| Literature DB >> 31911003 |
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.Entities:
Keywords: Hereditary spastic paraplegia; History of neurology; Motor neuron disease; Neurodegeneration; Strümpell-Lorrain syndrome; Weakness
Mesh:
Year: 2020 PMID: 31911003 DOI: 10.1016/j.neurol.2019.11.003
Source DB: PubMed Journal: Rev Neurol (Paris) ISSN: 0035-3787 Impact factor: 2.607