Magnetic Resonance Imaging and Histopathology in Dementia, Clinically of Frontotemporal Type

Abstract

The magnetic resonance imaging (MRI) and computed tomography findings in 28 patients with the clinical diagnosis of frontotemporal dementia (FTD) were compared with the findings in a control group of 76 individuals without dementia or stroke. A pattern of frontal and temporal atrophy with predominantly frontal white matter changes was found in the FTD patients, and this was significantly different from the radiological findings in the control group. Six of the FTD patients have undergone autopsy. Histopathological evaluation showed a primary cortical degenerative disease (frontal lobe degeneration of non-Alzheimer type) in 3 of them, and primary white matter disorder, mainly frontal, of basically ischemic type (selective incomplete white matter infarction) in 3 of them. MRI could be a helpful tool to support the clinical diagnosis FTD, especially in young patients. MRI may also be helpful for the differentiation of a primary neurodegenerative from a mainly ischemic-vascular type of dementia.

MRI with Diffusion Tensor Imaging Post-Mortem at 3.0 T in a Patient with Frontotemporal Dementia

“…Diffuse increase in white matter signal intensity in the frontal and/or temporal lobes on MRI has been reported in patients with the clinical diagnosis of FTD [2]. In a previous study of 28 FTD patients compared with 76 normal controls, we found an MRI pattern with frontotemporal atrophy and predominantly frontal white matter changes in FTD, correlating with the histopathological findings at neuropathological examination, which was performed in 6 of these FTD patients [4].…”

“…The histopathological white matter pathology has been shown to appear in at least two different forms, each of which associating differently with the microscopical grey matter changes [3,4]. The interpretation of white matter magnetic resonance imaging (MRI) changes may thus be difficult, and this may partly be due to additional pathologic alterations appearing in the interval between MRI and post-mortem histopathology.…”

The heterogeneity of frontotemporal dementia with regard to initial symptoms, qEEG and neuropathology

“…Besides pathology of the basal ganglia, white matter pathology is frequent, albeit underrecognized in FTD (Englund and Brun, 1987;Larsson et al, 2000;Larsson et al, 2004) and the present findings may indicate that white matter changes here influenced the early reported clinical symptoms. EEG has not been widely used in FTD, although a normal or near normal visual EEG has been considered as a criterion of a degenerative disorder of nonAlzheimer type (The Lund and Manchester Groups, 1994).…”

The Tau R406W Mutation Causes Progressive Presenile Dementia with Bitemporal Atrophy

“…The visuospatial ability and praxis were preserved after 13 years of illness. CT and MRI showed bilateral temporal lobe atrophy and white matter changes in the frontal lobes [4,5]. Neuropathological data from the pedigree are unfortunately not available to date.…”