title: Functional MRI changes in the central motor system in myotonic dystrophy type 1.
abstract: Myotonic dystrophy type 1 (DM1) is a multisystemic disease involving multiple organ systems including central nervous system (CNS) and muscles. Few studies have focused on the central motor system in DM1, pointing to a subclinical abnormality in the CNS. The aim of our study was to investigate patterns of cerebral activation in DM1 during a motor task using functional MRI (fMRI). Fifteen DM1 patients, aged 20 to 59 years, and 15 controls of comparable age were scanned during a self-paced sequential finger-to-thumb opposition task of their dominant right hand. Functional MRI images were analyzed using SPM99. Patients underwent clinical and genetic assessment; all subjects underwent a conventional MR study. Myotonic dystrophy type 1 patients showed greater activation than controls in bilateral sensorimotor areas and inferior parietal lobules, basal ganglia and thalami, in the ipsilateral premotor area, insula and supplementary motor area (corrected P<.05). Analysis of the interaction between disease and age showed that correlation with age was significantly greater in patients than in controls in bilateral sensorimotor areas and in contralateral parietal areas. Other clinical and MR characteristics did not correlate with fMRI. Functional changes in DM1 may represent compensatory mechanisms such as reorganization and redistribution of functional networks to compensate for ultrastructural and neurochemical changes occurring as part of the accelerated aging process.
authors.affiliation: Neuroradiologia, I Facoltà di Medicina e Chirurgia, Università di Roma La Sapienza, Viale dell'Università 30, 00185 Rome, Italy. francesca.caramia@uniroma1.it
authors.forename: Francesca
authors.initials: F
authors.lastname: Caramia
authors.forename: Caterina
authors.initials: C
authors.lastname: Mainero
authors.forename: Francesca
authors.initials: F
authors.lastname: Gragnani
authors.forename: Emanuele
authors.initials: E
authors.lastname: Tinelli
authors.forename: Marco
authors.initials: M
authors.lastname: Fiorelli
authors.forename: Vanessa
authors.initials: V
authors.lastname: Ceschin
authors.forename: Patrizia
authors.initials: P
authors.lastname: Pantano
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Bucci
authors.forename: Veronica
authors.initials: V
authors.lastname: Barra
authors.forename: Luigi
authors.initials: L
authors.lastname: Bozzao
authors.forename: Giovanni
authors.initials: G
authors.lastname: Antonini
mesh_terms: D000328:Adult; D019054:Evoked Potentials, Motor; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D009044:Motor Cortex / Q000503:physiopathology*; D009068:Movement; D009223:Myotonic Dystrophy / Q000503:physiopathology*; D013003:Somatosensory Cortex / Q000503:physiopathology*; D055815:Young Adult