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May 1950

La neuro-angiomatose encéphalofaciale: Syndrome hypothalamohypophysaire.

Arch NeurPsych. 1950;63(5):840. doi:10.1001/archneurpsyc.1950.02310230162016

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This small book is a thorough study of a rather rare condition, only about 200 cases having been described. The author is the chief of the ophthalmologic clinic at the faculty of medicine in Algiers, where he had an opportunity during the war to examine some 30,000 recruits. Among them he found 5 cases of encephalofacial neuroangiomatosis, 3 of which are described in detail.

The history of the development of knowledge of the disorder is detailed and the confused and complicated terminology discussed. The author adopts the term used in the title, which seems to be quite appropriate. The observations on 3 of his cases are given in detail, and the interesting fact is noted that they all concerned Europeans. No case was discovered among the native North Africans.

The clinical syndrome is discussed exhaustively, noting the nevus flammeus, the craniofacial hypertrophy, the mental troubles, the hydrophthalmos and the cortical