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Síndrome de Marcus-Gunn
ORPHA:91412CID-10 · Q07.8CID-11 · 9A03.00OMIM 154600DOENÇA RARA

A síndrome de Marcus-Gunn é caracterizada por ptose associada à sincinesia maxilopalpebral.

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Introdução

O que você precisa saber de cara

📋

A síndrome de Marcus-Gunn é caracterizada por ptose associada à sincinesia maxilopalpebral.

Pesquisas ativas
1 ensaio
1 total registrados no ClinicalTrials.gov
Publicações científicas
45 artigos
Último publicado: 2025

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
0.0
Worldwide
Início
Infancy
+ neonatal
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: Q07.8
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (5)
0202010503
Cariótipo — bandas G, Q ou Rgenetic_test
0202010600
Pesquisa de microdeleções/microduplicações por FISHlab_test
0202010694
Sequenciamento completo do exoma (WES)rehabilitation
0202010260
Dosagem de alfa-fetoproteína
0301070040
Atendimento em reabilitação — doenças raras
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

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Sinais e sintomas

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

Partes do corpo afetadas

👁️
Olhos
4 sintomas
😀
Face
2 sintomas
🧠
Neurológico
1 sintomas
👂
Ouvidos
1 sintomas
🫘
Rins
1 sintomas
📏
Crescimento
1 sintomas

+ 10 sintomas em outras categorias

Características mais comuns

100%prev.
Sincinesia de Marcus Gunn (piscar da mandíbula)
55%prev.
Estrabismo
Frequente (79-30%)
55%prev.
Hipotropia
Frequente (79-30%)
55%prev.
Ptose unilateral
Frequente (79-30%)
55%prev.
Ambliopia
Frequente (79-30%)
55%prev.
Morfologia anormal do quinto nervo craniano
Frequente (79-30%)
23sintomas
Muito frequente (1)
Frequente (5)
Ocasional (5)
Muito raro (10)
Sem dados (2)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 23 características clínicas mais associadas, ordenadas por frequência.

Sincinesia de Marcus Gunn (piscar da mandíbula)Marcus Gunn jaw winking synkinesis
Muito frequente100%
EstrabismoStrabismus
Frequente (79-30%)55%
HipotropiaHypotropia
Frequente (79-30%)55%
Ptose unilateralUnilateral ptosis
Frequente (79-30%)55%
AmbliopiaAmblyopia
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2025
Total histórico45PubMed
Últimos 10 anos15publicações
Pico20193 papers
Linha do tempo
2025Hoje · 2026🧪 2004Primeiro ensaio clínico📈 2019Ano de pico
Publicações por ano (últimos 10 anos)

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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

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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.
·Pré-clínico1
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 1 ensaio
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Síndrome de Marcus-Gunn

🗺️

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

1 pesquisa recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

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Publicações mais relevantes

Timeline de publicações
15 papers (10 anos)
#1

Temperature-related atypical first-bite syndrome: a rare case report.

Frontiers in oral health2025

First bite syndrome (FBS) manifests is characterized by severe parotid pain triggered by the first bite of food, with or without muscle spasms. The pain typically diminishes with subsequent bites. We report a case of 32-year-old male with Marcus-Gunn syndrome (30-year history), no prior head or neck surgery, tumors, infections, or temporomandibular joint disease. Since age of 15, he experiences bilateral parotid pain and spasms exclusively upon consuming ice cream in high ambient temperatures post-summer exercise. Symptoms resolve spontaneously within 30 s and persist despite trials of heat therapy, massage, and physiotherapy. While approximately 42% of FBS cases have identifiable etiology, others implicate aberrant activity in the auriculotemporal, greater auricular, or cervical sympathetic nerves. In this patient, symptoms occur only with cold food ingestion in hot environments, suggesting involvement of the trigeminal nerve's mandibular branch in oral thermosensation. To our knowledge, this is the first reported case linking FBS symptoms to temperature sensation.

#2

sc-MULTI-omics approach in nano-rare diseases: understanding the pathophysiological mechanism of Mulvihill-Smith Syndrome.

Functional &amp; integrative genomics2025 May 09

Mulvihill-Smith Syndrome (MSS) is a clinically complex and genetically unsolved nano-rare disorder with only 12 patients reported in the literature. Most patients (91%) have immunological impairments, succumb to infection, and might develop cancer later in life. Its pathogenesis remains elusive and therapeutic options are limited. We used single-cell MULTI-omics (sc-MULTI-omics), combining transcriptomics (gene expression, TCR, and BCR repertoire) and proteogenomic (Cellular Indexing of Transcriptomes and Epitopes by Sequencing; CITE-seq), to decipher the pathophysiology of nano-rare disease patient. We report a new patient who is a 16-year-old girl. She had an increased leukocyte counts and typical manifestations of MSS such as short stature, older appearance, multiple pigmented nevi, microcephaly, monolateral keratoconus, Marcus-Gunn syndrome, hearing loss, vitamin D deficiency, mild hypercortisolism, and diabetes mellitus with very high insulin resistance (T3DM). sc-MULTI-omics CITE-seq showed that the MSS patient had increased central memory CD4+ T cells as well as effector memory CD8+ T cells, whilst reduced naïve T cells (both CD4+ and CD8+ T cells). Furthermore, we identified genes and pathways associated with the progeria-like phenotype, inflammation, and cancer progression, which may contribute to the clinical signs of MSS. sc-MUTLI-omics CITE-seq analyses improve our understanding of complex human disease pathophysiology and provides an alternative approach in personalized medicine in nano-rare disease.

#3

Marcus Gunn syndrome: a released phylogenetic old reflex?

Medicina2025
#4

Marcus Gunn Syndrome - A Rare Neural Misdirection Phenomenon Case Report.

Annals of maxillofacial surgery2024

This case report describes the Marcus Gunn phenomenon (MGP), an infrequent type of congenital unwanted contractions of facial muscles that occurs in 4%-6% of patients with congenital ptosis. The defining characteristic of MGP is the affected eyelid lifting in a manner similar to the opening of the mouth. It addresses a 45-year-old man who was examined in the dental clinic with a severely decayed tooth. He exhibited evidence of normal vision and synchronous movement of his lips and eyelid. The conditions matched those of the autosomal dominant disorder with incomplete penetration, the MGP. The carious tooth was restored without the necessity for any surgical intervention. The patient was given appropriate dental treatment without any surgical intervention for Marcus Gunn Syndrome. After receiving routine fillings to treat dental caries, the patient stopped complaining of pain. This case report reveals insight into a situation that has been speculated to be an example of neural redirection syndrome, in which impulses from the trigeminal nerve's motor unit are naturally redirected into the levator palpebrae superioris muscles of the upper eyelid and the superior pterygoid muscle.

#5

Mesencephalic trigeminal nucleus neurons with collaterals to both eyelid and masseter muscles shown by fluorescent double-labeling, revealing a potential mechanism for Marcus Gunn Syndrome.

PloS one2023

Poking palpebral conjunctiva evoked upper-eyelid retraction during ophthalmic surgery. Iatrogenic eyelid ptosis occurred if eyelid branch of lachrymal nerve was sectioned. Mesencephalic trigeminal nucleus (Vme) neurons were labeled when tracer injected into lachrymal nerve innervating eyelid Mueller's muscle. Masseter afferent Vme neurons projecting to oculomotor nucleus (III) was observed in toad and rat, which helps amphibians to stare prey when they open mouth widely to prey. We hypothesized single Vme neurons may have peripheral collaterals to both eyelid and masseter muscles. WGA-594 was injected into upper eyelid, and WGA-488 was simultaneously delivered into ipsilateral masseter muscle in the same rat. Then, double labeled Vme neurons were found under both conventional and confocal microscope. Meanwhile, contact of WGA-594 positive eyelid afferent Vme neurons with WGA-488 labeled masseter afferent ones were observed sometimes. Combined with our previous observation of oculomotor projection Vme neurons, we thought WGA-594/488 double labeled Vme cells, at least some of them, are oculomotor projecting ones. Contact between eyelid and masseter afferent Vme neurons are supposed to be electrotonically coupled, based on a line of previous studies. If exogenous or genetic factors make these Vme neurons misinterpret masseter input as eyelid afferent signals, these Vme neurons might feedforward massages to eyelid retractor motoneurons in the III. Besides, oculomotor projecting Vme neurons might be co-fired by adjacent masseter afferent Vme neurons through electrotonic coupling once the masseter muscle is activated. In these cases, Marcus Gunn Syndrome might occur. This finding leads to a new hypothesis for the Syndrome.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC31 artigos no totalmostrando 15

2025

Temperature-related atypical first-bite syndrome: a rare case report.

Frontiers in oral health
2025

Marcus Gunn syndrome: a released phylogenetic old reflex?

Medicina
2025

sc-MULTI-omics approach in nano-rare diseases: understanding the pathophysiological mechanism of Mulvihill-Smith Syndrome.

Functional &amp; integrative genomics
2024

Marcus Gunn Syndrome - A Rare Neural Misdirection Phenomenon Case Report.

Annals of maxillofacial surgery
2023

Mesencephalic trigeminal nucleus neurons with collaterals to both eyelid and masseter muscles shown by fluorescent double-labeling, revealing a potential mechanism for Marcus Gunn Syndrome.

PloS one
2023

Marcus Gunn Syndrome in Primary Care: A Case Report.

Cureus
2021

Oculomotor Paresis with Cyclic Spasms in Chinese Populations: A Review of the Chinese Literature and a Case Report.

Pediatric neurosurgery
2021

Neonatal diagnosis of Marcus Gunn jaw-winking syndrome.

Clinical case reports
2020

A reformed "release hypothesis" for Marcus Gunn Syndrome, based on newer clinic observations and experimental evidences.

Medical hypotheses
2020

Is there a primitive reflex residue underlying Marcus Gunn Syndrome? Rat electrophysiology.

International journal of ophthalmology
2019

Unilateral Levator Aponeurosis Excision for Marcus Gunn Syndrome and Risk Factors of Residual Jaw Winking.

Journal of ophthalmology
2019

Marcus Gunn Jaw-Winking Syndrome: a Case Report.

Medical archives (Sarajevo, Bosnia and Herzegovina)
2019

Marcus Gunn (jaw-winking) phenomenon in pediatric otorhinolaryngology practice.

International journal of pediatric otorhinolaryngology
2018

Is Marcus Gunn jaw winking a primitive reflex? Rat neuroanatomy.

International journal of ophthalmology
2015

Surgical Outcomes in Cases of Marcus-Gunn Jaw-Winking Phenomenon.

Kathmandu University medical journal (KUMJ)
Ver todos os 31 no EuropePMC

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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. Temperature-related atypical first-bite syndrome: a rare case report.
    Frontiers in oral health· 2025· PMID 41458465mais citado
  2. sc-MULTI-omics approach in nano-rare diseases: understanding the pathophysiological mechanism of Mulvihill-Smith Syndrome.
    Functional &amp; integrative genomics· 2025· PMID 40343591mais citado
  3. Marcus Gunn syndrome: a released phylogenetic old reflex?
    Medicina· 2025· PMID 40577165mais citado
  4. Marcus Gunn Syndrome - A Rare Neural Misdirection Phenomenon Case Report.
    Annals of maxillofacial surgery· 2024· PMID 39184408mais citado
  5. Mesencephalic trigeminal nucleus neurons with collaterals to both eyelid and masseter muscles shown by fluorescent double-labeling, revealing a potential mechanism for Marcus Gunn Syndrome.
    PloS one· 2023· PMID 37934736mais citado

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:91412(Orphanet)
  2. OMIM OMIM:154600(OMIM)
  3. MONDO:0007946(MONDO)
  4. GARD:6972(GARD (NIH))
  5. Busca completa no PubMed(PubMed)
  6. Q1476789(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 de Marcus-Gunn
Compêndio · Raras BR

Síndrome de Marcus-Gunn

ORPHA:91412 · MONDO:0007946
Prevalência
Unknown
Herança
Autosomal dominant
CID-10
Q07.8 · Outras malformações congênitas especificadas do sistema nervoso
CID-11
Ensaios
1 ativos
Início
Infancy, Neonatal
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0266521
EuropePMC
Wikidata
Papers 10a
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