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Updated: Dec 31, 2025

Analyzing Mitochondrial Transport and Morphology in Human Induced Pluripotent Stem Cell-Derived Neurons in Hereditary Spastic Paraplegia
Published on: February 9, 2020
A historical approach to hereditary spastic paraplegia
1Private practice, 20, rue de Chartres, 28160 Brou, France.
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.
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