Frontotemporal Dementia

Last updated on Monday, September 8 2008 by jdmiles

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Frontotemporal lobar degeneration, ubiquitin immunoreactivity within dentate gyrus of hippocampus.
The frontotemporal lobar dementias lacking tau pathology are composed of several disease categories. The largest comprises frontotemporal lobar degenerations demonstrating ubiquitinated cytoplasmic inclusions (FTLD-U). Most, but not all, FTLD-U have been...

Frontotemporal lobar degeneration, coronal section through temporal lobe.
The frontotemporal lobar dementias occasionally demonstrate predominance of temporal lobe atrophy, resembling that seen in Alzheimer disease, but usually quantitatively more striking, as can be seen in this medial temporal lobe.

Frontotemporal lobar degeneration, lateral surface of brain
As the name implies, most cases of frontotemporal lobar degeneration demonstrate predominant atrophy and the frontal and temporal lobes, seen here as widened sulci.

Frontotemporal lobar degeneration, coronal section of medial temporal lobe, close-up
Compared with the inferior temporal gyrus at the right side of the photograph, the hippocampus and parahippocampal gyrus demonstrate strikingly severe atrophy. Both the severity and the abrupt transition to relatively spared neocortex would be very unusual in Alzheimer's disease.

Hereditary dysphasic dementia, coronal section of brain.
Frontotemporal lobar degenerations often demonstrate prominent abnormalities in language function at presentation, related to predominance of pathologic changes surrounding the Sylvian fissure. As demonstrated in this photograph, lobar degeneration is often bilaterally asymmetric.

Pick Disease, ventral surface of brain.
Pick's disease demonstrates the prototypical severe (knife edge) bilateral frontotemporal atrophy, giving the anterior portion of the brain the appearance of a walnut.

Posterior Lobar atrophy, lateral surface of brain
Although unusual, posterior lobar atrophies may be produced by the same spectrum of diseased processes generally associated with frontotemporal lobar degeneration. The abrupt transition between normal and involved brain recapitulates that seen with the more typical anteriorly situated lobar atrophies.