Progressive Spinal Cord Degeneration in Friedreich's Ataxia: Results from ENIGMA-Ataxia.
Thiago Junqueira Ribeiro de RezendeIsaac M AdanyeguhFilippo ArrigoniBenjamin BenderFernando CendesLouise A CorbenAndreas DeistungMartin DelatyckiImis DoganGary F EganSophia L GörickeNellie Georgiou-KaristianisPierre-Gilles HenryDiane HutterNeda JahanshadJames M JoersChristophe LengletTobias LindigAlberto R M MartinezAndrea MartinuzziGabriella PaparellaDenis PeruzzoKathrin ReetzSandro RomanzettiLudger SchölsJörg B SchulzMatthis SynofzikSophia I ThomopoulosPaul M ThompsonDagmar TimmannIan H HardingMarcondes Cavalcante de França JuniorPublished in: Movement disorders : official journal of the Movement Disorder Society (2022)
Previous research has shown that increased eccentricity reflects dorsal column (DC) damage, while decreased CSA reflects either DC or corticospinal tract (CST) damage, or both. Hence our data support the hypothesis that damage to the DC and damage to CST follow distinct courses in FRDA: developmental abnormalities likely define the DC, while CST alterations may be both developmental and degenerative. These results provide new insights about FRDA pathogenesis and indicate that CSA of the cervical spinal cord should be investigated further as a potential biomarker of disease progression. © 2022 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.