Table 2:

Summary of antemortem and postmortem MR imaging findings in three cases of ALSD

Case No.Age/SexDuration of Illness (yrs/mo)Antemortem T2-Weighted Imaging FindingsPostmortem T2-Weighted Imaging Findings
169/M2/1
  • Atrophy in anteromedial part of bilateral temporal lobes, more marked on right side.

  • Mild atrophy of frontal lobes.

  • Atrophy of cerebral cortices, but no signal changes in cerebral cortices.

  • No signal changes in cerebral white matter.

  • No atrophy or signal changes in corticospinal tracts in cerebrum and brain stem and in substantia nigra (obtained 1 year before death).

  • Atrophy in anteromedial part of bilateral temporal lobes, more marked on right side.

  • Mild atrophy of frontal lobes.

  • Atrophy of cerebral cortices, but no signal changes in cerebral cortices.

  • Hyperintensities in anteromedial white matter of temporal lobes. Linear subcortical hyperintensities in temporal lobes, more marked on right side.

  • No atrophy or signal changes in corticospinal tracts in cerebrum and brain stem and in substantia nigra.

250/M5/4
  • Atrophy in anteromedial part of bilateral temporal lobes, more marked on right side.

  • Mild atrophy of frontal lobes.

  • Atrophy of cerebral cortices, but no signal changes in cerebral cortices.

  • Faint hyperintensities in subcortical white matter in anteromedial part of right temporal lobe.

  • No atrophy or signal changes in corticospinal tracts in cerebrum and brain stem and in substantia nigra (obtained 4 months before death)

.
  • Atrophy in anteromedial part of bilateral temporal lobes, more marked on right side.

  • Mild atrophy of frontal lobes.

  • Atrophy of cerebral cortices, but no signal changes in cerebral cortices.

  • Hyperintensities in subcortical white matter of frontal and temporal lobes.

  • No atrophy or signal changes in corticospinal tracts in cerebrum and brain stem and in substantia nigra.

369/M5Not available
  • Symmetric atrophy of frontal and temporal lobes, more marked in anteromedial part of temporal lobes.

  • Atrophy of cerebral cortices, but no signal changes in cerebral cortices.

  • Hyperintensities in subcortical and deep white matter of frontal and temporal lobes.

  • No atrophy or signal changes in corticospinal tracts in cerebrum and brain stem and in substantia nigra.

  • Note:—ALSD indicates amyotrophic lateral sclerosis with dementia.