The Clinical Pathological Correlates of Lobar Atrophy

Neuropsychological differences between 14 patients with a clinical diagnosis of frontal lobe degeneration of non-Alzheimer type (FLD) and 15 patients with a clinical diagnosis of Alzheimer’s disease (AD) were studied. The most efficient psychometric predictors of FLD were Digit Symbol, estimation tasks, word list recall, and particularly the FAS word fluency test. Behavioral predictors of FLD and AD showed a double dissociation: regression and impulsivity characterized FLD, whereas lack of motivation and slowness were typical of AD. Cognitive and behavioral differences between groups were not related to the degree of dementia, as measured by Mini Mental State Examination, indicating that differences remained during progression of the diseases.

Section: Discussionsupporting

confidence: 90%

Cognitive and Behavioral Differentiation of Frontal Lobe Degeneration of the Non-Alzheimer Type and Alzheimer’s Disease

Lindau

Almkvist

Johansson

et al. 1998

“…The clinical diagnosis may be strongly supported by brain imaging such as regional cerebral blood flow measurement with the xenon inhalation technique [33,34], single-photon emission computerized tomography [35,36] and positron emission tomography. Most often, however, these patients are investigated with CT and MRI [37][38][39] because of the severe deterioration of personality and behavior.…”

Section: Discussionmentioning

confidence: 99%

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

Larsson

Passant²,

Sundgren³

et al. 2000

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.

“…Recently the term frontotemporal dementia (FTD) was advanced to cover a divergent spectrum including FLD, Pick's disease (PD) and motor neuron disease (MND) with dementia (MNDD) in addition to corticobasal degeneration (CBD) and slowly progressive aphasia [7,8]. Few pathological hallmarks including senile plaques (SPs), neurofibrillary tangles (NFTs), Pick bodies (PBs) and achromatic cells (ACs) are yet known for FLD, one form of FTD.…”

Section: Phf-tau In Frontotemporal Dementiamentioning

confidence: 99%

Selective Expression of Ser 199/202 Phosphorylated Tau in a Case of Frontotemporal Dementia

Takamatsu

Kondo

Ikegami

et al. 1998

We examined a 65-year-old patient with clinicopathological features that met the criteria of frontotemporal dementia (FTD), particularly frontal lobe degeneration (FLD). He came from a family with concentrated occurrence of dementia symptoms in the presenium. Neuropathological examination disclosed brain atrophy locally pronounced on the frontotemporal lobes with characteristic neuronal loss, microvacuolation and astrocytic gliosis. There were no pathological hallmarks such as senile plaques, Pick bodies (PBs), achromatic cells and neurofibrillary tangles. Precise separation of FTD from Pick disease (PD) and motor neuron disease with dementia (MNDD) has not yet been established, and they are included in one spectrum. Antibodies against paired helical filament tau protein demonstrated immunopositive cytoskeletal structures within the neurons as well as the glial cells in the brain of the present case. They were selectively stained with tau 199/202 but not tau 396, which were provided newly to recognize phosphorylation at Ser 199/202 or Ser 396 in tau, respectively. We investigated tau pathology in the present case in comparison to 8 cases with PD that were clinicopathologically confirmed. Neither tau 199/202 nor tau 396 stained the CNS structures in PD cases with few PBs, while both stained evidently those as well as PBs in PD cases associated with many PBs; so that the present case could be distinguished from PD on the basis of the immunoreactivity to site-specific phosphorylated tau. Our result suggests that FTD, especially familial FLD type might involve unique tau pathology, no matter whether FLD is a distinct entity from PD, or a variant form in the wide FTD spectrum including PD and MNDD and other related disorders.