2025

Partial Anterior Opercular Syndrome as Surgical Complication: Case Presentation and Brief Review of Literature.

Case reports in neurological medicine
2025

Speech motor rehabilitation in opercular syndrome: a case study.

European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery
2025

Foix-Chavany-Marie syndrome: A rare presentation with arachnoid cyst involvement.

Radiology case reports
2025

Opercular Syndrome without Involvement of Opercular Area-An Uncommon Presentation of Stroke: A Case Report.

The Journal of the Association of Physicians of India
2024

A Rare Case of a Good Neurological Outcome following Traumatic Foix-Chavany-Marie Syndrome.

Case reports in critical care
2024

Recurrent lacunar strokes in a patient with small vessel disease: rare but not negligible cause of Foix-Chavany-Marie syndrome.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2024

Foix-Chavany-Marie syndrome due to unilateral opercular infarction-A case report.

Clinical case reports
2023

Corticobasal syndrome mimicking Foix-Chavany-Marie syndrome with suggested 4-repeat tauopathy by tau PET.

BMC geriatrics
2023

Atypical Presentations of Foix-Chavany-Marie Syndrome (FCMS) in Stroke.

Cureus
2022

[A rare caseof anarthriaand dysphagia].

Ugeskrift for laeger
2022

A Case of Foix-Chavany-Marie Syndrome With Asynchronous Bilateral Opercular Infarcts and Chronic Bilateral Cerebellar Infarcts.

Cureus
2022

Foix-Chavany-Marie syndrome as a heralding feature of acute disseminated encephalomyelitis.

Acta neurologica Belgica
2022

Foix-Chavany-Marie Syndrome as Result of Acute Bilateral Frontal-Opercular Strokes.

The Neurohospitalist
2022

The nature of the automatic-voluntary dissociation in Foix-Chavany-Marie syndrome.

Acta neurologica Belgica
2021

Foix-Chavany-Marie Syndrome - A Rare Presentation of Unilateral Opercular Infarction.

Neurology India
2021

Foix-Chavany-Marie syndrome due to bilateral opercular ischemic lesions.

Acta neurologica Belgica
2021

The Foix-Chavany-Marie syndrome due to herpes symplex virus encephalitis type 2.

Neurologia
2021

A case of aphemia following non-dominant sub-insular stroke: unveiling the Foix-Chavany-Marie phenomenon.

Neurocase
2021

Foix-Chavany-Marie Syndrome as a Manifestation of Unilateral Opercular Stroke.

European journal of case reports in internal medicine
2021

Foix-Chavany-Marie syndrome due to unilateral anterior opercular infarction with leukoaraiosis.

Proceedings (Baylor University. Medical Center)
2021

Importance of Rapid Clinical Recognition of the Anterior Opercular Syndrome (Foix-Chavany-Marie Syndrome): A Case Report.

Case reports in neurology
2021

The Frontal Aslant Tract: A Systematic Review for Neurosurgical Applications.

Frontiers in neurology
2021

Ischemic stroke during the puerperium presenting as a bilateral anterior opercular (Foix-Chavany-Marie) syndrome.

Clinical neurology and neurosurgery
2020

Rare case of bilateral carotid artery dissection presenting with Foix-Chavany-Marie syndrome.

BMJ case reports
2020

Syndrome of the Trephined presenting as Foix-Chavany-Marie syndrome.

Clinical neurology and neurosurgery
2019

Foix-Chavany-Marie Syndrome due to Unilateral Anterior Opercular Damage with Contralateral Infarction of Corona Radiata.

Case reports in neurology
2020

Foix-Chavany-Marie syndrome due to type E TDP43 pathology.

Neuropathology and applied neurobiology
2020

French neurologists Charles Foix and Jean Alfred Émile Chavany and French pediatrician Julien Marie and the Foix-Chavany-Marie syndrome.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2019

An unusual case of bilateral anterior opercular syndrome from a neuro-rehabilitation perspective.

JAAPA : official journal of the American Academy of Physician Assistants
2019

Teaching Video NeuroImages: Foix-Chavany-Marie syndrome.

Neurology
2019

[Foix-Chavany-Marie Syndrome: A Clinical Overview].

Brain and nerve = Shinkei kenkyu no shinpo
2019

The patient's perspective: follow-up Foix-Chavany-Marie syndrome secondary to bilateral traumatic operculum injury.

Acta neurochirurgica
2018

Foix-Chavany-Marie syndrome as the presenting sign of HIV-related PML.

Neurology. Clinical practice
2018

Foix-Chavany-Marie syndrome secondary to bilateral traumatic operculum injury.

Acta neurochirurgica
2019

Foix-Chavany-Marie Syndrome Induced by a Unilateral Brain Abscess.

Internal medicine (Tokyo, Japan)
2018

Jaw clonus and opercular syndrome in ALS: a rare and interesting finding.

Acta neurologica Belgica
2017

Dysphagia management in bilateral frontal opercular syndrome (Foix-Chavany-Marie syndrome).

BMJ case reports
2018

Progressive anterior operculum syndrome due to frontotemporal lobar degeneration.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2017

Bilateral Anterior Opercular Syndrome.

The Journal of the Association of Physicians of India
2017

Reversible opercular syndrome secondary to osmotic demyelination.

Clinical neurology and neurosurgery
2017

Prolonged acyclovir treatment in a child with opercular syndrome related to herpes simplex encephalitis.

Journal of infection and public health
2017

Neural substrates of the 'low-level' system for speech articulation: Evidence from primary opercular syndrome.

Journal of neuropsychology
2016

Clinical Reasoning: An 11-year-old boy with language disorder and epilepsy.

Neurology
2015

Foix-Chavany-Marie syndrome after an isolated pontine infarct: A 7-year follow-up.

Neurology India
2015

[Foix-Chavany-Marie syndrome presenting as multiple sclerosis].

Revista de neurologia
2015

Foix-Chavany-Marie Syndrome after Unilateral Stroke.

European neurology
2015

Foix-Chavany-Marie or opercular syndrome.

JBR-BTR : organe de la Societe royale belge de radiologie (SRBR) = orgaan van de Koninklijke Belgische Vereniging voor Radiologie (KBVR)
2016

Anterior opercular syndrome induced by Epstein-Barr virus encephalitis.

Neurocase
Ver todos os 74 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

Ainda não temos associações cadastradas para Síndrome Foix-Chavany-Marie.

É de uma associação que acompanha esta doença? Fale com a gente →

Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Síndrome Foix-Chavany-Marie

Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.

Tire suas dúvidas

Perguntas, dicas e experiências compartilhadas aqui na página

Participe da discussão

Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.

Fazer login

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. A Case of Foix-Chavany-Marie Syndrome with History of Glioblastoma Showing Partial Recovery.
    European journal of case reports in internal medicine· 2025· PMID 41536455mais citado
  2. Partial Anterior Opercular Syndrome as Surgical Complication: Case Presentation and Brief Review of Literature.
    Case reports in neurological medicine· 2025· PMID 41041017mais citado
  3. Speech motor rehabilitation in opercular syndrome: a case study.
    European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery· 2025· PMID 40699226mais citado
  4. Foix-Chavany-Marie syndrome: A rare presentation with arachnoid cyst involvement.
    Radiology case reports· 2025· PMID 40677864mais citado
  5. Opercular Syndrome without Involvement of Opercular Area-An Uncommon Presentation of Stroke: A Case Report.
    The Journal of the Association of Physicians of India· 2025· PMID 39893537mais citado

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:2048(Orphanet)
  2. MONDO:0023171(MONDO)
  3. GARD:2351(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q1435202(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

Síndrome Foix-Chavany-Marie
Compêndio · Raras BR

Síndrome Foix-Chavany-Marie

ORPHA:2048 · MONDO:0023171
Prevalência
<1 / 1 000 000
Casos
150 casos conhecidos
Herança
Not applicable
CID-10
G12.2 · Doença do neurônio motor
Início
All ages
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C2931412
EuropePMC
Wikidata
Papers 10a
DiscussaoAtiva

Nenhuma novidade ainda. O agente esta monitorando.

0membros
0novidades