ABSTRACT
Holmes' priority in establishing an extensive body of knowledge that defines much of neuro-ophthalmology is given. His original descriptions are reviewed and his findings reappraised in light of present-day knowledge.
Subject(s)
Neurology/history , Ophthalmology/history , England , Eye Movements , History, 20th Century , Humans , Iris Diseases/history , Papilledema/history , Reflex, Abnormal/history , Reflex, Pupillary , Syndrome , Visual Cortex/anatomy & histologyABSTRACT
This survey of Roussy-Lévy disease begins with an historical account of the three neurological conditions from which this entity has been separated: Friedreich disease, described in 1861-1863, which proved with time to be a genuine anatomoclinical disorder: Charcot-Marie-Tooth atrophy, described in 1886, particular because of its morphology and evolution but due to various processes: Dejerine-Sottas hypertrophic neuritis, described in 1893, which was the first variant to be individualized within the heterogenous group of primary and familial hypertrophic neuritis. The initial description of Roussy-Lévy disease--in 1926, 1932, and 1934--and the controversies raised by this concept are recalled as well as the present state of the original family: five out of seven members have been examined since 1956 and it has been demonstrated that they are suffering from a form of hypertrophic neuritis. However it is this author's opinion that the concept of an autonomous Roussy-Lévy disease within hypertrophic neuritis is justified by the following criteria: dominant transmission, very precocious onset, extreme slowness of the evolution, remarkable benignity of the prognosis.