Carol Gregory

Which neuropsychiatric and behavioural features distinguish frontal and temporal variants of frontotemporal dementia from Alzheimer's disease?

OBJECTIVES To investigate the prevalence of changes in mood, personality, and behaviour in frontotemporal dementia (FTD) and Alzheimers disease (AD) and hence, which features reliably distinguish between them. To establish whether the frontal and temporal variants of FTD are characterised by different behavioural changes. METHODS A questionnaire was designed to assess a wide range of neuropsychiatric changes; it incorporated features reported in previous studies of FTD and components of the neuropsychiatric inventory.1 This was completed by 37 carers of patients with Alzheimers disease (AD) and 33 patients with frontotemporal dementia (FTD), comprising 20 with temporal variant FTD (tv FTD) or semantic dementia and 13 with frontal variant FTD (fv FTD). An exploratory principal components factor analysis and discriminant function analysis was applied. RESULTS Factor analysis showed four robust and meaningful symptom clusters: factor 1—stereotypic and eating behaviour; factor 2—executive dysfunction and self care; factor 3—mood changes; factor 4—loss of social awareness. Only stereotypic and altered eating behaviour and loss of social awareness reliably differentiated AD from FTD with no effect of disease severity. By contrast, executive dysfunction, poor self care, and restlessness showed a significant effect of disease severity only, with the more impaired patients scoring more highly. Changes in mood were found to be equally prevalent in the three patient groups. Analysis of individual symptoms showed increased rates of mental rigidity and depression in the patients with semantic dementia compared with those with fv FTD. Conversely, the latter group showed greater disinhibition. Discriminant function analysis correctly classified 71.4% overall and 86.5% of the patients with AD. CONCLUSIONS This questionnaire disclosed striking differences between patients with FTD and AD, but only stereotypic behaviour, changes in eating preference, disinhibition, and features of poor social awareness reliably separated the groups. The patients with fv FTD and semantic dementia were behaviourally very similar, reflecting the involvement of a common network, the ventral frontal lobe, temporal pole, and amygdala. Dysexecutive symptoms and poor self care were found to be affected by the severity of the disease, reflecting perhaps spread to dorsolateral prefrontal areas relatively late in the course of both FTD and AD. This questionnaire may be of value in the diagnosis and the monitoring of therapies.

The differentiation of semantic dementia and frontal lobe dementia (temporal and frontal variants of frontotemporal dementia) from early Alzheimer's disease: A comparative neuropsychological study.

The authors compared age-matched groups of patients with the frontal and temporal lobe variants of frontotemporal dementia (FTD; dementia of frontal type [DFT] and semantic dementia), early Alzheimers disease (AD), and normal controls (n = 9 per group) on a comprehensive neuropsychological battery. A distinct profile emerged for each group: Those with AD showed a severe deficit in episodic memory with more subtle, but significant, impairments in semantic memory and visuospatial skills; patients with semantic dementia showed the previously documented picture of isolated, but profound, semantic memory breakdown with anomia and surface dyslexia but were indistinguishable from the AD group on a test of story recall; and the DFT group were the least impaired and showed mild deficits in episodic memory and verbal fluency but normal semantic memory. The frontal and temporal presentations of FTD are clearly separable from each other and from early AD.

Dissociation of Social Cognition and Executive Function in Frontal Variant Frontotemporal Dementia.

In this paper, we adopt a neurodevelopmental stance to examining frontal variant frontotemporal dementia (fv-FTD) by using experimental procedures from the literature on the growth of social behaviour in children to examine the deficits in social reasoning which may underpin behavioural disturbance in fv-FTD. We present the case of a 47-year-old man with a diagnosis of fv-FTD and severe antisocial behaviour. Tests of general neuropsychology and of executive function were performed. In addition, the patient, JM, was assessed on tasks which test theory of mind. Theory of mind develops in distinct stages through childhood and is a core ability to represent the thoughts and feelings of others, independent of the level of intellectual ability. The results indicate relatively intact general neuropsychological and executive function, but extremely poor performance on tasks of theory of mind. This indicates a dissociation of social cognition and executive function suggesting that in psychiatric presentations of fv-FTD there may be a fundamental deficit in theory of mind independent of the level of executive function. The implications of this finding for diagnostic procedures and possible behavioural management are discussed.

Comprehensive Textbook of Psychiatry/VI

Volume 1: neural sciences neuropsychiatry and behavioural neurology contributions of psychological sciences contributions of the sociocultural sciences quantitative and experimental methods in psychiatry theories of personality and psychopathology - psychoanalysis diagnosis and psychiatry - examination of the psychiatric patient clinical manifestations of psychiatric disorders classification of mental disorders delirium, dementia and amnesia and other cognitive disorders and mental disorders due to a general medical condition substance-related disorders schizophrenia other psychotic disorders mood disorders anxiety disorders somatoform disorders fractious disorders dissociative disorders normal human sexuaity, and sexual and gender identity disorders. Volume 2: eating disorders sleep disorders impulse-control disorders not elsewhere classified and adjustment disorders personality disorders psychologic factors affecting medical condition (psychosomatic disorders) relational problems additional problems that may be a focus of clinical attention psychiatry and other specialties psychiatric emergencies psychotherapies biological therapies child psychiatry psychiatric examination of the infant, child and adolescent mental retardation learning disorders, motor skills disorders, and communication disorders pervasive developmental disorders attention-deficit disorders disruptive behaviour disorders feeding and eating disorders of infancy and early childhood TIC disorders elimination disorders other disorders of infancy, childhood and adolescence mood disorders and suicide schizophrenia with childhood onset child psychiatry - psychiatric treatment child psychiatry - special areas of interest adulthood geriatric psychiatry hospital and community psychiatry psychiatric education forensic psychiatry psychiatry - past and future epilogue.

A study of stereotypic behaviours in Alzheimer’s disease and frontal and temporal variant frontotemporal dementia

Objective: To document the prevalence and pattern of stereotypic behaviour in patients with Alzheimer’s dementia and frontal and temporal variants of frontotemporal dementia. Secondly, to examine the relationship between stereotypic and other neuropsychiatric behaviours. Methods: Patients with the following were studied; Alzheimer’s disease (n=28), frontal variant frontotemporal dementia (fvFTD, n=18), and semantic dementia—the temporal lobe variant of FTD (n=13). All patients were assessed using the Neuropsychiatric Inventory (NPI), the Mini-Mental State Examination, Addenbrooke’s Cognitive Examination, and the Clinical Dementia Rating scale. Patients were also rated on the newly devised Stereotypic and Ritualistic Behaviour (SRB) subscale, which was designed as an addendum to the NPI. Results: There was no significant difference across diagnostic groups in terms of age, sex, or severity of cognitive deficits. The overall NPI was significantly higher in patients with fvFTD compared with the other two groups, but fvFTD and semantic dementia showed a similar, and significantly increased, prevalence of stereotypic behaviours on the SRB subscale. Within the FTD group as a whole these behaviours were more likely to be complex, whereas in Alzheimer’s disease, when present, such behaviours tended to be more simple stereotypies or stimulus bound repetitive behaviours. Stereotypic behaviours were not correlated with either disease severity or the extent of cognitive impairment in the fvFTD group, but were in the other two diagnostic groups. Conclusion: Complex stereotypic behaviours are a core feature of the dementing syndrome in FTD and may reflect early and specific deficits in orbitofrontal circuitry and basal ganglia involvement.

Clinical features of frontal lobe dementia in comparison to Alzheimer's disease.

Over the past decade it has become evident that a substantial minority of patients with primary dementing diseases, particularly those presenting in the presenium, have dementia of frontal lobe type (DFT) due to non-Alzheimers pathology. Although post-mortem remains the only method of definitive diagnosis, DFT and Alzheimers disease (AD) can, we would claim, be separated with a high degree of certainty based on a combination of informant history, neuropsychology and neuroimaging. In DFT, changes in personality, motivation, social interaction and organisational abilities, in the presence of well preserved memory and visuospatial abilities, are characteristic. The lack of insight emphasises the need for independent information particularly as patients may perform normally on bedside (and more sophisticated) tests of cognition. Psychiatric features especially mood disturbance appear to be common, but their prevalence remains to be established. In contrast, AD is a progressive amnestic disorder with episodic and semantic memory deficits, followed by breakdown in other attentional, perceptual and visuo-spatial abilities, which reflects the major locus of pathology in AD, namely the medial temporal lobe. These features are illustrated by reference to a longitudinal study of 52 patients with minimal to moderate AD. In addition, we shall describe the results of retrospective and prospective neuropsychiatric studies of a group of DFT patients.

Frontal variant of frontotemporal dementia: a cross-sectional and longitudinal study of neuropsychiatric features

BACKGROUND The term frontotemporal dementia (FTD) covers both the temporal and frontal presentations of this condition. The frontal variant (fv) presents with insidious changes in personality and behaviour, with neuropsychological evidence of disproportionate frontal dysfunction. Although psychiatric features are well recognized, there is little systematic data examining the mental state using assessment instruments and no reported studies of the longitudinal progress and assessment. METHODS Fifteen patients with a diagnosis of FTD(fv) were assessed using the Comprehensive Psychiatric Rating Scale (CPRS). A subgroup of five patients were reassessed annually using the same instrument, generating data over a 3-year period. RESULTS At initial assessment a third of 15 patients had no psychiatric symptoms to report. Three patients reported symptoms of sadness, but only one patient met criteria for DSM-IV major depressive episode. One patient experienced symptoms of elation, but did not meet criteria for manic episode, while two patients had hypochondriacal complaints but did not meet DSM-IV criteria for hypochondriasis. One of these patients also experienced the compulsion to count but did not meet criteria for obsessive compulsive disorder. The objective mental state was, on the whole, not congruent with the reported symptoms. Five patients assessed over a 3-year period showed no progression of their subjectively reported symptoms. CONCLUSIONS Psychiatric symptoms although often present were characterized by their shallowness, lack of elaboration and non-development over time.

Dementia of frontal type and the focal lobar atrophies

Dementia of frontal type(DFT) and the other focal lobar atrophies challenge the clinician to assess patients with dementia thoroughly. DFT presents with characteristic behavioural and personality changes, and operational criteria have recently been devised to assist in the assessment and diagnosis. A significant proportion of cases present with functional psychiatric disorders including mood disturbance. Diogenes syndrome may be a variant of DFT. A number of syndromes associated with focal lobar atrophy of other brain regions have also been described, most common of these is primary progressive aphasia (PPA). Bedside cognitive and formal neuropsychological testing, and neuroimaging—both functional (SPECT) and anatomical—may assist in the diagnosis of these disorders. At post-mortem, non-specific atrophy is present; in DFT, the frontal lobes are affected, and in PPA, the focus of pathology is the dominant temporal lobe. Only a minority of cases show Pick cells and Picks bodies, in the remainder non-specifi...

Dementia of frontal type and simple schizophrenia: Two sides of the same coin?

Abstract Dementia of frontal type and simple schizophrenia have many overlapping features; both present with insidious onset of functional, social and occupational decline and have features of affective blunting, poor motivation and lack of insight in common. Neuropsychological assessment and neuroimaging may not always separate these two conditions in their early stages. We present an illustrative case and discuss pitfalls in the diagnosis and suggest areas for future research.

Pharmacological Management of Schizophrenia in Older Patients

SummaryAlthough antipsychotic drugs are the mainstay of treatment in older patients with schizophrenia, much of the theoretical work underpinning their use is based on evidence gained from younger patients. With respect to dosages, there has been little work comparing plasma concentrations of antipsychotics in older patients with those of younger patients. However, there are well documented changes in the pharmacokinetics of these drugs in the elderly, particularly in their hepatic metabolism and renal excretion. There is also evidence that older patients experience more adverse effects from antipsychotics than younger patients. Such effects include extrapyramidal symptoms, postural hypotension and falls. For these reasons it is recommended that starting doses of antipsychotic drugs in older patients should be in the region of 25 to 50% of that recommended for younger patients, and should be slowly increased. Selection of a particular antipsychotic agent is best made on the basis of individual patient characteristics and the adverse effect profiles of particular drugs.