ABSTRACT
Insular degeneration has been linked to symptoms of frontotemporal dementia (FTD). Presented in this case is a patient exhibiting semantic variant primary progressive aphasia, behavioral disturbance. Upon autopsy, he was found to have severe insular atrophy. In addition, selective serotonin reuptake inhibitors were ineffective in reducing symptoms of obsessive-compulsive behaviors or emotional blunting. This case suggests that Seeley et al.'s (2007 , Alzheimer Disease & Associated Disorders, 21, S50) hypothesis that von Economo neurons and fork cell-rich brain regions, particularly in the insula, are targeted in additional subtypes of FTD beyond the behavioral variant.
Subject(s)
Aphasia, Primary Progressive/pathology , Cerebral Cortex/pathology , Frontotemporal Dementia/pathology , Aphasia, Primary Progressive/physiopathology , Aphasia, Primary Progressive/psychology , Atrophy , Autopsy , Behavioral Symptoms , Cerebral Cortex/physiopathology , Compulsive Behavior/pathology , Frontotemporal Dementia/physiopathology , Frontotemporal Dementia/psychology , Humans , Male , Middle Aged , Neuropsychological Tests , SemanticsABSTRACT
BACKGROUND/AIMS: Automated, volumetrically defined atrophy in the left anterior cingulate (LAC) and anterior temporal regions (LAT) on MRI can be used to distinguish most patients with frontotemporal dementia (FTD) from controls. FTD and Alzheimer's disease (AD) can differ in the degree of anterior temporal atrophy. We explored whether clinicians can visually detect this atrophy pattern and whether they can use it to classify the 2 groups of dementia patients with the same accuracy. METHODS: Four neurologists rated atrophy in the LAC and LAT regions on MRI slices from 21 FTD, 21 controls, and 14 AD participants. Inter-rater reliability and diagnostic accuracy were assessed. RESULTS: All 4 raters agreed on the presence of clinically significant atrophy, and their atrophy scoring correlated with the volumes, but without translation into high inter-rater diagnostic agreement. CONCLUSIONS: Volumetric analyses are difficult to translate into routine clinical practice.
Subject(s)
Frontotemporal Dementia/diagnosis , Frontotemporal Dementia/pathology , Aged , Aged, 80 and over , Alzheimer Disease/diagnosis , Alzheimer Disease/pathology , Atrophy , Autopsy , Diagnosis, Differential , Female , Gyrus Cinguli/pathology , Humans , Image Processing, Computer-Assisted , Magnetic Resonance Imaging , Male , Middle Aged , Observer Variation , Reproducibility of Results , Socioeconomic Factors , Temporal Lobe/pathologyABSTRACT
OBJECTIVE: Primitive reflexes and parkinsonian signs are used by clinicians to differentiate among dementias. We reviewed our clinical sample to determine whether primitive reflexes were more prevalent in frontally-based dementias and whether parkinsonian signs were more common in dementia with Lewy bodies (DLB) than in other types of dementia. DESIGN: We retrospectively reviewed charts from 204 patients with dementia who presented for consultation at Baycrest's Ross Memory Clinic between April, 2003, to December, 2007. RESULTS: A greater proportion of subjects with DLB and dementia of the Alzheimer type with cardiovascular disease had primitive reflexes than subjects with frontotemporal dementia (FTD). Primitive reflexes were not positively predictive of FTD or vascular dementia (VaD). Dementia with Lewy bodies subjects were more likely to have parkinsonian signs than the other dementias, and bradykinesia and rigidity were positively predictive of FTD. The palmomental reflex was the most common primitive reflex in the sample, and cogwheeling was the most common parkinsonian sign. There was no significant difference between early- and late-stage groups in presence of primitive reflexes or parkinsonian signs. CONCLUSIONS: Primitive reflexes appear not to be clinically discriminative of frontally-based dementias such as FTD and VaD.
