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Background: The criteria for behavioral variant frontotemporal dementia (bvFTD) incorporate MRI and [18F]-FDG-PET. Cerebrospinal fluid (CSF) analysis is merely advised for excluding Alzheimer's disease., Aims: We aimed to assess the impact of biomarkers on diagnostic certainty and contingent changes of bvFTD diagnosis within the clinically relevant neuropsychiatric differential diagnosis of subjects with a late-onset frontal lobe syndrome (LOF)., Methods: We included 137 patients with LOF, aged 45-75 years, 72% males. Biomarker disclosure was considered contributing after any substantial difference in diagnostic certainty or a diagnostic change. Percentages of contributing biomarkers were compared between three major diagnostic groups (bvFTD, psychiatry, other neurological disorders). Certainty levels in stable diagnostic groups were compared to those with a diagnostic change., Results: Biomarkers contributed in 53, 60 and 41% of the LOF patients for MRI, [18F]-FDG-PET and CSF, respectively. Biomarkers changed the diagnosis in 14% of cases towards bvFTD and in 13% from bvFTD into an alternative. Those that changed had a lower level of a priori diagnostic certainty compared to stable diagnoses., Conclusion: Our study not only supports the widely accepted use of MRI and [18F]-FDG-PET in diagnosing or excluding bvFTD, but also shows that CSF biomarkers aid clinicians in the diagnostic process., (© 2015 S. Karger AG, Basel.)

Objective: The behavioral variant of frontotemporal dementia (bvFTD) can be difficult to diagnose because of the extensive differential diagnosis, including many other diseases presenting with a frontal lobe syndrome. We aimed to identify the diagnostic spectrum causing a late onset frontal lobe syndrome and examine the quality of commonly used instruments to distinguish between bvFTD and non-bvFTD patients, within this syndrome., Methods: A total of 137 patients fulfilling the criteria of late onset frontal lobe syndrome, aged 45 to 75 years, were included in a prospective observational study. Diagnoses were made after clinical and neuropsychological examination, and neuroimaging and cerebral spinal fluid results were taken into account. Baseline characteristics and the scores on the Mini-Mental State Exam (MMSE), frontal assessment battery (FAB), Frontal Behavioral Inventory (FBI), and Stereotypy Rating Inventory (SRI) were compared between the bvFTD and the non-bvFTD group., Results: Fifty-five (40%) of the patients received a bvFTD diagnosis (33% probable and 7% possible bvFTD). Fifty-one patients (37%) had a psychiatric disorder, including 20 with major depressive disorder. Thirty-one patients received an alternative neurological, including neurodegenerative, diagnosis. MMSE and FAB scores were unspecific for a particular diagnosis. A score above 12 on the positive FBI subscale or a score above 5 on the SRI were indicative of a bvFTD diagnosis., Conclusion: A broad spectrum of both neurological and psychiatric disorders underlies late onset frontal lobe syndrome, of which bvFTD was the most prevalent diagnosis in our cohort. The commonly used MMSE and the FAB could not successfully distinguish between bvFTD and non-bvFTD, but this could be achieved with the more specific FBI and SRI., (Copyright © 2015 American Association for Geriatric Psychiatry. Published by Elsevier Inc. All rights reserved.)

Aims: A clinical frontal lobe syndrome (FLS) is generally attributed to functional or structural disturbances within frontal-subcortical circuits. We studied the distribution of pathological brain changes in FLS. Additionally, the prevalence of FLS among various disorders was studied., Methods: We systematically screened clinical files of donors to the Netherlands Brain Bank (n = 2,814) for FLS. A total of 262 FLS cases were identified, and the distribution of postmortem pathological changes within the frontal-subcortical circuits was extracted from their neuropathological reports., Results: In 244 out of 262 patients (93%), pathological changes within the frontal-subcortical circuits were found: 90 subjects (34%) with frontal cortical pathology and 18 (7%) with pathology restricted to subcortical grey matter nuclei, whereas 136 subjects (52%) showed both cortical and subcortical pathology. In 18 subjects (7%), no pathology was found in the examined areas. The prevalence of FLS was highest in frontal-temporal lobar degeneration, followed by progressive supranuclear palsy and vascular dementia [χ(2)(6, n = 1,561) = 222.64, p < 0.01]., Conclusion: In this large brain bank study, the distribution of pathological changes in subjects with FLS was shown to be frontal-subcortical for the first time. A minority of FLS cases had pathology in the subcortical regions only or no frontal pathology at all., (© 2015 S. Karger AG, Basel.)

The function of the frontal lobes and the related frontal lobe syndrome have not been described in detail until relatively late in history. Slowly, the combination of knowledge from animal models, the detailed examination of symptoms after traumatic frontal lobe injuries, and the rise and fall of psychosurgery has led to increasing insight into frontal lobe function. The frontosubcortical circuits have been described and increasingly related to clinical syndromes, confirmed by the latest developments in functional connectivity networks., (© 2015 S. Karger AG, Basel.)

Background: Frontotemporal dementia (FTD) is characterised clinically by progressive changes in behaviour and personality; these changes are followed by cognitive disorder. FTD needs to be differentiated from other forms of dementia and from psychiatric conditions such as schizophrenia. Both FTD and schizophrenia lead to cognitive disorders and particularly to executive impairments., Aim: To compare executive and general cognitive functioning in patients with FTD and in patients with schizophrenia in later life., Method: As cognitive screening instruments we used the 'Frontal Assessment Battery' (FAB) and the 'Mini-Mental State Examination' (MMSE). The FAB en MMSE test results (retrieved from the database of the Alzheimer centre of the VU medical centre) for 25 outpatients diagnosed as having FTD were compared with the test results (retrieved from the 'SOUL' study database) for 31 elderly schizophrenia patients., Results: In both the fab and the MMSE tests the scores for the patients with FTD were significantly lower than the scores for the patients with schizophrenia., Conclusion: Our study suggests that, despite the clinical similarities, there are differences between patients with FTD and elderly patients with schizophrenia with regard to executive and general cognitive functioning. Further studies are needed in order to differentiate between the two illnesses.

Introduction: The presence of frontal and/or temporal atrophy on neuroimaging has been designated as supportive in the clinical consensus criteria for behavioral variant frontotemporal dementia (bvFTD). As magnetic resonance imaging (MRI) has a relatively low sensitivity for bvFTD, a substantial proportion of patients may present with a normal MRI. Thus, there may be clinical differences between patients with and without lobar abnormalities on MRI. We compared clinical characteristics of bvFTD patients with frontotemporal lobar atrophy on MRI to those lacking the typical pattern at presentation., Methods: MRIs of 49 patients from our memory clinic, diagnosed with bvFTD were rated for the presence or absence of frontal and/or temporal atrophy. Demographic, behavioral, and cognitive features were compared between subjects with and without typical bvFTD atrophy pattern., Results: Twenty-three patients showed lobar atrophy on MRI, whereas 26 patients lacked the characteristic frontotemporal lobar atrophy, including 13 patients with a normal MRI and 13 with other abnormalities. Disinhibition occurred more often in the group with frontal and/or temporal atrophy on MRI compared with the group with other abnormalities, whereas imitation did not occur in patients lacking the typical bvFTD atrophy pattern. No differences were found in neuropsychologic profiles. There was a trend for a lower mean Clinical Dementia Rating and less severe language impairment in patients with a normal MRI compared with the group with frontal and/or temporal atrophy., Conclusions: The clinical phenotype of FTD cannot be predicted by the presence or absence of lobar atrophy on MRI, although imitation and disinhibition are more prevalent in bvFTD patients with characteristic MRI abnormalities. Furthermore, patients with a normal MRI seem to be less severely demented in comparison to patients with frontal and/or temporal atrophy.

The diagnosis of psychotic disorders that develop later in life is complicated, as can be seen from the case of a 65-year-old woman. Initially she was admitted to hospital for psychotic depression, but after some time doubts arose regarding the diagnosis. The most striking symptoms were bizarre delusions with acoustic, haptic and gustatory hallucinations. In addition, she showed behavioral and personality changes. It is difficult to establish whether a patient has late-onset schizophrenia or frontotemporal dementia. The similarities and differences between the symptoms of these two disorders are discussed and advice is given to assist with clinicians with diagnosis in the future.

Objective: To compare frontal cortex activation in patients with early frontotemporal dementia (FTD) with that in patients with early AD., Methods: Seven patients with FTD and seven patients with AD were studied (Clinical Dementia Rating: four patients with FTD 0.5, three patients with FTD 1, all patients with AD 1; mean Mini-Mental State Examination score: FTD 28.0 +/- 2.1, AD 23.1 +/- 2.7). Cerebral atrophy on MRI was mild, with no differences between FTD and AD. A parametric working memory task was applied to assess frontal activation as a function of working memory load., Results: The activated working memory network in FTD and AD included frontal and parietal lobe and thalamus. In frontal and parietal cortex, brain activation was significantly decreased in FTD. Frontal regions in patients with FTD showed less linear activation increase with working memory load than in AD. Possibly as a compensation mechanism, the cerebellum showed a stronger increasing response in FTD., Conclusions: These data on regional functional loss in the frontal cortex in early FTD suggest that fMRI can identify FTD when results on structural MRI are normal.