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Van Bogaert-Divry syndrome

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A familial syndrome characterised by angiomatosis of skin and cerebral meninges with progressive demyelinisation of white matter, hemianopsia and cutis marmorata due to telangiectases.

Description

A familial syndrome characterised by angiomatosis of skin and cerebral meninges with progressive demyelinisation of white matter, hemianopsia and cutis marmorata due to telangiectases. Clinically marked by mental development retardation and progressive dementia, pyramidal and extrapyramidal disturbances, visual field defects, migraine. Present from infancy with progressive course. At autopsy diffuse angiomatosis is present in the meninges. Inheritance is autosomal recessive.

Van Bogaert and Divry documented the condition in three affected brothers.

Bibliography

  • L. van Bogaert, P. Divry:
    Sur une maladie familiale caractérisée par une angiomatose diffuse cortico-méningée et une démyélinisation de la substance blanche du centra ovale.
    Bruxelles médical, 1945, 25: 1090-1091.

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