Background: Predominant and progressive complex visual disorders are often due to posterior cortical atrophy (PCA), a rare early-onset dementing syndrome presenting with visual complaints. In clinicopathological studies, PCA is most commonly considered a form of Alzheimer’s disease (AD); no prior study has evaluated clinical differences between PCA and AD. Methods: This study identified 15 patients who presented with progressive complex visual disorders and predominant occipitoparietal hypoperfusion on SPECT. These patients were retrospectively compared on clinical variables with 30 patients with clinically probable AD matched for gender, age and duration of illness. Results: The PCA patients presented with alexia, elements of Balint’s syndrome, apperceptive visual agnosia, dressing apraxia and environmental disorientation along with elements of Gerstmann’s syndrome. Compared to the AD patients, the 15 PCA patients (mean age of onset 58 years, range 51–64) had significantly better verbal fluency, less memory difficulty, more depression and greater insight into their illness but similar familial and apolipoprotein E risk factors. In the PCA patients, MRI often showed occipitoparietal atrophy without detectable mesiotemporal atrophy. Conclusions: PCA is a distinct clinical syndrome and not just AD with prominent visual deficits. Compared to AD controls, PCA patients have better language and memory but more insight and depression and more posterior atrophy on MRI. These results indicate clinical criteria for the diagnosis of PCA and recommend specific interventions such as visual aids and antidepressant medications. Similar risk factors and course suggest that PCA is most commonly an early-onset posteriorly shifted AD variant.

Frontotemporal dementia (FTD) is a common neurodegenerative dementia that can be difficult to distinguish clinically from Alzheimer's disease (AD). The differential distribution of pathology in FTD and AD predicts the presence of differential cognitive features on mental status examination. We compared 39 FTD patients with 101 AD patients on the Consortium to Establish a Registry in AD examination supplemented by cognitive areas from the Neurobehavioral Cognitive Status Examination. The FTD patients were diagnosed using noncognitive clinical and neuroimaging criteria and were comparable to the AD patients in terms of gender, educational level, and dementia severity ratings. The FTD patients performed significantly better than the AD patients on constructions and calculations. These findings were at the lower limits of normal for older normal controls and persisted after covarying for younger age and higher Mini-Mental Status Examination scores in the FTD group. In addition to personality and neuroimaging features, relatively preserved performance of elementary drawings and calculations in FTD suggests additional features for distinguishing FTD patients from comparably demented AD patients.

No abstract

Frontotemporal dementia (FTD) is a common neurodegenerative dementia syndrome. Compulsive behaviors frequently occur in FTD and may be presenting symptoms of this disorder. This study evaluated compulsive behaviors as presenting symptoms in 29 patients with FTD compared to 48 patients with Alzheimer's disease (AD) enrolled in the UCLA Alzheimer's Disease Center. The FTD patients met the Lund and Manchester criteria for FTD and had predominant frontal hypoperfusion on single-photon emission computer tomography neuroimaging. The AD patients met National Institute of Neurological and Communicative Disorders-Alzheimer's Disease and Related Disorders criteria for clinically probable AD. Compulsive behaviors occurred in 11 FTD patients (38%) versus 5 AD patients (10%) (chi2 = 6.73, P < .01). This difference persisted after controlling for the younger age of the FTD group. There was a range of compulsive behaviors, with the most frequent being repetitive checking activities. Compulsive behaviors are common presenting symptoms among FTD patients and may result from an inability to inhibit urges to perform compulsive movements from damage to frontal-striatal circuits.

Based on this study, type and degree of cognitive impairment are better predictors of driving skills than age or medical diagnosis per se. Specific testing protocols for drivers with potential cognitive impairment may detect unsafe drivers more effectively than using age or medical diagnosis alone as criteria for license restriction or revocation.

Frontotemporal dementia (FTD) is a dementing syndrome characterized by the occurrence of neuropsychiatric features early in the clinical course. Patients with Alzheimer's disease (AD) also have neuropsychiatric symptoms, but these symptoms typically emerge later in the course after the development of memory and other cognitive impairment. The BEHAVE-AD, an instrument developed to evaluate neuropsychiatric features in dementia, may help characterize the behavioral features of FTD and differentiate FTD from AD. This study evaluated BEHAVEAD results of 29 patients with the diagnosis of FTD compared to those of 29 patients with the diagnosis of probable AD of similar dementia severity. The FTD patients had significantly worse global BEHAVE-AD scores compared to the AD patients. Verbal outbursts and inappropriate activity characterized the FTD patients, and three BEHAVE-AD subscales correctly classified 69% of the patients. The assessment of neuropsychiatric symptoms with a standardized scale or inventory can help distinguish dementia patients with FTD and AD.

No abstract

The sense of empathy may be altered by brain disease. We report the drawing performance of four artists who developed frontotemporal dementia (FTD). The first three, but not the fourth, had a prominent decrease in empathy for others as well as alterations in their caricatures of people. Their drawings of faces became distorted, menacing, skeleton-like, or 'alien'. None of the four had facial recognition difficulties, problems in interpreting facial emotions, or a decreased appreciation of the distinction between animate and inanimate objects. Functional brain imaging in the patients revealed bilateral frontal hypometabolism or hypoperfusion, and the three with altered drawings had additional prominent involvement of the right temporal lobe. These FTD patients and the literature suggest that FTD, possibly with greater right temporal involvement, disrupts the sense of empathy from human faces.