A historical approach to hereditary spastic paraplegia - 15/04/20
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Abstract |
Hereditary spastic paraplegia (HSP) is a group of rare neurological disorders, characterised by their extreme heterogeneity in both their clinical manifestations and genetic origins. Although Charles-Prosper Ollivier d’Angers (1796–1845) sketched out a suggestive description in 1827, it was Heinrich Erb (1840–1921) who described the clinical picture, in 1875, for “spastic spinal paralysis”. Jean-Martin Charcot (1825–1893) began teaching the disorder as a clinical entity this same year. Adolf von Strümpell (1853–1925) recognised its hereditary nature in 1880 and Maurice Lorrain (1867–1956) gained posthumous fame for adding his name to that of Strümpell and forming the eponym after his 1898 thesis, the first review covering twenty-nine affected families. He benefited from the knowledge accumulated over a dozen years on this pathology by his teacher, Fulgence Raymond (1844–1910). Here I present a history across two centuries, leading to the clinical, anatomopathological, and genetic description of hereditary spastic paraplegia which today enables a better understanding of the causative cellular dysfunctions and makes it possible to envisage effective treatment.
Le texte complet de cet article est disponible en PDF.Keywords : Hereditary spastic paraplegia, Weakness, Motor neuron disease, Neurodegeneration, Strümpell-Lorrain syndrome, History of neurology
Plan
Vol 176 - N° 4
P. 225-234 - mai 2020 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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