A short neuropsychologic and cognitive evaluation of frontotemporal dementia

Objective

To elaborate a brief but efficient neuropsychological assessment of frontotemporal dementia (FTD), selecting the most specific and sensitive cognitive and behavioural items for distinguish between AD and FTD in the earlier dementia stages.

Methods

Retrospective study with three groups, 35 patients with FTD, 46 with AD and 36 normal subjects, were administered the MMSE, FAB, Tower of London and Stoop's test along with a 98 items behavioural and cognitive questionnaire. The most sensitive items were selected and validated internally for diagnosis by lineal discriminant analysis.

Results

From the 98 items in the questionnaire, 29 showed significant discriminatory power. Non-cognitive symptoms with higher odd-ratio for FTD compared to AD were impairment in social behaviour (disinhibition, aggressiveness), loss of insight and inappropriate acts. Language disorders, such as echolalia, verbal apraxia or aggramatism, dominate in the cognitive profile of FTD. FAB was confirmed as the best cognitive instrument to differentiate FTD and AD. A linear discriminant function with the combination of the FAB score and the items from our questionnaire with higher OR for FTD accurately classified 97% of individuals.

Conclusions

The neuropsychological tests allow the differentiation between FTD and AD. The combination of FAB test with the assessment of key behavioural and cognitive symptoms appears helpful in this distinction.     

Clinical profiles of late‐onset semantic dementia,compared with early‐onset semantic dementia and late‐onset Alzheimer's disease

Background: Semantic dementia (SD) has been recognized as a representative of dementia with presenile onset; however, recent epidemiological studies have shown that SD also occurs in the elderly. There have been few studies about the differences of clinical profiles between early‐onset SD (EO‐SD) and late‐onset SD (LO‐SD). Age‐associated changes in the brain might cause some additional cognitive and behavioural profiles of LO‐SD in contrast to the typical EO‐SD cases. The aim of the present study was to clarify the characteristics of neuropsychological, and behavioural and psychological symptoms of dementia (BPSD) profiles of LO‐SD patients observed in screening tests in comparison with EO‐SD patients and late‐onset Alzheimer's disease (LO‐AD) patients as controls. Methods: Study participants were LO‐SD (n = 10), EO‐SD (n = 15) and LO‐AD (n = 47). We examined the Mini‐Mental State Examination (MMSE), the Raven's Coloured Progressive Matrices (RCPM), the Short‐Memory Questionnaire (SMQ), the Neuropsychiatric Inventory (NPI) and the Stereotypy Rating Inventory (SRI). Results: Both SD groups scored significantly lower than the LO‐AD patients in ‘naming’ of the MMSE. In the ‘construction’ score of the MMSE and the RCPM score, however, the LO‐SD patients as well as the LO‐AD patients were significantly lower than the EO‐SD patients. In the SMQ score, ‘euphoria’ and ‘disinhibition’ scores of the NPI, the SRI total and subscale scores, both SD groups were significantly higher, whereas in the ‘delusion’ score of the NPI, both SD groups were significantly lower than the LO‐AD patients. Conclusions: Visuospatial and constructive skills of LO‐SD patients might be mildly deteriorated compared with EO‐SD patients, whereas other cognitive and behavioural profiles of LO‐SD are similar to EO‐SD. Age‐associated changes in the brain should be considered when we diagnose SD in elderly patients.     

Frontotemporal dementia: a clinically complex diagnosis

OBJECTIVE: To compare the time taken to establish a clinical diagnosis of Frontotemporal dementia (FTD) relative to a diagnosis of early onset Alzheimer's dementia (AD). METHODS: The data came from 89 patients under the age of 65 years, 52 of whom met the Manchester-Lund criteria for Frontotemporal dementia; 20 of these came from Lund University Hospital in Sweden. The other 32 patients with FTD along with 37 subjects who fulfilled the ICD-10 criteria for early onset Alzheimer's disease were recruited from four memory clinics and two neurology departments in Norway. RESULTS: For FTD patients in Norway it took 59.2 months (SD 36.1) from the onset of illness until a clinical FTD diagnosis was made. The corresponding time period for FTD patients in Sweden is 49.5 months (SD 24.5) and for AD patients in Norway 39.1 months (SD 19.9). The time from the first visit to a medical doctor until a diagnosis was made for the FTD patients in Norway was 34.5 months (SD 22.6), for the Swedish FTD patients 23.1 months (SD 22.4) and for the AD patients 25.9 months (SD 13.1). In all, 71% of FTD patients and 30% of AD patients initially received a non-dementia diagnosis. CONCLUSION: More knowledge about early presenting cognitive and behavioural signs of FTD is needed in both primary and secondary health care to reduce the time period needed to establish a clinical diagnosis of FTD.     

The relationship between pre-morbid personality and challenging behaviour in people with dementia: A systematic review

It has been suggested that challenging behaviour in people with dementia reflects a person's pre-morbid personality traits and a number of studies have explored this hypothesis. However, inconsistencies in outcome between studies suggest a need to review the available evidence systematically. As a result, major bibliographic databases were searched for studies examining the relationship between pre-morbid personality and challenging behaviour in order to conduct a systematic review. We included all English language studies published in referenced journals that assessed pre-morbid personality via a valid comprehensive personality measure, and also explored a relationship with challenging behaviour in people with dementia. A total of 18 studies were identified that covered a wide range of challenging behaviours including ‘wandering’, affective states, aggression, anxiety and delusions/hallucinations. Studies were assessed for their methodological quality and statistical findings. Studies lacked representative samples, were affected by confounding variables and suffered from small sample sizes. However, 72% of the studies reported significant relationships between pre-morbid personality and behaviour. In terms of specific relationships, the strongest evidence was found for a positive relationship between pre-morbid neuroticism and mood, and aggression and overall behavioural acts, thus supporting the inclusion of personality as one factor in the formulation of behaviour (Ballard, C., O’Brien, J., James, I., & Swann, A. (2001). Dementia: Management of Behavioural and Psychological Symptoms. Oxford: Oxford University Press; Kitwood, T. (1993 Kitwood, T. 1993. Person and process in dementia: Editorial. International Journal of Geriatric Psychiatry, 1: 541–545. [Crossref] [Google Scholar]). Person and process in dementia: Editorial. International Journal of Geriatric Psychiatry, 1, 541–545).     

Electroencephalographic markers in dementia

Dementia is a global health problem with a huge impact on the lives of those afflicted. There are several distinct diseases that are classified under the umbrella term “dementia” ranging from neurodegenerative disorders such as Alzheimer's disease to chronic infections of the central nervous system such as subacute sclerosing panencephalitis (SSPE), a rare complication of measles virus infection in childhood. Clinical features, neuropsychological profiles and imaging characteristics of the various dementia syndromes can be sufficiently distinct to distinguish them from one another. However, in some cases, the cognitive, psychiatric and behavioural features can sufficiently overlap such that neurophysiologic testing may be of help. While it is recognized the electroencephalogram (EEG) may have a special role to play in the diagnosis of certain dementing illnesses such as SSPE and Creutzfeldt–Jakob disease (CJD) that have characteristic EEG changes, current research focusses on the potential utility of quantitative EEG as one more tool in the armamentarium of clinicians dealing with patients who suffer from a dementing illness. We searched PubMed and the Cochrane Database from 1 January 1946 up to 1 January 2016, combining the search terms “EEG,” “electroencephalography,” “dementia” and “status epilepticus”; identified papers from these searches were then read in detail and summarized. Here, we discuss both the qualitative and quantitative EEG findings in the various types of dementia.     

Frontotemporal dementia: Pick type

The status of Pick's disease within the concept of frontotemporal dementia (FTD) is unclear. Some researchers have defined Pick's disease as FTD with Pick bodies. Alternatively, the confusion may be clarified by using the term Pick body dementia (PBD) rather than by using the term Pick's disease in a narrow sense. Pick body dementia is characterized by a prominent frontotemporal lobar atrophy, gliosis, severe neuronal loss, ballooned neurons, and the presence of neuronal inclusions called Pick bodies. In recent years, studies of Pick body dementia have advanced from the standpoint of the tau pathology. Tau-positive glial inclusions as well as neuronal inclusions have been observed in PBD and its related disorders. Various forms of FTD have been proposed based on the presence of neuronal or glial inclusions. We propose a new variant of FTD termed ‘glial tangle-predominant type’. More research is required to understand the tau abnormalities in the various forms of FTD.     

A retrospective study of the behavioural and psychological symptoms of mid and late phase Alzheimer's disease

AIM: To document the behavioural and psychological symptoms in patients with a diagnosis of established Alzheimer's disease (AD) for at least 3 years. METHODS: Patients with a > or =3 year history of AD (NINCDS/ADRDA) were recruited from old age psychiatrist and elderly care memory clinics. Information regarding duration of symptoms and non-cognitive symptomatology was obtained during interview with a carer or next-of-kin who had contact with the patient at least 3 times a week and for at least 3 years. MMSE, FAST and NPI including caregiver distress, were used to assess cognition, function and behavioural/psychological disturbance respectively. With each non-cognitive symptom the carer was asked to estimate its onset. RESULTS: The mean age of patients was 77 years and duration of illness 87 months. Mean MMSE was 8/30 and FAST score 6d. Of the psychological symptoms occurring at any stage, depression (56%), delusions (55%) and anxiety (52%) were most common, with hallucinations, elation and disinhibition occurring less frequently. In general, behavioural changes were more common with apathy occurring in 88% of patients, motor behaviour in 70%, aggression in 66%, irritability and appetite changes in 60% and sleep disturbance in 54%. All symptoms except apathy became less common when the carer was asked if they were still present in the last month. Mean onset of psychological symptoms was 47 months. Mean onset of behavioural symptoms was 48 months. Behavioural disturbance seemed to cause more care-giver distress than psychological change. CONCLUSION: The results show behavioural and psychological symptoms in AD are common and distressing for carers. They appear to require a consistent period of neurodegeneration in order to emerge.     

Aspirin,anti-inflammatory drugs and risk of dementia

Objective. To test the hypothesis that aspirin and non-steroidal anti-inflammatory drugs (NSAIDs) may prevent dementia or cognitive impairment. Design. A two-wave longitudinal study over 3.6 years. Setting. A community survey of elderly persons living in Canberra, Australia. Participants. There were 1045 elderly persons aged 70 at the start of the study; cognitive assessment was obtained at both waves on 588. Main outcome measures. Cognitive functioning was measured using the Mini-Mental State Examination, an episodic memory test, a test of mental speed and the National Adult Reading Test. Dementia was assessed using the Canberra Interview for the Elderly. Results. On cross-sectional data, those who had been taking NSAIDs or aspirin performed no better on the cognitive tests after account had been taken of other confounding variables. There was no interaction with apolipoprotein E genotype. On longitudinal data, no difference was found between NSAID or aspirin users and controls, either in cognitive decline or incidence of dementia. Conclusions. The results do not support the hypothesis that aspirin or NSAIDs have a protective effect, but it remains possible that various sources of measurement error may have attenuated an effect of clinical significance from either type of drug. Conclusive evidence can be obtained only by a prospective trial. © 1997 John Wiley & Sons, Ltd.     

Apolipoprotein E genotypes and longevity across dementia disorders

Introduction

The ε4 allele of the apolipoprotein E (APOE) gene is a prominent risk factor for Alzheimer's disease (AD), but its implication in other dementias is less well studied.

A figurative proverb test for dementia: rapid detection of disinhibition,excuse and confabulation,causing discommunication

Background: Communicative disability is regarded as a prominent symptom of demented patients, and many studies have been devoted to analyze deficits of lexical‐semantic operations in demented patients. However, it is often observed that even patients with preserved lexical‐semantic skills might fail in interactive social communication. Whereas social interaction requires pragmatic language skills, pragmatic language competencies in demented subjects have not been well understood. We propose here a brief stress‐free test to detect pragmatic language deficits, focusing on non‐literal understanding of figurative expression. We hypothesized that suppression of the literal interpretation was required for figurative language interpretation. Methods: We examined 69 demented subjects, 13 subjects with mild cognitive impairment and 61 healthy controls aged 65 years or more. The subjects were asked the meaning of a familiar proverb categorized as a figurative expression. The answers were analyzed based on five factors, and scored from 0 to 5. To consider the influence of cognitive inhibition on proverb comprehension, the scores of the Stroop Colour–Word Test were compared concerning correct and incorrect answers for each factor, respectively. Furthermore, the characteristics of answers were considered in the light of excuse and confabulation qualitatively. Results: The proverb comprehension scores gradually decreased significantly as dementia progressed. The literal interpretation of the proverb, which showed difficulties in figurative language comprehension, was related to disinhibition. The qualitative analysis showed that excuse and confabulation increased as the dementia stage progressed. Conclusions: Deficits in cognitive inhibition partly explains the difficulties in interactive social communication in dementia. With qualitative analysis, asking the meaning of a proverb can be a brief test applied in a clinical setting to evaluate the stage of dementia, and to illustrate disinhibition, confabulation and excuse, which might cause discommunication and psychosocial maladjustment in demented patients.