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Bonnet-Dechaume-Blanc disease (Paul Bonnet)
Also known as:
Wyburn-Mason's syndrome
Bonnet's syndrome I (Paul Bonnet)
Dechaume et Blanc, syndrome de (French)
Synonyms:
Cerebral-retinal arteriovenous aneurysm syndrome, neuroretinoangiomatosis, neuroretinal angiomatosis, racemose angioma.
Associated persons:
Emile Blanc
Paul Bonnet
Jean Dechaume
Roger Wyburn-Mason
Description:
A syndrome characterized by an arteriovenous aneurysm of one or both sides of the midbrain, with retinal angiomatosis, and associated with arteriovenous angiomas of the optic nerve, the thalamus, and the mesencephalon; and the presence of multiple cutaneous facial nevi.
The onset of symptoms is usually before the age of 30.
Symptoms include loss of vision, severe headache, vomiting and sudden proptosis. In some patients, mental retardation, psychotic symptoms. The syndrome is very rare. More frequent in males; present from birth. Aetiology unknown. Autosomal dominant inheritance.
Bibliography:
- P. Bonnet, J. Dechaume, E. Blanc:
L'anévrysme cirsoïde de la rétine (anévrysme racémeux). Ses relations avec l'anévrysme cirsoïde de la face et avec l`anévrysme cirsoïde du cerveau.
Le Journal de médecine de Lyon, 1937, 18: 165-178.
- R. Wyburn-Mason:
Arteriovenous aneurysm of mid-brain and retina, facial nævi and mental changes.
Brain, Oxford, 1943, 66: 163-203.
Biographical:
- L. Paufiquel:
Paul Bonnet (1884-1959). Article in French.
Le Journal de médecine de Lyon, January 5, 1960, 41: 1-3.
Archives d'ophtalmologie et Revue générale d'ophthalmologie, Paris, January/February 1960, 20: 77-80.
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