Angiomatosis, diffuse corticomeningealQ85.8

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Divry van Bogaert syndrome

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HistoryThis section has been translated automatically.

van Bogaert and Divry, 1945

DefinitionThis section has been translated automatically.

Corticomeningeal angiomatosis with progressive, severe neurological defects (epilepsy) and cutis marmorata

EtiopathogenesisThis section has been translated automatically.

Autosomal recessive inheritance with family clustering.

ManifestationThis section has been translated automatically.

Congenital, in infancy or early childhood.

Clinical featuresThis section has been translated automatically.

  • Integument: Congenital Cutis marmorata (see below Livedo reticularis) on back, flanks, buttocks and legs. Acrocyanosis on hands, elbows and knees. Spotted hyperpigmentation on the trunk.
  • CNS: non-calcifying corticomeningeal angiomatosis (especially occipital) with epilepsy, progressive dementia, pyramidal and extrapyramidal movement disorders, hemianopsia and retinal angiomas.

LiteratureThis section has been translated automatically.

  1. Bussone G et al (1984) Divry-Van Bogaert syndrome. Clinical and ultrastructural findings. Arch Neurol 41: 560-562
  2. De A Aquino Gondim F et al (2003) Intracerebral hemorrhage associated with Sneddon's syndrome: ischemia-related angiogenesis the cause? Case report and review of the literature. Neuroradiology 45: 368-372
  3. Stone J et al (2001) Divry-Van Bogaert syndrome in a female: relationship to Sneddon's syndrome and radiographic appearances. Neuroradiology 43: 562-564
  4. van Bogaert L, Divry P (1945) Sur une maladie familiale caractérisée par une angiomatose diffuse cortico-méningée et une démyélinisation de la substance blanche du centra ovale. Bruxelles médical 25: 1090-1091

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer