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Medullated nerve fibers. A sign of multiple basal cell nevi (Gorlin's) syndrome. 1985

P T De Jong, and B Bistervels, and J Cosgrove, and G de Grip, and A Leys, and M Goffin

Four patients had Gorlin's (or basal cell nevi) syndrome and pronounced medullated nerve fibers. The medullation is an additional ophthalmic manifestation of Gorlin's syndrome, which is autosomally dominant and noted for its variable expressivity.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009413 Nerve Fibers, Myelinated A class of nerve fibers as defined by their structure, specifically the nerve sheath arrangement. The AXONS of the myelinated nerve fibers are completely encased in a MYELIN SHEATH. They are fibers of relatively large and varied diameters. Their NEURAL CONDUCTION rates are faster than those of the unmyelinated nerve fibers (NERVE FIBERS, UNMYELINATED). Myelinated nerve fibers are present in somatic and autonomic nerves. A Fibers,B Fibers,Fiber, Myelinated Nerve,Fibers, Myelinated Nerve,Myelinated Nerve Fiber,Myelinated Nerve Fibers,Nerve Fiber, Myelinated
D012160 Retina The ten-layered nervous tissue membrane of the eye. It is continuous with the OPTIC NERVE and receives images of external objects and transmits visual impulses to the brain. Its outer surface is in contact with the CHOROID and the inner surface with the VITREOUS BODY. The outer-most layer is pigmented, whereas the inner nine layers are transparent. Ora Serrata
D002280 Carcinoma, Basal Cell A malignant skin neoplasm that seldom metastasizes but has potentialities for local invasion and destruction. Clinically it is divided into types: nodular, cicatricial, morphaic, and erythematoid (pagetoid). They develop on hair-bearing skin, most commonly on sun-exposed areas. Approximately 85% are found on the head and neck area and the remaining 15% on the trunk and limbs. (From DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1471) Carcinoma, Basal Cell, Pigmented,Epithelioma, Basal Cell,Rodent Ulcer,Ulcer, Rodent,Basal Cell Carcinoma,Basal Cell Carcinomas,Basal Cell Epithelioma,Basal Cell Epitheliomas,Carcinomas, Basal Cell,Epitheliomas, Basal Cell,Rodent Ulcers,Ulcers, Rodent
D005260 Female Females
D005654 Fundus Oculi The concave interior of the eye, consisting of the retina, the choroid, the sclera, the optic disk, and blood vessels, seen by means of the ophthalmoscope. (Cline et al., Dictionary of Visual Science, 4th ed) Fundus of the Eye,Ocular Fundus,Fundus, Ocular
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D001478 Basal Cell Nevus Syndrome Hereditary disorder consisting of multiple basal cell carcinomas, odontogenic keratocysts, and multiple skeletal defects, e.g., frontal and temporoparietal bossing, bifurcated and splayed ribs, kyphoscoliosis, fusion of vertebrae, and cervicothoracic spina bifida. Genetic transmission is autosomal dominant. Gorlin Syndrome,Nevoid Basal Cell Carcinoma Syndrome,Nevus Syndrome, Basal Cell,Fifth Phacomatosis,Gorlin-Goltz Syndrome,Multiple Basal Cell Nevi, Odontogenic Keratocysts, and Skeletal Anomalies,NBCCS,Fifth Phacomatoses,Gorlin Goltz Syndrome,Syndrome, Gorlin,Syndrome, Gorlin-Goltz

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