Gomez-López-Hernández syndrome: A case report with clinical and molecular evaluation and literature review.

TitleGomez-López-Hernández syndrome: A case report with clinical and molecular evaluation and literature review.
Publication TypeJournal Article
Year of Publication2020
AuthorsPerrone, E, D'Almeida, V, Sobreira, NLygia de M, de Mello, CBerlim, de Oliveira, AChiaratti, Burlin, S, Soares, Mde Fátima, Cernach, MCecília S, Perez, ABeatriz Al
JournalAm J Med Genet A
Volume182
Issue7
Pagination1761-1766
Date Published2020 07
ISSN1552-4833
KeywordsAbnormalities, Multiple, Alopecia, Cerebellum, Child, Child, Preschool, Craniofacial Abnormalities, Female, Growth Disorders, Humans, Magnetic Resonance Imaging, Misoprostol, Neurocutaneous Syndromes, Phenotype, Rhombencephalon, Trigeminal Nerve
Abstract

Gomez-López-Hernández syndrome (GLHS) is characterized by rhombencephalosynapsis (RES), alopecia, trigeminal anesthesia and a distinctive phenotype, including brachyturricephaly. It has been suggested that GLHS should be considered as part of the spectrum of RES-associated conditions that include alopecia, trigeminal anesthesia, and craniofacial anomalies, rather than a distinct entity. To the best of our knowledge, 57 patients with GLHS have been described. Despite its first description in 1979, the etiology of this syndrome remains unknown. Here, we describe, to our knowledge, the first case of a patient with GLHS who was molecularly evaluated and had been prenatally exposed to misoprostol. We also reviewed the clinical and morphological features of the patients described to date to better delineate the phenotype and focus on any evidence for adverse pregnancy outcomes or exposure, including teratogens.

DOI10.1002/ajmg.a.61594
Alternate JournalAm J Med Genet A
PubMed ID32302043
Grant ListUM1 HG006542 / HG / NHGRI NIH HHS / United States