Biomaterial Database

Neurocutaneous melanosis with hydrocephalus and syringomyelia. Case report. 1985

B J Leaney, and P W Rowe, and G L Klug

The authors report the investigation, treatment, and progress of a unique case of neurocutaneous melanosis with syringomyelia. The suspected etiology, the pathology, and the variable clinical manifestations of this rare phakomatosis are recounted. The potential for malignant change and the possible mechanisms of syrinx development in this syndrome are discussed.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D008548	Melanosis	Disorders of increased melanin pigmentation that develop without preceding inflammatory disease.	Chloasma,Freckles,Melanism,Melasma,Chloasmas,Freckle,Melanoses,Melasmas
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006849	Hydrocephalus	Excessive accumulation of cerebrospinal fluid within the cranium which may be associated with dilation of cerebral ventricles, INTRACRANIAL HYPERTENSION; HEADACHE; lethargy; URINARY INCONTINENCE; and ATAXIA.	Communicating Hydrocephalus,Congenital Hydrocephalus,Obstructive Hydrocephalus,Post-Traumatic Hydrocephalus,Aqueductal Stenosis,Cerebral Ventriculomegaly,Fetal Cerebral Ventriculomegaly,Hydrocephalus Ex-Vacuo,Hydrocephaly,Aqueductal Stenoses,Cerebral Ventriculomegalies,Cerebral Ventriculomegalies, Fetal,Cerebral Ventriculomegaly, Fetal,Fetal Cerebral Ventriculomegalies,Hydrocephalus Ex Vacuo,Hydrocephalus Ex-Vacuos,Hydrocephalus, Communicating,Hydrocephalus, Congenital,Hydrocephalus, Obstructive,Hydrocephalus, Post-Traumatic,Post Traumatic Hydrocephalus,Stenoses, Aqueductal,Stenosis, Aqueductal,Ventriculomegalies, Cerebral,Ventriculomegalies, Fetal Cerebral,Ventriculomegaly, Cerebral,Ventriculomegaly, Fetal Cerebral
D013595	Syringomyelia	Longitudinal cavities in the spinal cord, most often in the cervical region, which may extend for multiple spinal levels. The cavities are lined by dense, gliogenous tissue and may be associated with SPINAL CORD NEOPLASMS; spinal cord traumatic injuries; and vascular malformations. Syringomyelia is marked clinically by pain and PARESTHESIA, muscular atrophy of the hands, and analgesia with thermoanesthesia of the hands and arms, but with the tactile sense preserved (sensory dissociation). Lower extremity spasticity and incontinence may also develop. (From Adams et al., Principles of Neurology, 6th ed, p1269)	Hydrosyringomyelia,Morvan Disease,Morvan's Disease,Myelosyringosis,Syringomyelus,Hydrosyringomyelias,Morvan Diseases,Morvan's Diseases,Morvans Disease,Myelosyringoses,Syringomyelias