TY - JOUR T1 - Structural MRI correlates of amyotrophic lateral sclerosis progression JF - Journal of Neurology, Neurosurgery & Psychiatry JO - J Neurol Neurosurg Psychiatry DO - 10.1136/jnnp-2016-314337 SP - jnnp-2016-314337 AU - Joe Senda AU - Naoki Atsuta AU - Hirohisa Watanabe AU - Epifanio Bagarinao AU - Kazunori Imai AU - Daichi Yokoi AU - Yuichi Riku AU - Michihito Masuda AU - Ryoichi Nakamura AU - Hazuki Watanabe AU - Mizuki Ito AU - Masahisa Katsuno AU - Shinji Naganawa AU - Gen Sobue Y1 - 2017/05/13 UR - http://jnnp.bmj.com/content/early/2017/05/13/jnnp-2016-314337.abstract N2 - Purpose Amyotrophic lateral sclerosis (ALS) presents with varying degrees of brain degeneration that can extend beyond the corticospinal tract (CST). Furthermore, the clinical course and progression of ALS varies widely. Brain degeneration detected using structural MRI could reflect disease progression.Subjects and methods On study registration, 3-Tesla volumetric MRI and diffusion tensor imaging scans were obtained at baseline in 38 healthy controls and 67 patients with sporadic ALS. Patients had Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) scores of ≥36 and did not have the chromosome 9, open reading frame 72 repeat expansion. Six months later, changes in ALSFRS-R (ΔALSFRS-R) scores were calculated and patients were grouped into three categories, namely, patients with slow progression with ΔALSFRS-R scores ≤3 (n=19), intermediate progression with ΔALSFRS-R scores =4, 5 and 6 (n=36) and rapid progression with ΔALSFRS-R scores ≥7 (n=12). We analysed voxel-based morphometry and tract-based spatial statistics among these subgroups and controls.Results In comparison with controls, patients with ALS showed grey matter atrophy and decreased fractional anisotropy beyond the motor cortex and CST, especially in the frontotemporal lobes and basal ganglia. Moreover, the degree of change was highly proportional to ΔALSFRS-R at the 6-month assessment.Conclusion A more rapid disease progression and poorer functional decline were associated with greater involvement of the extra-motor cortex and basal ganglia, suggesting that the spatial extent of brain involvement can be an indicator of the progression in ALS. ER -