Biomaterial Database

Neurocutaneous melanosis associated with Dandy-Walker syndrome. 1987

H S Narayanan, and D H Gandhi, and S R Girimaji

Department of Psychiatry, National Institute of Mental Health and Neurosciences, Bangalore, India.

A young boy presented with mental retardation and seizures with extensive hairy naevi. After a fluctuating clinical course over the next 2 years, he worsened rapidly with signs of rising intracranial pressure. A CT scan revealed unsuspected features of Dandy-Walker syndrome. The clinical course is traced till the patient's death, and the implications of the coexistence of these rare conditions are discussed in view of the proposed hypotheses regarding the pathogenesis of Dandy-Walker syndrome.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D008297	Male		Males
D008548	Melanosis	Disorders of increased melanin pigmentation that develop without preceding inflammatory disease.	Chloasma,Freckles,Melanism,Melasma,Chloasmas,Freckle,Melanoses,Melasmas
D008607	Intellectual Disability	Subnormal intellectual functioning which originates during the developmental period. This has multiple potential etiologies, including genetic defects and perinatal insults. Intelligence quotient (IQ) scores are commonly used to determine whether an individual has an intellectual disability. IQ scores between 70 and 79 are in the borderline range. Scores below 67 are in the disabled range. (from Joynt, Clinical Neurology, 1992, Ch55, p28)	Disability, Intellectual,Idiocy,Mental Retardation,Retardation, Mental,Deficiency, Mental,Intellectual Development Disorder,Mental Deficiency,Mental Retardation, Psychosocial,Deficiencies, Mental,Development Disorder, Intellectual,Development Disorders, Intellectual,Disabilities, Intellectual,Disorder, Intellectual Development,Disorders, Intellectual Development,Intellectual Development Disorders,Intellectual Disabilities,Mental Deficiencies,Mental Retardations, Psychosocial,Psychosocial Mental Retardation,Psychosocial Mental Retardations,Retardation, Psychosocial Mental,Retardations, Psychosocial Mental
D009508	Nevus, Pigmented	A nevus containing melanin. The term is usually restricted to nevocytic nevi (round or oval collections of melanin-containing nevus cells occurring at the dermoepidermal junction of the skin or in the dermis proper) or moles, but may be applied to other pigmented nevi.	Nevus, Melanocytic,Nevi, Melanocytic,Nevi, Pigmented,Pigmented Moles,Melanocytic Nevi,Melanocytic Nevus,Pigmented Nevi,Pigmented Nevus
D003616	Dandy-Walker Syndrome	A congenital abnormality of the central nervous system marked by failure of the midline structures of the cerebellum to develop, dilation of the fourth ventricle, and upward displacement of the transverse sinuses, tentorium, and torcula. Clinical features include occipital bossing, progressive head enlargement, bulging of anterior fontanelle, papilledema, ataxia, gait disturbances, nystagmus, and intellectual compromise. (From Menkes, Textbook of Child Neurology, 5th ed, pp294-5)	Dandy-Walker Malformation,Dandy-Walker Complex,Dandy-Walker Cyst,Dandy-Walker Deformity,Dandy-Walker Syndrome, Familial,Hydrocephalus, Internal, Dandy-Walker Type,Hydrocephalus, Noncommunicating, Dandy-Walker Type,Luschka-Magendie Foramina Atresia,Cyst, Dandy-Walker,Cysts, Dandy-Walker,Dandy Walker Complex,Dandy Walker Deformity,Dandy Walker Malformation,Dandy Walker Syndrome,Dandy Walker Syndrome, Familial,Dandy-Walker Complices,Dandy-Walker Cysts,Dandy-Walker Deformities,Familial Dandy-Walker Syndrome,Luschka Magendie Foramina Atresia,Malformation, Dandy-Walker
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
D012878	Skin Neoplasms	Tumors or cancer of the SKIN.	Cancer of Skin,Skin Cancer,Cancer of the Skin,Neoplasms, Skin,Cancer, Skin,Cancers, Skin,Neoplasm, Skin,Skin Cancers,Skin Neoplasm
D013036	Spasms, Infantile	An epileptic syndrome characterized by the triad of infantile spasms, hypsarrhythmia, and arrest of psychomotor development at seizure onset. The majority present between 3-12 months of age, with spasms consisting of combinations of brief flexor or extensor movements of the head, trunk, and limbs. The condition is divided into two forms: cryptogenic (idiopathic) and symptomatic (secondary to a known disease process such as intrauterine infections; nervous system abnormalities; BRAIN DISEASES, METABOLIC, INBORN; prematurity; perinatal asphyxia; TUBEROUS SCLEROSIS; etc.). (From Menkes, Textbook of Child Neurology, 5th ed, pp744-8)	Cryptogenic Infantile Spasms,Hypsarrhythmia,Infantile Spasms,Jackknife Seizures,Nodding Spasm,Salaam Seizures,Spasmus Nutans,Symptomatic Infantile Spasms,West Syndrome,Cryptogenic West Syndrome,Lightning Attacks,Salaam Attacks,Symptomatic West Syndrome,Attack, Lightning,Attacks, Lightning,Attacks, Salaam,Cryptogenic Infantile Spasm,Hypsarrhythmias,Infantile Spasm,Infantile Spasm, Cryptogenic,Infantile Spasm, Symptomatic,Infantile Spasms, Cryptogenic,Infantile Spasms, Symptomatic,Jackknife Seizure,Lightning Attack,Nodding Spasms,Seizure, Jackknife,Seizures, Jackknife,Seizures, Salaam,Spasm, Cryptogenic Infantile,Spasm, Nodding,Spasm, Symptomatic Infantile,Spasms, Cryptogenic Infantile,Spasms, Nodding,Spasms, Symptomatic Infantile,Symptomatic Infantile Spasm,Syndrome, Cryptogenic West,Syndrome, Symptomatic West,Syndrome, West,West Syndrome, Cryptogenic,West Syndrome, Symptomatic