Melkersson-Rosenthal Syndrome and Migraine: A New Phenotype Associated with


Journal

Genes
ISSN: 2073-4425
Titre abrégé: Genes (Basel)
Pays: Switzerland
ID NLM: 101551097

Informations de publication

Date de publication:
20 07 2023
Historique:
received: 28 06 2023
revised: 14 07 2023
accepted: 18 07 2023
medline: 31 7 2023
pubmed: 29 7 2023
entrez: 29 7 2023
Statut: epublish

Résumé

Peripheral facial palsy rarely occurs as part of Melkersson-Rosenthal syndrome (MRS), which is characterized by the classical triad of tongue cheilitis, recurrent episodes of orofacial swelling, and palsy. MRS is a disorder with variable expressivity and clinical as well as genetic heterogeneity; however, the causative gene remains to be identified. Migraine is a common neurological disorder, presenting with or without aura, which may be associated with neurological symptoms. The classical example of monogenic migraine is familial hemiplegic migraine (FHM), which has phenotypic variability in carriers of variants in the same gene or even carriers of the same variant. We present a family in which two sisters displayed recurrent migraines, one of which presented recurrent facial palsy and had clinical diagnosis of MRS. We performed WES and Sanger sequencing for segregation analysis in the available family members. We identified a c.3521C>G missense heterozygous variant in

Identifiants

pubmed: 37510386
pii: genes14071482
doi: 10.3390/genes14071482
pmc: PMC10378782
pii:
doi:

Substances chimiques

SCN1A protein, human 0
NAV1.1 Voltage-Gated Sodium Channel 0

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

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Auteurs

Alessia Azzarà (A)

Research Unit of Medical Genetics, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma, Via Alvaro del Portillo 21, 00128 Rome, Italy.

Ilaria Cassano (I)

Research Unit of Medical Genetics, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma, Via Alvaro del Portillo 21, 00128 Rome, Italy.

Carla Lintas (C)

Research Unit of Medical Genetics, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma, Via Alvaro del Portillo 21, 00128 Rome, Italy.
Operative Research Unit of Medical Genetics, Fondazione Policlinico Universitario Campus Bio-Medico, Via Alvaro del Portillo 200, 00128 Rome, Italy.

Fabio Pilato (F)

Research Unit of Neurology, Neurophysiology, Neurobiology and Psichiatry, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma, Via Alvaro del Portillo 21, 00128 Rome, Italy.
Operative Research Unit of Neurology, Neurophysiology, Neurobiology and Psichiatry, Fondazione Policlinico Universitario Campus Bio-Medico, Via Alvaro del Portillo 200, 00128 Rome, Italy.

Fioravante Capone (F)

Research Unit of Neurology, Neurophysiology, Neurobiology and Psichiatry, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma, Via Alvaro del Portillo 21, 00128 Rome, Italy.
Operative Research Unit of Neurology, Neurophysiology, Neurobiology and Psichiatry, Fondazione Policlinico Universitario Campus Bio-Medico, Via Alvaro del Portillo 200, 00128 Rome, Italy.

Vincenzo Di Lazzaro (V)

Research Unit of Neurology, Neurophysiology, Neurobiology and Psichiatry, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma, Via Alvaro del Portillo 21, 00128 Rome, Italy.
Operative Research Unit of Neurology, Neurophysiology, Neurobiology and Psichiatry, Fondazione Policlinico Universitario Campus Bio-Medico, Via Alvaro del Portillo 200, 00128 Rome, Italy.

Fiorella Gurrieri (F)

Research Unit of Medical Genetics, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma, Via Alvaro del Portillo 21, 00128 Rome, Italy.
Operative Research Unit of Medical Genetics, Fondazione Policlinico Universitario Campus Bio-Medico, Via Alvaro del Portillo 200, 00128 Rome, Italy.

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Classifications MeSH