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Test battery for the diagnosis of dementia in individuals with intellectual disability. Working Group for the Establishment of Criteria for the Diagnosis of Dementia in Individuals with Intellectual Disability.

A working battery of tests for the diagnosis of dementia, which is applicable to most adults with intellectual disability, is proposed by an international Working Group. The battery, reflecting contemporary research and practice findings, includes scales for informant report of functioning and tests for direct assessment. The Working Group recommends the international use of the battery both as part of ongoing and new longitudinal research, and in clinical practice. The widespread use of a common battery will enhance communication and collaborative opportunities among researchers and clinicians at various sites, and will help to standardize diagnostic protocols and research findings. The collaborative evaluation of such a battery will address one of the greatest challenges in the field, that of differentiating change associated with ageing from that associated with dementia.

Adult↗

Awareness in dementia: ethical and legal issues in relation to people with dementia.

Our improved understanding of the experience of people with dementia provides a new impetus to address legal and ethical issues. This paper explores emerging issues in relation to awareness in dementia and its impact on legal and ethical matters. The different approaches and principles demonstrated in relation to ethical issues are discussed, with an exploration of the concepts of beneficence, non-maleficence, autonomy, and paternalism. Application of these concepts is discussed in relation to advance directives, capacity, and decision making, participation in research and treatment, including informed consent, and truth telling. The tensions that exist between the imperatives of doing no harm and of maintaining autonomy in addressing legal and ethical issues are highlighted, and attention drawn to the manner in which the attribution of unawareness is used to justify withholding autonomy. The review emphasizes the importance of considering competency and awareness as being multi-faceted, to be understood in the context of social interaction.

Cognition↗

Saccadic eye movement changes in Parkinson's disease dementia and dementia with Lewy bodies.

Neurodegeneration in Parkinson's disease dementia (PDD) and dementia with Lewy bodies (DLB) affect cortical and subcortical networks involved in saccade generation. We therefore expected impairments in saccade performance in both disorders. In order to improve the pathophysiological understanding and to investigate the usefulness of saccades for differential diagnosis, saccades were tested in age- and education-matched patients with PDD (n = 20) and DLB (n = 20), Alzheimer's disease (n = 22) and Parkinson's disease (n = 24), and controls (n = 24). Reflexive (gap, overlap) and complex saccades (prediction, decision and antisaccade) were tested with electro-oculography. PDD and DLB patients had similar impairment in all tasks (P > 0.05, not significant). Compared with controls, they were impaired in both reflexive saccade execution (gap and overlap latencies, P < 0.0001; gains, P < 0.004) and complex saccade performance (target prediction, P < 0.0001; error decisions, P < 0.003; error antisaccades: P < 0.0001). Patients with Alzheimer's disease were only impaired in complex saccade performance (Alzheimer's disease versus controls, target prediction P < 0.001, error decisions P < 0.0001, error antisaccades P < 0.0001), but not reflexive saccade execution (for all, P > 0.05). Patients with Parkinson's disease had, compared with controls, similar complex saccade performance (for all, P > 0.05) and only minimal impairment in reflexive tasks, i.e. hypometric gain in the gap task (P = 0.04). Impaired saccade execution in reflexive tasks allowed discrimination between DLB versus Alzheimer's disease (sensitivity > or =60%, specificity > or =77%) and between PDD versus Parkinson's disease (sensitivity > or =60%, specificity > or =88%) when +/-1.5 standard deviations was used for group discrimination. We conclude that impairments in reflexive saccades may be helpful for differential diagnosis and are minimal when either cortical (Alzheimer's disease) or nigrostriatal neurodegeneration (Parkinson's disease) exists solely; however, they become prominent with combined cortical and subcortical neurodegeneration in PDD and DLB. The similarities in saccade performance in PDD and DLB underline the overlap between these conditions and underscore differences from Alzheimer's disease and Parkinson's disease.

Aged↗

Xenon contrast CT-CBF scanning of the brain differentiates normal age-related changes from multi-infarct dementia and senile dementia of Alzheimer type.

Local cerebral blood flow (LCBF) and partition coefficients (L lambda) were measured during inhalation of stable xenon gas with serial CT scanning among normal volunteers (N = 15), individuals with multi-infarct dementia (MID, N = 10), and persons with senile dementia of Alzheimer type (SDAT, N = 8). Mean gray matter flow values were reduced in both MID and SDAT. Age-related declines in LCBF values in normals were marked in frontal cortex and basal ganglia. LCBF values were decreased beyond normals in frontal and temporal cortices and thalamus in MID and SDAT, in basal ganglia only in MID. Unlike SDAT and age-matched normals, L lambda values were reduced in fronto-temporal cortex and thalamus in MID. Multifocal nature of lesions in MID was apparent. Coefficients of variation for LCBFs were greater in MID compared with SDAT and/or age-matched normals.

Age Factors↗

Evaluating the effectiveness of community-based dementia care networks: the Dementia Care Networks' Study.

PURPOSE: The Dementia Care Networks' Study examined the effectiveness of four community-based, not-for-profit dementia networks. The study involved assessing the relationship between the types of administrative and service-delivery exchanges that occurred among the networked agencies and the network members' perception of the effectiveness of these exchanges. DESIGN AND METHODS: With the use of a case-study method, the evolution, structure, and processes of each network were documented. Social network analysis using a standardized questionnaire completed by member agencies identified patterns of administrative and clinical exchanges among networked agencies. RESULTS: Differences were found between the four networks in terms of their perceptions of service-delivery effectiveness; perceptions of administrative effectiveness did not factor significantly. Exchanges between groups of agencies (cliques) within each of the four networks were found to be more critical than those between individual agencies within each network. IMPLICATIONS: Integration-measured by the types of exchanges within as opposed to across networks-differentiated the four networks studied. This research contributes to our understanding of the use of multiple measures to evaluate the inner workings of service delivery and their impact on elder health and elder health care.

Aged↗

Behaviors associated with dementia: whether resisting care or exhibiting apathy, an older adult with dementia is attempting communication. Nurses and other caregivers must learn to 'hear' this language.

Seemingly simple adjustments in care routines and approaches can make a significant difference in the experiences of people with dementia. This article provides an overview of cognitive impairment in older adults and suggests interventions that can help providers understand the "behavioral symptoms" of dementia, which are often considered a way of communicating unmet needs. By focusing on the person rather than on the disease, nurses promote comfort and functional autonomy in older adults whose cognitive impairments have progressed and yet who are very much alive and deserving of respectful, dignified care.

Aged↗

Informant-based staging of dementia using the clinical dementia rating.

The staging of dementia is ideally based on both an examination of the patient and a history taken from an informant. However, in some circumstances, only an informant history is possible. The aim of this study was to assess the validity of the Clinical Dementia Rating (CDR) when the rating is based solely on informant data. The CDR was used in a study of 360 persons aged 78 or older who were participants in a community survey, the Sydney Older Persons Study. The CDR was completed in two ways: (1) a social scientist made the ratings based on an informant interview; and (2) a physician made the ratings after an examination of the subject. All CDRs were scored in the conventional way, as well as by the revised method proposed by Gelb and St. Laurent (Alzheimer Dis Assoc Disord 1993;4:202-11). The informant CDR showed moderate agreement with the clinician CDR, showing that it would be a valid substitute in situations in which the subject could not be examined. The revised scoring method was slightly easier to implement than the conventional method.

Aged↗

Is motor neuron disease-inclusion dementia a forme fruste of amyotrophic lateral sclerosis with dementia? An autopsy case further supporting the disease concept.

We report the autopsy findings of a 62-year-old man who exhibited progressive FTD 10 years before the appearance of muscle weakness and wasting, and who died approximately 11 years after onset of the symptoms. Degeneration and atrophy of the frontal and temporal lobes, which contained ubiquitin-positive neuronal inclusions and dystrophic neurites, were evident. Circumscribed degeneration affecting the hippocampal CA1-subiculum border zone was also a feature. Moreover, degeneration was present in both the upper and lower motor neuron systems, the latter being more severely affected. A few lower motor neurons were found to contain the cytoplasmic inclusions characteristic of ALS (i.e. Bunina bodies and ubiquitin-positive skeins). Also of interest was the presence of pallidonigroluysian atrophy, which appeared to be responsible for the chorea-like involuntary movements that developed in this patient approximately 2 months before death. The clinical and pathological features of our patient further support the idea that motor neuron disease-inclusion dementia (MND-ID), which has been classified as a pathological subgroup of FTD, is a forme fruste of ALS with dementia. In other words, if patients with MND-ID live long enough, they may develop ALS.

Amyotrophic Lateral Sclerosis↗

Circulating vitamins in Alzheimer's dementia as compared with other dementias.

Blood levels of 12 vitamins were compared in 55 patients with Alzheimer's disease and 58 control subjects (10 intellectually alert, 12 depressed, 28 multi-infarct dementias, 8 with other specific dementias). No significant differences in vitamin blood levels were found between the Alzheimer's disease group and the control groups either individually or in total. Conventional parenteral vitamin treatment of seven patients with vitamin deficits did not benefit their cognitive state. Conventional vitamin malnutrition did not seem to contribute to the disability in these subjects.

Aged↗

Cerebrospinal fluid investigations in multi-infarct dementia and senile dementia.

Thirty-eight demented patients were clinically investigated and classified into a multi-infarct dementia (MID) and senile dementia (SD) group. Total protein, albumin, immunoglobulin G, agar gel electrophoretic protein and iso-electric focusing pattern were determined in CSF and serum as well as the lactate dehydrogenase, LD-iso-enzyme pattern and uric acid. No significant differences were found regarding total protein, albumin and immunoglobulin G. The agar gel electrophoresis showed a tau-globulin increase in 18% of the SD-group against 6% of the MID group. The patterns were, however, plasma-like in 56% of the MID group against 23% of the SD group. The iso-electric focusing pattern showed an abnormal band in some SD-patients which was not found in the MID group. The uric acid and LD-enzyme concentration was equal in both CSF and serum. An index [Formula: see text] was calculated to give an approximate correction for the influence of different permeability of the blood-brain and CSF barrier. A significantly higher LD-index was found in the SD-group, suggesting a continuous cell degeneration.

Aged↗

Dementia in two histologically confirmed cases of multiple sclerosis: one case with isolated dementia and one case associated with psychiatric symptoms.

During the past 10 years, considerable attention has been devoted to cognitive impairment in multiple sclerosis. Occasionally this impairment may be so severe that multiple sclerosis presents as a dementia associated with only minor neurological signs and symptoms. The cases of two women affected by multiple sclerosis who presented with a pure dementia are reported. In the first patient, a progressive apragmatic behavioural disturbance with reduced short term memory and learning abilities were the main clinical features. Neuropathological examination of the brain disclosed numerous plaques in the periventricular white matter, with severe atrophy of the corpus callosum. Plaques were also seen in the white matter of both hippocampus and in the columns of the fornix. The impairment of short term memory could be linked to these lesions. Behavioural changes were probably related to the bilateral lesions of the long associative bundles that disconnected the frontal lobes from other parts of the cerebral hemispheres. In the second patient, visual hallucinations were associated with cognitive dysfunction. MRI showed large plaques in the white matter of both left frontal and temporal lobes. Smaller plaques were also present in the periventricular white matter of the occipital lobes, the nature of which were confirmed by a stereotactic biopsy.

Adult↗

Sensitivity of the dementia rating scale in vascular dementia: comparison between two sets of criteria to define cognitive impairment.

BACKGROUND: The Dementia Rating Scale (DRS) is a common measure of cognitive function, but its sensitivity to identify deficits across cognitive domains in vascular dementia (VaD) remains unclear. METHODS: We compared the sensitivity and specificity of two recommended cutoff scores of the DRS. Thirty-eight patients diagnosed with VaD participated in the current study. RESULTS: The original recommendations resulted in poor sensitivity for the DRS total score and attention, construction, and memory subscales. The more recent recommendations greatly improved the sensitivity of the subscales and the total DRS score, but resulted in decreased specificity. Correlations between the specific DRS subscales and criterion measures of cognitive function revealed good convergent and divergent validity for most subscales. CONCLUSIONS: The DRS is a valid measure of cognitive dysfunction in VaD, but clinicians should consider using the more recent recommendations developed for AD to determine impaired performances in VaD.

Aged↗

The Rapid Dementia Screening Test (RDST): a new economical tool for detecting possible patients with dementia.

The Rapid Dementia Screening Test (RDST) is a new psychometric screening tool to support the diagnosis of dementia. It includes two parts--a word generation task and a number transcoding task; it is short (taking approximately 3 min) and easy to administer, and it is well accepted by patients. After transformation of the raw scores in two age groups (under and over 60 years), the assessed cognitive abilities can be interpreted as age-appropriate or below average with good sensitivity and specificity, and subsequent diagnostic measures can be determined accordingly. The RDST is thus an economical tool for detecting demented patients by general practitioners.

Age Factors↗

Initial symptoms in frontotemporal dementia and semantic dementia compared with Alzheimer's disease.

BACKGROUND: Despite many reports about cognitive decline and behavioral changes in patients with frontotemporal lobar degeneration (FTLD), there have been very few systematic studies of initial symptoms of frontotemporal dementia (FTD) and semantic dementia (SD). OBJECTIVE: It was the aim of this study to investigate FTD and SD and to establish whether they are characterized by different initial symptoms. METHODS: Three groups of patients were studied: FTD (n = 36), SD (n = 17) and age-matched Alzheimer's disease (AD) patients (n = 52). Information on initial symptoms was obtained from caregivers. Symptoms were classified into 22 distinct categories from the following domains, based on previous studies of symptoms of FTLD: (1) change in social behavior, affection, and daily activities, (2) cognitive decline, (3) language impairments, and (4) other abnormal symptoms. RESULTS: Change in social behavior, affection, and daily activities was significantly more common in patients with FTD; on the other hand, language impairments were significantly more common in patients with SD as initial symptoms. Apathy and stereotypic behaviors were the most common initial symptoms among patients with FTD, while anomia, paraphasia, and impairment in word comprehension were the most common initial symptoms among patients with SD. Memory disturbance was the most common initial symptom among patients with AD. CONCLUSIONS: Behavioral and psychiatric symptoms are predominant initial symptoms in FTD, while language symptoms are predominant initial symptoms in SD. In addition to the assessment of current symptoms, the assessment of initial symptoms is useful for differential diagnosis in patients with FTD, SD and AD..

Aged↗

Effects of donepezil on central processing speed and attentional measures in Parkinson's disease with dementia and dementia with Lewy bodies.

BACKGROUND: We examined attention-enhancing effects of the cholinesterase inhibitor donepezil in Dementia with Lewy bodies (DLB) and Parkinson's disease with dementia (PDD) by means of open label study. METHODS: 22 DLBs and 23 PDDs were assessed over 20 weeks using the Cognitive Drug Research Computerized Attentional Tasks. We examined how much closer our patients moved towards being normal for their age by comparing them to a non-demented elderly control sample (n = 183, aged 71-75 years). RESULTS: Donepezil treatment improved power of attention, continuity of attention and reaction time variability. The deficit in responses was moved towards normal by 38 and 56% for power of attention and 22 and 10% for continuity of attention in PDD and DLB, respectively. CONCLUSIONS: Improvements in attention were found with donepezil in PDD and DLB.

Aged↗

Haptoglobin groups in dementia of Alzheimer type and multi-infarct dementia.

Haptoglobin (Hp) groups were determined in 65 patients with dementia of Alzheimer type (DAT) and 74 with multi-infarct dementia (MID). The increased Hp1 gene frequency among patients with DAT described by other investigators could not be confirmed in this study. The patients showed no significant difference from the controls with respect to phenotype and gene frequencies, and there were no significant differences between early and late onset cases of DAT.

Age Factors↗

Neuroimaging correlates of dementia rating scale performance at baseline and 12-month follow-up among patients with vascular dementia.

The authors previously reported that subcortical hyperintensity (SH) and whole-brain volume (WBV) each covary with different subscale scores of the Mattis Dementia Rating Scale (MDRS) among vascular dementia (VaD) patients. The present longitudinal analysis examined these relationships for change. The authors found that SH volume increased and WBV decreased significantly over 12 months. At baseline, SH volume accounted for significant variance in MDRS total score and every subscale score, except Memory. WBV was unrelated to any MDRS measure. After 12 months, SH volume was related only to the Construction subscale score, whereas WBV accounted for the majority of variance in Attention and Memory subscale performance. These findings indicate that although SH volume increases with disease progression, the relative impact of SH volume on cognitive status decreases among patients with advanced VaD.

Aged↗

Correlation of rCBF (SPECT), CSF tau, and cognitive function in patients with dementia of the Alzheimer's type, other types of dementia, and control subjects.

BACKGROUND: The diagnosis of Alzheimer's disease (AD) during life remains difficult and a definite diagnosis of AD relies on histopathological confirmation at post-mortem or by cerebral biopsy. It is well known that levels of tau proteins are consistently and significantly increased in the cerebrospinal fluid (CSF) of Alzheimer's patients versus levels in normal controls. However, the sole use of this biochemical marker as a test for AD is hampered by mediocre specificity, since tau concentrations may also be elevated in certain other neurological disorders (OND). Studies of the regional cerebral blood flow (rCBF) are widely performed because of their convenience and usefulness in a variety of neurological disorders. Most studies have reported high diagnostic accuracy for brain perfusion single-photon emission tomography (SPECT) in Alzheimer's disease. METHODS: In order to improve specificity, in this study, correlation of 99mTc-HMPAO SPECT scanning and CSF tau protein levels was made in 117 patients with AD, 67 patients with OND (26 of which had other dementias), and 23 age-matched controls. Means and standard deviations of tau protein levels were 297, 42 +/- 221, 12 in AD patients and 78, 07 +/- 98, 51 in patients with OND (p = 0.0006). No correlation was noted between CSF tau protein levels and age, duration of the disease, and neuropsychological scores of mini-mental state examination (MMSE), Cambridge Cognitive Examination (CAMCOG), and Functional Rating Scale for Symptoms of Dementia (FRSSD). FINDINGS: There was a bilateral parietal and temporal hypoperfusion in patients with AD in SPECT in comparison to normal subjects (p < 0.05) and there was a statistical correlation between this hypoperfusion and neuropsychological tests, such as MMSE and CAMCOG (p < 0.01). There was no correlation between tau protein levels and hypoperfusion in SPECT. INTERPRETATION: Conclusively, the correlation between elevated levels of tau proteins and hypoperfusion in SPECT in AD patients therefore cannot improve the specificity of tests in AD and this means that the determination of CSF tau proteins levels is not a specific diagnostic test for AD.

Aged↗