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Research paper
Grey matter correlates of clinical variables in amyotrophic lateral sclerosis (ALS): a neuroimaging study of ALS motor phenotype heterogeneity and cortical focality
  1. Peter Bede1,
  2. Arun Bokde2,
  3. Marwa Elamin1,
  4. Susan Byrne1,
  5. Russell L McLaughlin3,
  6. Norah Jordan4,
  7. Harald Hampel5,
  8. Laura Gallagher4,
  9. Catherine Lynch6,
  10. Andrew J Fagan7,
  11. Niall Pender8,
  12. Orla Hardiman1
  1. 1Trinity College Institute of Neuroscience (TCIN), Trinity College Dublin, Dublin, Ireland
  2. 2Discipline of Psychiatry, Trinity College Institute of Neuroscience (TCIN), and Cognitive Systems Group, School of Medicine, Trinity College Dublin, Dublin, Ireland
  3. 3Smurfit Institute of Genetics, Trinity College Dublin, Dublin, Ireland
  4. 4Department of Psychology, Trinity College Dublin, Dublin, Ireland
  5. 5Department of Psychiatry, Psychosomatic Medicine and Psychotherapy, Goethe University, Frankfurt, Germany
  6. 6Department of Neurology, Clinical Research Centre, Beaumont Hospital, Dublin, Ireland
  7. 7Centre for Advanced Medical Imaging (CAMI), St. James's Hospital, Trinity College Dublin, Dublin, Ireland
  8. 8Department of Psychology, Beaumont Hospital, Dublin, Ireland
  1. Correspondence to Dr Peter Bede, Trinity College Institute of Neuroscience (TCIN), Lloyd Building, Trinity College Dublin, Dublin, Ireland, bedepeter{at}hotmail.com

Abstract

Background Body region of onset and functional disability are key components of disease heterogeneity in amyotrophic lateral sclerosis (ALS).

Objectives To evaluate patterns of grey matter pathology in the motor cortex and correlate focal structural changes with functional disability.

Methods We conducted a single-centre neuroimaging study of a cohort of 33 cognitively normal patients with amyotrophic lateral sclerosis (ALS) and 44 healthy controls. A voxel-wise generalised linear model was used to investigate the distribution of disease burden within the motor cortex in relation to clinical disability.

Results Patients with bulbar onset have bilateral focal atrophy in the bulbar segment of the motor homunculus compared with patients with limb onset who have focal cortical changes in the limb segment of their motor strip. Furthermore, the extent to which different body regions are affected in ALS corresponds to the extent of focal grey matter loss in the primary motor cortex. Cortical ALS pathology also extends beyond the motor cortex affecting frontal, occipital and temporal regions.

Conclusions Focal grey matter atrophy within the motor homunculus corresponds with functional disability in ALS. The findings support the existing concepts of cortical focality and motor phenotype heterogeneity in ALS.

  • ALS
  • Motor Neuron Disease
  • MRI
  • Neuroanatomy
  • Neuroradiology

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