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White matter alterations differ in primary lateral sclerosis and amyotrophic lateral sclerosis.
Brain. 2011 Sep; 134(Pt 9):2642-55.B

Abstract

Primary lateral sclerosis is a sporadic disorder characterized by slowly progressive corticospinal dysfunction. Primary lateral sclerosis differs from amyotrophic lateral sclerosis by its lack of lower motor neuron signs and long survival. Few pathological studies have been carried out on patients with primary lateral sclerosis, and the relationship between primary lateral sclerosis and amyotrophic lateral sclerosis remains uncertain. To detect in vivo structural differences between the two disorders, diffusion tensor imaging of white matter tracts was carried out in 19 patients with primary lateral sclerosis, 18 patients with amyotrophic lateral sclerosis and 19 age-matched controls. Fibre tracking was used to reconstruct the intracranial portion of the corticospinal tract and three regions of the corpus callosum: the genu, splenium and callosal fibres connecting the motor cortices. Both patient groups had reduced fractional anisotropy, a measure associated with axonal organization, and increased mean diffusivity of the reconstructed corticospinal and callosal motor fibres compared with controls, without changes in the genu or splenium. Voxelwise comparison of the whole brain white matter using tract-based spatial statistics confirmed the differences between patients and controls in the diffusion properties of the corticospinal tracts and motor fibres of the callosum. This analysis further revealed differences in the regional distribution of white matter alterations between the patient groups. In patients with amyotrophic lateral sclerosis, the greatest reduction in fractional anisotropy occurred in the distal portions of the intracranial corticospinal tract, consistent with a distal axonal degeneration. In patients with primary lateral sclerosis, the greatest loss of fractional anisotropy and mean diffusivity occurred in the subcortical white matter underlying the motor cortex, with reduced volume, suggesting tissue loss. Clinical measures of upper motor neuron dysfunction correlated with reductions in fractional anisotropy in the corticospinal tract in patients with amyotrophic lateral sclerosis and increased mean diffusivity and volume loss of the corticospinal tract in patients with primary lateral sclerosis. Changes in the diffusion properties of the motor fibres of the corpus callosum were strongly correlated with changes in corticospinal fibres in patients, but not in controls. These findings indicate that degeneration is not selective for corticospinal neurons, but affects callosal neurons within the motor cortex in motor neuron disorders.

Authors+Show Affiliations

MD EMG section, National Institute of Neurological Disease and Stroke, National Institute of Health, 10 Center Drive, Building 10 Room 7-5680, Bethesda, MD 20892-1404, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, N.I.H., Intramural

Language

eng

PubMed ID

21798965

Citation

Iwata, Nobue K., et al. "White Matter Alterations Differ in Primary Lateral Sclerosis and Amyotrophic Lateral Sclerosis." Brain : a Journal of Neurology, vol. 134, no. Pt 9, 2011, pp. 2642-55.
Iwata NK, Kwan JY, Danielian LE, et al. White matter alterations differ in primary lateral sclerosis and amyotrophic lateral sclerosis. Brain. 2011;134(Pt 9):2642-55.
Iwata, N. K., Kwan, J. Y., Danielian, L. E., Butman, J. A., Tovar-Moll, F., Bayat, E., & Floeter, M. K. (2011). White matter alterations differ in primary lateral sclerosis and amyotrophic lateral sclerosis. Brain : a Journal of Neurology, 134(Pt 9), 2642-55. https://doi.org/10.1093/brain/awr178
Iwata NK, et al. White Matter Alterations Differ in Primary Lateral Sclerosis and Amyotrophic Lateral Sclerosis. Brain. 2011;134(Pt 9):2642-55. PubMed PMID: 21798965.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - White matter alterations differ in primary lateral sclerosis and amyotrophic lateral sclerosis. AU - Iwata,Nobue K, AU - Kwan,Justin Y, AU - Danielian,Laura E, AU - Butman,John A, AU - Tovar-Moll,Fernanda, AU - Bayat,Elham, AU - Floeter,Mary Kay, Y1 - 2011/07/28/ PY - 2011/7/30/entrez PY - 2011/7/30/pubmed PY - 2011/11/9/medline SP - 2642 EP - 55 JF - Brain : a journal of neurology JO - Brain VL - 134 IS - Pt 9 N2 - Primary lateral sclerosis is a sporadic disorder characterized by slowly progressive corticospinal dysfunction. Primary lateral sclerosis differs from amyotrophic lateral sclerosis by its lack of lower motor neuron signs and long survival. Few pathological studies have been carried out on patients with primary lateral sclerosis, and the relationship between primary lateral sclerosis and amyotrophic lateral sclerosis remains uncertain. To detect in vivo structural differences between the two disorders, diffusion tensor imaging of white matter tracts was carried out in 19 patients with primary lateral sclerosis, 18 patients with amyotrophic lateral sclerosis and 19 age-matched controls. Fibre tracking was used to reconstruct the intracranial portion of the corticospinal tract and three regions of the corpus callosum: the genu, splenium and callosal fibres connecting the motor cortices. Both patient groups had reduced fractional anisotropy, a measure associated with axonal organization, and increased mean diffusivity of the reconstructed corticospinal and callosal motor fibres compared with controls, without changes in the genu or splenium. Voxelwise comparison of the whole brain white matter using tract-based spatial statistics confirmed the differences between patients and controls in the diffusion properties of the corticospinal tracts and motor fibres of the callosum. This analysis further revealed differences in the regional distribution of white matter alterations between the patient groups. In patients with amyotrophic lateral sclerosis, the greatest reduction in fractional anisotropy occurred in the distal portions of the intracranial corticospinal tract, consistent with a distal axonal degeneration. In patients with primary lateral sclerosis, the greatest loss of fractional anisotropy and mean diffusivity occurred in the subcortical white matter underlying the motor cortex, with reduced volume, suggesting tissue loss. Clinical measures of upper motor neuron dysfunction correlated with reductions in fractional anisotropy in the corticospinal tract in patients with amyotrophic lateral sclerosis and increased mean diffusivity and volume loss of the corticospinal tract in patients with primary lateral sclerosis. Changes in the diffusion properties of the motor fibres of the corpus callosum were strongly correlated with changes in corticospinal fibres in patients, but not in controls. These findings indicate that degeneration is not selective for corticospinal neurons, but affects callosal neurons within the motor cortex in motor neuron disorders. SN - 1460-2156 UR - https://www.unboundmedicine.com/medline/citation/21798965/White_matter_alterations_differ_in_primary_lateral_sclerosis_and_amyotrophic_lateral_sclerosis_ L2 - https://academic.oup.com/brain/article-lookup/doi/10.1093/brain/awr178 DB - PRIME DP - Unbound Medicine ER -