{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T09:50:15Z","timestamp":1791366615132,"version":"4.3.1"},"reference-count":16,"publisher":"Georg Thieme Verlag KG","issue":"07","license":[{"start":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T00:00:00Z","timestamp":1786579200000},"content-version":"vor","delay-in-days":43,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arq Neuropsiquiatr"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>In 1869, Charcot and Alix Joffroy published the first detailed clinical and neuropathological description of amyotrophic lateral sclerosis (ALS), establishing the correlation involving muscle weakness, atrophy, spasticity, and degeneration of the lateral corticospinal tracts. Charcot unified the involvement of upper and lower motor neurons into a single clinical entity. His pioneering description was limited to the motor system, reflecting the scientific constraints of his time. Charcot interpreted ALS primarily as a disorder of the motor system, a conclusion consistent with the clinical and pathological methods available in the late nineteenth century. Neurological investigation at that time relied mainly on detailed clinical observation, anatomical correlation at autopsy, and relatively-simple physiological techniques. These approaches were well suited to identify motor dysfunction but were far less capable of revealing subtle cognitive or behavioral alterations. Currently, ALS is recognized as a multisystem neurodegenerative disorder. Thus, Charcot's historical contribution was crucial for the initial understanding of ALS, while modern perspectives acknowledge its broader clinical complexity beyond the motor system.<\/jats:p>","DOI":"10.1055\/s-0046-1827042","type":"journal-article","created":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T23:16:11Z","timestamp":1786662971000},"page":"001-004","source":"Crossref","is-referenced-by-count":0,"title":["Amyotrophic lateral sclerosis (ALS): new insights 156 years after Charcot's masterful description"],"prefix":"10.1055","volume":"84","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0778-5376","authenticated-orcid":false,"given":"Otto Jesus Hernandez","family":"Fustes","sequence":"additional","affiliation":[{"name":"Universidade Federal do Paran\u00e1, Hospital de Cl\u00ednicas, Departamento de Medicina Interna, Curitiba PR, Brazil."},{"name":"Universidade Federal do Paran\u00e1, Hospital de Cl\u00ednicas, Departamento de Medicina Interna, Servi\u00e7o de Neurologia, Curitiba PR, Brazil."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2305-1073","authenticated-orcid":false,"given":"H\u00e9lio Afonso Ghizoni","family":"Teive","sequence":"additional","affiliation":[{"name":"Universidade Federal do Paran\u00e1, Hospital de Cl\u00ednicas, Departamento de Medicina Interna, Curitiba PR, Brazil."},{"name":"Universidade Federal do Paran\u00e1, Hospital de Cl\u00ednicas, Departamento de Medicina Interna, Servi\u00e7o de Neurologia, Curitiba PR, Brazil."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2026,8,13]]},"reference":[{"issue":"03","key":"ref1","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1002\/(SICI)1097-4598(200003)23:3<336::AID-MUS4>3.0.CO;2-L","article-title":"Amyotrophic lateral sclerosis: early contributions of Jean-Martin Charcot","volume":"23","author":"C G Goetz","year":"2000","journal-title":"Muscle Nerve"},{"issue":"07","key":"ref2","doi-asserted-by":"publisher","first-page":"470","DOI":"10.1007\/s00415-025-13198-7","article-title":"Alix Joffroy (1844-1908)","volume":"272","author":"H AG Teive","year":"2025","journal-title":"J Neurol"},{"key":"ref3","first-page":"744","article-title":"Deux cas d'atrophie musculaire progressive avec l\u00e9sion de la substance grise et des faisceaux ant\u00e9rolat\u00e9raux de la moelle \u00e9pini\u00e8re","volume":"2","author":"J M Charcot","year":"1869","journal-title":"Arch Physiol Norm Pathol."},{"key":"ref4","doi-asserted-by":"publisher","first-page":"12","DOI":"10.17879\/freeneuropathology-2021-3323","article-title":"Charcot identifies and illustrates amyotrophic lateral sclerosis","volume":"2","author":"C Duyckaerts","year":"2021","journal-title":"Free Neuropathol"},{"issue":"11","key":"ref5","doi-asserted-by":"publisher","first-page":"1055","DOI":"10.3390\/brainsci14111055","article-title":"Gold Coast Criteria in ALS Diagnosis: A Real-World Experience","volume":"14","author":"L Ferullo","year":"2024","journal-title":"Brain Sci"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"1170996","DOI":"10.3389\/fnins.2023.1170996","article-title":"Recent progress of the genetics of amyotrophic lateral sclerosis and challenges of gene therapy","volume":"17","author":"H Wang","year":"2023","journal-title":"Front Neurosci"},{"issue":"09","key":"ref7","doi-asserted-by":"publisher","first-page":"642","DOI":"10.1038\/s41576-023-00592-y","article-title":"Amyotrophic lateral sclerosis: translating genetic discoveries into therapies","volume":"24","author":"F Ak\u00e7imen","year":"2023","journal-title":"Nat Rev Genet"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1159\/000548249","article-title":"Jean-Martin Charcot and the Clinical Foundations of Amyotrophic Lateral Sclerosis: A Historical Case Revisited","author":"E Drouin","journal-title":"Eur Neurol"},{"issue":"10","key":"ref9","doi-asserted-by":"publisher","first-page":"1918","DOI":"10.1111\/ene.14393","article-title":"Amyotrophic lateral sclerosis: a clinical review","volume":"27","author":"P Masrori","year":"2020","journal-title":"Eur J Neurol"},{"issue":"02","key":"ref10","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1016\/j.neuron.2011.09.011","article-title":"Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS","volume":"72","author":"M DeJesus-Hernandez","year":"2011","journal-title":"Neuron"},{"issue":"02","key":"ref11","doi-asserted-by":"publisher","first-page":"257","DOI":"10.1016\/j.neuron.2011.09.010","article-title":"A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD","volume":"72","author":"A E Renton","year":"2011","journal-title":"Neuron"},{"issue":"05","key":"ref12","doi-asserted-by":"publisher","first-page":"551","DOI":"10.1515\/revneuro-2017-0004","article-title":"Potential skin involvement in ALS: revisiting Charcot's observation - 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