GLHS also known as cerebello-trigeminal dermal dysplasia, is a rare neurocutaneous condition of variable phenotype which was first described by two Hispanic renowned pediatric neurologists: Drs. Manuel Gomez and Arturo Lopez-Hernandez. GLHS is characterized by rhombencephalosynapsis, alopecia, and trigeminal anesthesia.
Dr. Gomez of Spanish-Cuban origin is known for his long tenure as former head of Pediatric Neurology, Mayo Clinic, founder of the Child Neurology Society and his strong interest in the phakomatoses. His index GLHS case was of a 4.5-year-old girl with absent corneal reflexes, alopecia, delayed motor function, and bilateral facial analgesia [1979]. Gomez postulated that arrest of the neuroectoderm development resulted in the abnormal trigeminal innervation.
Dr. Lopez-Hernandez is one of the fathers of Pediatric Neurology in Mexico having established the first pediatric neurology service at the National Institute of Pediatrics and later serving as its first head of the Neurology Department. In 1982 Lopez-Hernandez reported two unrelated girls with craniosynostosis, ataxia, trigeminal anesthesia and parietal alopecia associated with pons-vermis fusion.
The GLHS eponym was first used in the literature in 1997. To date there are nearly 60 cases reported. The phenotype has been expanded to include short stature, craniofacial anomalies, growth hormone deficiency, hydrocephalus, and neuropsychiatric symptoms. No genetic cause has been identified.
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The specialty of Pediatric Neurology emerged during the 20th century. During this period, many neurologists played significant roles in revolutionizing this field. The Gomez-Lopez-Hernandez syndrome serves to highlight the contributions of two notable giants of pediatric neurology of Hispanic origin, at a time when there was limited representation of minorities in the medical profession.