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skos:definition |
MSH: 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),NCI: A rare congenital brain malformation disorder characterized by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle, and enlargement of the posterior fossa.,JABL: Hydrocephalus, absence of the cerebellar vermis, and posterior fossa cyst continuous with the fourth ventricle are the main characteristics of this syndrome. About 4% of all cases of hydrocephalus are complicated by DWS. Associated disorders may include Aase-Smith syndrome 1, Aicardi, Coffin-Siris, cryptophthalmos, Ehlers-Danl
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