Ronald E. Saul

Psychotic Symptoms in Frontotemporal Dementia: Prevalence and Review

Background/Aims: Although most patients with frontotemporal dementia (FTD) present with neuropsychiatric symptoms, the frequency of psychotic symptoms is unclear. This study aims to determine the prevalence of psychotic symptoms in a large cohort of well-diagnosed and followed FTD patients compared to age-matched patients with Alzheimer’s disease (AD) and to further review the literature on psychosis in FTD. Methods: Delusions, hallucinations and paranoia were evaluated among 86 patients who met consensus criteria for FTD, had frontotemporal changes on functional neuroimaging and were followed for 2 years. They were compared to 23 patients with early-onset AD on a caregiver-administered psychiatric questionnaire. Results: Among the FTD patients, only 2 (2.3%) had delusions, 1 of whom had paranoid ideation; no FTD patient had hallucinations. This was significantly less than the AD patients, 4 (17.4%) of whom had delusions and paranoia. Other investigations fail to establish a significant association of psychosis with FTD. Conclusions: These findings, and a literature review, indicate that psychotic symptoms are rare in FTD, possibly due to limited temporal-limbic involvement in this disorder.

Cognitive Differences between Early- and Late-Onset Alzheimer's Disease:

Although neuropathologic studies showed that early-onset Alzheimers disease (EAD) and “senile dementia” were indistinguishable, clinical studies suggested that EAD and late-onset Alzheimers disease (LAD) were cognitively distinct. We sought to investigate whether EAD and LAD are cognitively different by comparing patients at the extremes of the ages of onset in order to maximize features that might separate them. We compared 44 men with EAD (age of onset less than 65 years) with 44 men with LAD (age of onset 84 years or older) on an intake cognitive screening examination on initial presentation. The EAD and LAD groups did not differ on dementia or most cognitive variables. Compared with EAD, the LAD group had worse verbal fluency and motor-executive functions. These differences disappeared when age differences were taken into account. We conclude that Alzheimers disease is a clinically heterogeneous disorder whose manifestations can vary with age of onset. These differences indicate age-related vulnerabilities in this disease.

Positron Emission Tomography Facilitates Diagnosis of Early-Onset Alzheimer’s Disease

Background: Clinical positron emission tomography (PET) may help in the evaluation of presenile patients with memory complaints for the presence of Alzheimer’s disease (AD). Methods: Clinical PET scans from 27 patients with clinically probable AD and early ages of onset (<65 years) were compared to PET scans from 27 age-matched controls presenting with memory complaints, but without dementia or mild cognitive impairment. Results: Compared to controls, the AD patients had significant frontal, temporal and parietal hypometabolism bilaterally, and AD diagnosis correlated with left temporal and right temporoparietal hypometabolism. The sensitivity of temporoparietal hypometabolism for AD was 92.6%, the specificity 85.2%. Conclusion: Clinical PET imaging helps distinguish early-onset AD from patients with memory complaints not meeting criteria for dementia or mild cognitive impairment.

The Spectrum of Sociopathy in Dementia

Although well-known from head trauma and acute strokes, sociopathic behavior from dementia is less known and understood. This study reviewed 33 dementia patients who had been in trouble with the law. They were divided into two groups: 22 who committed impulsive sociopathic acts and 11 who committed non-impulsive acts. The impulsive patients demonstrated nonviolent acts, such as disinhibited sexual behavior or pathological stealing, and had disproportionate frontal-caudate atrophy on neuroimaging. The majority of non-impulsive patients demonstrated agitation-paranoia, sometimes with reactive aggression, delusional beliefs, or aphasic paranoia, and had advanced memory and other cognitive impairment. The impulsive patients tended to have frontally predominant illnesses such as frontotemporal dementia or Huntingtons disease, whereas the non-impulsive group tended to have Alzheimers disease or prominent aphasia. Sociopathy has different causes in dementia. Two common mechanisms are disinhibition, with frontally predominant disease, and agitation-paranoia, with greater cognitive impairment. These forms of sociopathy differ significantly from the antisocial/psychopathic personality.

The scale for emotional blunting in patients with frontotemporal dementia

Emotional blunting may underlie many of the behavioral features of frontotemporal dementia (FTD). The Scale for Emotional Blunting (SEB) was evaluated in 12 patients with early FTD, 12 patients with Alzheimers disease (AD), and 12 normal controls. There were overall group differences on the SEB, and the FTD patients had greater emotional blunting than the AD patients. The SEB had good inter-rater reliability and a sensitivity of 92%, and a specificity of 83.5% for FTD. These findings suggest that the SEB may be a good instrument for the early detection and quantification of emotional blunting in patient with FTD.

Neuropsychology for Psychologists, Health Care Professionals, and Attorneys, Third Edition

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Frontal-executive Versus Posterior-perceptual Mental Status Deficits in Early-onset Dementias:

Background: Compared to late-onset dementias, early-onset dementias (EODs) may have greater focal cognitive involvement with differences in frontal-executive compared to posterior-perceptual deficits. Objective: This study evaluated whether mental status screening based on this frontal-posterior axis can distinguish EODs. Methods: Twenty-three patients each with early-onset Alzheimers disease (eAD), frontotemporal dementia (FTD), or subcortical ischemic vascular disease (SIVD), and 20 normal controls underwent the Frontal Assessment Battery (FAB) and the Perceptual Assessment Battery (PAB). Results: Compared to controls, SIVD and FTD groups were impaired on the FAB whereas eAD and SIVD groups were impaired on the PAB. The FAB/PAB ratio further differentiated the groups (F(3,85) = 26.49, P < .001). For sensitivities and specificities of 93%, a cut-off score of 1.25 on the FAB/PAB distinguished eAD, and a cut-off of 0.83 distinguishing FTD. Conclusion: Although preliminary, this study indicates that mental status screening based on frontal versus posterior cortical functions may help clinicians diagnose EODs.

P3-078: Bedside cognitive testing in patients with early vs. late onset Alzheimer’s disease

Conclusions: 1) the amnestic sub-type is the most common among the subgroups of MCI patients (52.5%). 2) the single domain non-amnestic and the multiple non-amnestic subgroups consist only of 7.3% of the total MCI group. 3) memory impairment is the most common finding through neuropsychological testing (94.2% of the total MCI). 4) the second most common finding is the executive function impairment (89.2%). 5) in amnestic-MCI 20% has only memory impairment on tests. In the other 80% there is also executive function impairment.

P4-112: Is early frontotemporal dementia a dementia?

Background: In the absence of a biomarker, the diagnosis of frontotemporal dementia (FTD) depends on recognizing the neuropsychiatric symptoms of this disease. The traditional model of cognitive loss of Alzheimer’s disease (AD) may not apply in the beginning stages of FTD, when patients have predominant behavioral symptoms. Patients with early FTD often lack the multiple cognitive impairments or memory loss necessary to meet established criteria (DSM-IV or ICD-10) for dementia. Objective: To determine whether patients with FTD meet criteria for dementia on presentation within two years of onset of their disease. Methods: We reviewed all patients meeting Consensus Criteria for FTD (Neary et al, 1998) and having corroborative frontal or frontotemporal changes on functional neuroimaging (either PET or SPECT). All FTD patients in this study presented for evaluation in universitybased neurological clinics. All patients were within two years of onset of their first symptoms. This study included only the behavioral variant of frontotemporal lobar degeneration and excluded patients with primary progressive aphasia or semantic dementia. All patients underwent initial neurobehavioral and neuropsychological testing at baseline and again at two years. Results: On initial presentation, 23 (36.5%) patients met all core criteria for FTD and were diagnosed with the disorder; an additional 40 patients met all core criteria at two years from the initial presentation. Of these 63 patients eventually diagnosed with FTD, none met criteria (DSM-IV or ICD-10) for dementia at baseline by neurobehavioral or neuropsychological testing, and 43 (68.3%) met dementia criteria at two years from the initial presentation. Most FTD patients initially lacked sufficient memory and other cognitive deficits to characterize as “dementia.” Conclusion: FTD is often difficult to diagnose. Patients with FTD present with subtle personality or behavioral changes rather than cognitive deficits typical of AD. Although neuropsychological studies report abnormalities in frontal-executive functions and language, with less impaired memory and visuospatial skills than AD, the non-cognitive behavioral changes are actually the earliest and most characteristic features of FTD. This report suggests that early FTD defies the neuropsychological model of dementia and that clinicians need better diagnostic tools for recognizing this neuropsychiatric disorder. P4-113 CLINICOPATHOLOGIC AND BIOCHEMICAL OVERLAP OF FRONTOTEMPORAL DEGENERATION, CORTICOBASAL DEGENERATION AND PROGRESSIVE SUPRANUCLEAR PALSY: THE LILLE-BAILLEUL FRENCH COHORT Vincent Deramecourt, Stéphanie Bombois, Claude-Alain Maurage, Florence Lebert, André Delacourte, Florence Pasquier, Memory Clinic, University Hospital, Lille, France; Department of Neuropathology, University Hospital, Lille, France; INSERM U815, Lille, France. Contact e-mail: v-deramecourt@chru-lille.fr