Alzheimer disease with cerebrovascular disease and vascular dementia: clinical features and course compared with Alzheimer disease.

OBJECTIVE: Vascular dementia (VaD) and Alzheimer disease with cerebrovascular disease (AD+CVD) are the leading causes of dementia after Alzheimer disease alone (AD). Little is known about the progression of either VaD or AD+CVD. The aim of this study was to compare demographic features, cognitive decline and survival of patients with VaD, AD+CVD and AD alone attending a memory clinic. METHODS: This study included 970 patients who were followed at the Lille-Bailleul memory clinic, France. Cognitive functions were measured with the Mini Mental State Examination (MMSE) and the Dementia Rating Scale (DRS). Survival rate was analysed with a left-truncated Cox model. Analyses were adjusted for age, sex, education, hypertension, diabetes and baseline MMSE and DRS. RESULTS: Of 970 patients, 141 had VaD, 663 AD alone and 166 AD+CVD. The latter were significantly older than AD or VaD patients at onset (71 (SD 7) vs 69 (9) and 68 (9) years, p = 0.01) and at first visit (75 (6) vs 73 (8) and 72 (8) years, p = 0.0002). Baseline MMSE and DRS evaluations were highest for VaD compared with AD alone or AD+CVD patients (p<0.006). Cognitive decline during follow-up was slowest for VaD, intermediate for AD+CVD and fastest for AD alone (p = 0.03). After adjustment, compared with AD patients, mortality risk was similar for those with VaD (relative mortality risk (RR) = 0.7 (0.5 to 1.1)) and tended to be lower for AD+CVD (RR = 0.7 (0.5 to 1.0)). The shorter the delay between first symptoms and first visit, the longer patients survived. CONCLUSION: This clinical cohort study shows that patients with VaD, AD+CVD and AD present different characteristics at baseline and during follow-up, and underlines the need to distinguish between them.

[Dementia following bipolar disorder]. / Syndrome démentiel dans les suites d'une bipolarité.

INTRODUCTION: Converging evidence suggests that people with bipolar disorder (BPD) exhibit persistent cognitive impairment independently from the emotional state. In old age BPD, the cognitive decline is more severe and can fulfill the criteria of dementia. However, the characteristics of bipolar disorder dementia are still unknown. AIM OF THE STUDY: The aim of the study was to characterise the cognitive and imaging profile of the dementia following bipolar disorder. METHOD: Patients fulfilling criteria of dementia and followed-up in the memory unit for at least two years were included. Patients with substance abuse were excluded. A battery of specific (assessing verbal memory, attention, frontal executive function, construction and visuospatial impairment), and global (MMSE and Mattis dementia rating scale) neuropsychological tests, behavioural assessment using the frontotemporal behavioural scale, MRI and HMPAO-SPECT imaging were performed in all patients during euthymic state. RESULTS: We included 13 patients with bipolar disorder (9W/4M). The mean age was 70.8 years (+/-7.7). Dementia began in average 29.2 years (+/-10.1) after the onset of the bipolar disorder. The mean score of MMSE was 24.0 (+/-4.3). The mean score of the Mattis dementia rating scale was 122.5 (+/-8.9). After an average of 6.1 years (+/-2.8) of follow-up, the mean score of MMSE was 23.5 (+/-3.2). The annual MMSE score decrease was of 0.5 (+/-4.4) per year. In more than 75% of the patients, Trail-Making Test-part B, Go-nogo test, Stroop test, delayed free recall in verbal explicit long-term memory test, category fluency tasks and code test were impaired. In more than 50% of patients, free recall, delayed cued recall, clock test, visuospatial battery and temporal orientation were impaired. On the other hand, spatial orientation and recognition were within the standards. The mean of the BREF score was 10.6 (+/-3.2). A moderate frontal behavioural syndrome was observed, but never persistent hallucinations. Seven patients had been treated with lithium and seven with antipsychotics, but none during the neurological assessment. Moderate extrapyramidal signs were reported in 10 patients, of which the seven patients treated in the past with antipsychotics. MRI showed no focal atrophy and no vascular lesions. Functional imaging conducted in 10 patients always showed uptake decrease in the frontotemporal regions and sometimes in the parietal region too. After six years of follow-up, no patient fulfilled the probable criteria for the main dementia, Alzheimer disease, vascular dementia, frontotemporal dementia and dementia with Lewy bodies. CONCLUSION: The data of this study support a possible specific dementia postbipolar disorder and not only mild cognitive decline. This hypothesis could be tested in a prospective study. Such dementia could be a main differential diagnosis from long lasting frontotemporal dementia. The pathogenic process of this dementia could also be determined.

Biochemical staging of synucleinopathy and amyloid deposition in dementia with Lewy bodies.

The primary feature of dementia with Lewy bodies (DLB) is the aggregation of alpha-synuclein into characteristic lesions: Lewy bodies (LBs) and Lewy neurites. However, in most of DLB cases, LBs are associated with neurofibrillary tangles and amyloid plaques (both Alzheimer disease [AD]-related lesions). We wanted to determine if this overlap of lesions is statistical, as a result of the late onset of both diseases, or results from a specific physiopathological synergy between synucleinopathy and either tauopathy or amyloid pathology. All patients with DLB from our prospective and multidisciplinary study were analyzed. These cases were compared with cases with pure AD and patients with Parkinson disease and controls. All cases were analyzed thoroughly at the neuropathologic and biochemical levels with a biochemical staging of aggregated alpha-synuclein, tau, and Abeta species. All sporadic cases of DLB were associated with abundant deposits of Abeta x-42 that were similar in quality and quantity to those of AD. Amyloid precursor protein (APP) dysfunction is a risk factor for AD as demonstrated by pathogenic mutations and Abeta accumulation. The constant and abundant Abeta x-42 deposition in sporadic DLB suggests that synucleinopathy is also promoted by APP dysfunction. Therefore, we conclude that APP is a therapeutic target for both AD and DLB.

[Treatment of dementia with Lewy bodies]. / Prise en charge thérapeutique de la démence à corps de Lewy.

Dementia with Lewy bodies (DLB) is known for its partial resistance and hypersensitivity to some treatments, but DLB is treatable with cholinesterase inhibitors, sometimes better than in Alzheimer's disease. Cholinesterase inhibitors have a symptomatic effect on cognition and behavior. Nevertheless, new antipsychotics are sometimes also useful to manage psychotic symptoms. Although DLB patients respond less well to levodopa than patients with Parkinson's disease, 75 percent of DLB patients improve with levodopa, which is the best-tolerated dopaminergic agent. Nonpharmacological strategies include speech therapy, physiotherapy, psychotherapy, and educational support groups for care givers.

The topography of cortical microbleeds in frontotemporal lobar degeneration: a post-mortem 7.0-tesla magnetic resonance study.

INTRODUCTION: Cerebrovascular lesions are rare in frontotemporal lobar degeneration (FTLD), in contrast to other neurodegenerative diseases. Cortical microbleeds (CoMBs) are frequent in Alzheimer's disease, in particular in cases associated with cerebral amyloid angiopathy. The present study investigates the gyral topographic distribution of CoMBs in post-mortem FTLD brains with 7.0-tesla magnetic resonance imaging. MATERIAL AND METHODS: The distribution of CoMBs in 11 post-mortem FTLD brains and in 12 control brains was compared on T2*-GRE MRI of six coronal sections of a cerebral hemisphere. The mean values of CoMBs were determined in twenty-two different gyri. The findings were correlated to those separately observed on neuropathological examination. RESULTS: As a whole there was a trend of more CoMBs in the prefrontal section of FTLD as well as of the control brains. CoMBs were significantly increased in the superior frontal gyrus and the insular cortex (p ≤ 0.001) and also in the inferior frontal gyrus and the superior temporal gyrus (p ≤ 0.01). CONCLUSIONS: CoMBs in FTLD are only increased in the regions mainly affected by the neurodegenerative lesions. They probably do not reflect additional cerebrovascular disease.

[Consensus statement on severe dementia]. / Consensus sur la démence de type Alzheimer au stade sévère.

Under the auspices of the French Society of Gerontology and Geriatrics, a multidisciplinary team including geriatritians, neurologists, epidemiologists, psychiatrists, pharmacologists and public health specialists developed a consensus on care for patients with severe dementia. They defined 21 recommendations for general practitioners, long-term care physicians and specialists based on knowledge available in 2005. At all stages of the disease, the objective of care is to improve as much as possible quality-of-life for the patient and his/her family, including a life project until the end of life. It is always possible to do something for these patients and their family: nutritional status, behavior disorders, and incapacities to deal with basic activities of daily life have to be taken in consideration. Resource allocation and proximity care have to be targeted. Research areas necessary to improve the care of patients with severe dementia has been selected.

[Consensus statement on severe dementia]. / Consensus sur la démence de type Alzheimer au stade sévère.

Under the auspices of the French Society of Gerontology and Geriatrics, a multidisciplinary group of experts, including geriatricians, neurologists, epidemiologists, psychiatrists, pharmacologists, and public health specialists developed consensus recommendations about care for patients with severe dementia. They defined 21 recommendations for general practitioners, long-term care physicians, and specialists, based on the knowledge currently available (2005). The aim of care at all stages is to mitigate the quality-of-life of patient, caregiver, and family insofar as possible, combining care and future planning until the end of life. Management, to take into account problems including nutritional status, behavior disorders, and ability (or inability) to perform activities of daily living, must be global, multidisciplinary, and coordinated and must optimize use of local medical and social resources. The group also stressed the importance of clinical research to improve knowledge of disease course and assess management strategies and recommended specific area for research.

Poststroke dementia: incidence and relationship to prestroke cognitive decline.

OBJECTIVE: To evaluate the 3-year incidence of poststroke dementia (PSD) and the influence of prestroke cognitive decline. METHODS: The authors evaluated prestroke cognitive functions in 202 consecutive stroke patients > or =40 years old using the Informant Questionnaire on Cognitive Decline in the Elderly (IQCODE), with a cut-off of 104 for the diagnosis of dementia. Six months and then annually after stroke, dementia was reassessed. The diagnosis of dementia was based on the International Classification of Diseases, 10th revision criteria in survivors who underwent a visit with a neurologist, or on the IQCODE score obtained by telephone contact with the family in survivors who did not. Statistics were performed using life-table methods. RESULTS: Thirty-three patients were excluded because of prestroke dementia. In the 169 remaining patients, the cumulative proportion of patients with dementia was 28.5% at the end of the follow-up period, with most of PSD occurring during the first 6 months. Using multivariate analysis, independent predictors of PSD were aging, preexisting cognitive decline, severity of deficit at admission, diabetes mellitus, and silent infarcts. Leukoaraiosis was an independent predictor of PSD when prestroke cognitive decline was not taken into account. The presumed etiology of dementia was vascular dementia (VaD) in two-thirds of patients and AD in one-third. CONCLUSIONS: The risk of PSD is high, and increased in patients with prestroke cognitive decline, with about one-third of patients meeting the criteria for AD and two-thirds meeting the criteria for VaD. These results confirm that, in stroke patients, an underlying degenerative pathology may play a role in the development of PSD.

Nonoverlapping but synergetic tau and APP pathologies in sporadic Alzheimer's disease.

OBJECTIVE: To determine the spatiotemporal mapping of tau pathologies and insoluble pools of Abeta in aging and sporadic AD, and their contribution to the physiopathologic, clinical, and neuropathologic features. METHODS: The authors studied 130 patients of various ages and different cognitive status, from nondemented controls (n = 60) to patients with severe definite AD (n = 70) who were followed prospectively. Insoluble Abeta 42 and 40 species were fully solubilized and quantified in the main neocortical areas, with a new procedure adapted to human brain tissue. Tau pathology staging was determined in 10 different brain areas, using Western blots. RESULTS: In AD, there is a constellation of amyloid phenotypes, extending from cases with exclusively aggregated Abeta 42 to cases with, in addition, large quantities of insoluble Abeta 40 species. Five other points were observed: 1) There was no spatial and temporal overlap in the distribution of these two insoluble Abeta species in cortical brain areas. 2) In contrast to solubilized Abeta 40 aggregates composed essentially of monomers and dimers, solubilized Abeta 42 was essentially observed as dimers and multimers. 3) Abeta 42 aggregates were observed at the early stages of tau pathology, whereas the insoluble Abeta 40 pool was found at the last stages. 4) During the progression of the disease, Abeta aggregates increase in quantity and heterogeneity, in close parallel to the extension of tau pathology. 5) There was no spatial overlap between Abeta aggregation that is widespread and heterogeneously distributed in cortical areas and tau pathology that is progressing sequentially, stereotypically, and hierarchically. CONCLUSIONS: These observations demonstrate that Abeta 42 aggregation, and not Abeta 40, is the marker that is close to Alzheimer etiology. It should be the main target for the early biological diagnosis of AD and modeling. Furthermore, the spatial mismatch between amyloid ss-precursor protein (APP) and tau pathologies in cortical brain areas demonstrates that neurodegeneration is not a direct consequence of extracellular Abeta neurotoxicity. Hence, there is a synergetic effect of APP dysfunction, revealed by Abeta aggregation, on the neuron-to-neuron propagation of tau pathology.

The biochemical pathway of neurofibrillary degeneration in aging and Alzheimer's disease.

OBJECTIVE: To determine the spatiotemporal mapping of neurofibrillary degeneration (NFD) in normal aging and the different stages of AD. BACKGROUND: The pathophysiologic significance of AD lesions, namely amyloid plaques and neurofibrillary tangles, is still unclear, especially their interrelationship and their link with cognitive impairment. METHODS: The study included 130 patients of various ages and different cognitive statuses, from nondemented control subjects (n = 60, prospective study) to patients with severe definite AD. Paired helical filaments (PHF)-tau and Abeta were used as biochemical and histologic markers of NFD and amyloid plaques, respectively. RESULTS: NFD with PHF-tau was systematically present in variable amounts in the hippocampal region of nondemented patients age >75 years. When NFD was found in other brain areas, it was always along a stereotyped, sequential, hierarchical pathway. The progression was categorized into 10 stages according to the brain regions affected: transentorhinal cortex (S1), entorhinal (S2), hippocampus (S3), anterior temporal cortex (S4), inferior temporal cortex (S5), medium temporal cortex (S6), polymodal association areas (prefrontal, parietal inferior, temporal superior) (S7), unimodal areas (S8), primary motor (S9a) or sensory (S9b, S9c) areas, and all neocortical areas (S10). Up to stage 6, the disease could be asymptomatic. In all cases studied here, stage 7 individuals with two polymodal association areas affected by tau pathologic states were cognitively impaired. CONCLUSIONS: The relationship between NFD and Alzheimer-type dementia, and the criteria for a biochemical diagnosis of AD, are documented, and an association between AD and the extent of NFD in defined brain areas is shown.

Behavioral effects of trazodone in Alzheimer's disease.

BACKGROUND: Central serotonin depletion may contribute to the anxiety, restlessness, irritability, and affective disturbance seen in a variety of psychiatric conditions, particularly dementia of the Alzheimer's type (DAT) in which brain concentrations of both 5-hydroxytryptamine (5-HT) and its 5-hydroxyindoleacetic acid (5-HIAA) metabolite are reduced. METHOD: Trazodone, a serotonergic antidepressant with alpha 2-adrenergic blocking activity, was administered to 13 patients with DAT in an open 10-week pilot study at a dose of 25 mg t.i.d. Behavioral and affective disturbance was assessed pretreatment and posttreatment using semistructured interview and Jouvent's Depressed Mood and Gottfries-Brane-Steen scales. RESULTS: Irritability, anxiety, restlessness, and affective disturbance were all decreased (p < .05). No side effects were observed. Mean Mini Mental State scores were unaffected by treatment. CONCLUSION: The hypothesis that trazodone corrects behavioral and affective disturbance induced by serotonin depletion in DAT requires confirmation in a double-blind placebo-controlled trial.

Medial temporal lobe atrophy in memory disorders.

Medial temporal lobe atrophy determined by temporal lobe oriented computed tomography (CT), 1 year before death, is strongly associated with histopathologically confirmed Alzheimer's disease (AD). The aim of this study was to assess the diagnostic accuracy of medial temporal lobe measurement for the diagnosis of AD in patients referred to a memory disorders clinic, especially those at an early stage of the disease. CT oriented to the temporal lobe was performed in 333 subjects aged 41-93 years consecutively recruited in a Memory Disorders Clinic: 124 had probable AD, Mini Mental State score (MMS) = 17 (8); 50 possible AD [MMS = 21 (5)]; and 119 patients had miscellaneous memory disorders [MMS = 22 (7): frontotemporal lobe dementia, subcortical dementia, cortical Lewy body disease, vascular dementia, Korsakoff syndrome, focal atrophy, etc.]. There were also 19 anxious/depressed patients [MMS = 29 (1)] with normal performance on memory tests, and 21 controls. The minimum width of the medial temporal lobe was measured. The best cut-off to distinguish AD patients from non-AD patients was 11.5 mm, in agreement with data in the literature. At this threshold, 84% of probable AD patients had a positive test and 90% of controls and anxious/depressed patients had a negative test. For the diagnosis of probable AD, sensitivity of the measurement was 0.81, specificity 0.95, predictive positive value 0.99, predictive negative value 0.45, and diagnostic accuracy 0.83. The test was positive in half the possible AD patients, and half those with miscellaneous memory disorders. It was negative in all anxious/depressed patients. Therefore, temporal lobe oriented CT might be a valuable tool for assessment of medial temporal lobe atrophy in AD routine practice.

Cognitive and psychiatric impairment in herpes simplex virus encephalitis suggest involvement of the amygdalo-frontal pathways.

The long-term neuropsychological and psychiatric sequelae of herpes simplex virus encephalitis (HSVE) and their relationship to the volume of temporal lesions and to amygdala and hippocampus damage remain undefined. We have conducted a prospective study of long-term sequelae in 11 patients with clinically presumed HSVE and detection of HSV DNA in the cerebrospinal fluid by polymerase chain reaction. Six months after encephalitis, patients underwent neuropsychological and language assessment. At the same stage, single photon emission computed tomography (SPECT) evaluated the occurrence of hypoperfusion with an index of asymmetry. MRI was used for the measurement of amygdala, hippocampus and cerebral lesions by two blind neurologists. The volume of the amygdala and hippocampus was compared with those of five controls, matched for age and level of education. Long-term memory disorders were seen in 6 patients, associated with the larger lesions and damage of at least two structures. Long-term behavioural changes with emotionalism, irritability, anxiety or depression were prominent in 7. Left prefrontal hypoperfusion appeared in 8 patients, associated with psychiatric disorders in 7 and left amygdala damage in 6. The reduction of amygdala and hippocampus volume was correlated with the overall volume of lesions. Different patterns of mesial temporal lobe damage occurred, involving either amygdala alone, or amygdala and hippocampus, but never hippocampus alone. MRI volumetric measurements in HSVE could be a good indicator of long-term prognosis. Persistant behavioural changes could be related to an amygdala and frontal dysfunction.

Generation effect in short-term verbal and visuospatial memory: comparisons between dementia of Alzheimer type and dementia of frontal lobe type.

Memory deficit is the predominant presenting symptom in dementia. To compare short-term memory (STM) deficit in early dementia of Alzheimer type (DAT) vs frontal lobe type (DFT), and determine the residual memory capacity for stimulation, the generation effect (the memory advantage of items generated rather than read) was tested on verbal and visuospatial STM in patients with DAT (n = 10), DFT (n = 9) and in age-matched normal controls (n = 12). The generation effect enhanced performance in all groups. However, the profile of STM deficit differed in the two dementias: verbal and visuospatial memory were both decreased in DAT vs verbal memory only in DFT. These results provide a further criterion for differentiating between DAT and DFT, and show that memory performance can be enhanced in early dementia using techniques such as the generation effect.

Sundowning syndrome in demented patients without neuroleptic therapy.

Characterisation of sundowning syndrome, defined as 'an exacerbation of symptoms indicating increased arousal or impairment in late afternoon, evening or at night, among elderly demented individuals', is complicated by neuroleptic therapy and frequent failure to specify the nature of the associated dementia. Screening by a memory disorders unit of an institutionalized population of 30 neuroleptic-free demented patients revealed 8 sundowners, with diagnoses of probable Alzheimer's disease (n = 5), frontal lobe dementia (n = 1), Lewy body disease (n = 1), and sequelae of herpes encephalitis (n = 1). Sundowners did not differ from non-sundowners in age, Mini Mental State score, degree of temporal and spatial disorientation or perceptual delusion. Sundowning was related to restlessness (P < 0.0001), sleep disorder (P < 0.003) and a history of hypotension lipothymia (P < 0.08). These results provide further evidence for a chronobiological explanation of sundowning syndrome.

[Organization of memory centers and prospects]. / Organisation des centres de la mémoire et perspectives.

The concept of memory centers is based on a multidisciplinary approach to memory disorders and dementia, especially Alzheimer's disease, a public health challenge. The first memory centers were established in the 80s in the US and the British Isles. The aims of these centers are to make a diagnosis (and to reassure the 'worried well'), to detect dementia, to provide a service for their management, to educate care takers, to evaluate new therapies, and to contribute to clinical and basic research. Follow-up is crucial. The first memory centers all experienced long delay to diagnosis of dementia and found that Alzheimer's disease was the first cause of consultation. These observations led to the creation of such centers in many countries. A survey of 20 French memory centers defined the "ideal memory center": an identified structure with a clinic and a day-care unit for diagnosis and follow-up, with neurological, psychological, psychiatric, and geriatric skills. It must be part of a medical and social network for the management of dementia and participate in a network of memory centers at the regional and national level. Relationships between dementia and somatic diseases, frequent in demented patients, are still poorly known. Dementia interferes with the clinical expression, the management, and the prognosis of somatic diseases, some of which, such as cardiovascular conditions, are possibly linked with dementia. Conversely, somatic diseases may rapidly worsen the cognitive state and induce delirium, leading to hospitalization. Medical wards are not all appropriate. Memory centers must also be involved in these care, educational and research issues.

[Management of behavioral disorders in dementia patients]. / Prise en charge des troubles du comportement dans les démences.

Behavioral disorders are major manifestations of Alzheimer's disease and other forms of dementia. They are associated with caregiver distress, increase the likelihood of institutionalization and may be associated with more rapid cognitive decline. The first step of treatment strategy is an assessment of these disorders. Treatment of behavioral signs is an etiological treatment. Acute behavioral signs are often related to an unknown somatic disease. Chronic signs are often symptoms of the neurological dementia and can be reduced, especially by serotonergic agents and anticonvulsivants. The new antipsychotics are a good alternative to classic neuroleptics known for their frequent cognitive side effects in demented patients. Anticholinesterasic drugs can positively influence noncognitive signs. The treatment of behavioral and psychological symptoms of dementia (BPSD) involves a number of specific interventions including cognitive stimulation which has shown effectiveness on both cognitive functions and quality of life. Prevention of BPSD includes safety measures such as evaluation of suicidality and violence, vigilance regarding neglect and abuse, planning for legal issues due to the patient's incapacity. Families or caregivers should be provided with counseling, education and support. The treatment of BPSD is part of a global and multimodal care which involves general practioners, nurses, social workers, physiotherapists, neuropsychologists, speech therapists, memory centers, psychogeriatric and geriatric units, and respite care units, nursing homes and long-term care facilities. The coordination of the professionals is a critical aspect of providing effective care for patients with Alzheimer's disease.

[Clinical diagnosis of frontotemporal dementia]. / Diagnostic clinique des démences fronto-temporales.

Frontotemporal dementia (FTD) is poorly recognized clinically. Of the 1,517 patients examined at the Lille Outpatients Memory Clinic data bank (1991-1995), 74 fulfilled the criteria of the Lund and Manchester groups for FTD. They accounted for 5 p, 100 of all patients, 1 for 10 probable or possible Alzheimer's disease. Mean patient age was 63 years, duration of the disease was 5 years, mean Mini Mental State score was 23; 45 p. 100 belonged to the active population. Behavioral disorders occurred before the cognitive decline and remained the major feature. All patients had at least 3 of the following symptoms: self-control impairment, affective disorder, loss of interest and self-neglect. Memory impairment consisted of correct encoding and impaired retrieval processes, without major storage impairment. No patients had spatial disorientation. Language was usually reduced, EEG was normal. Two diagnoses were confirmed by autopsy: both consisted of aspecific frontal and temporal degeneration, 41 p. 100 of the patients were referred by a general practitioner, 30 p. 100 by a psychiatrist, 16 p. 100 by a neurologist, 2 p. 100 by other specialists, and 11 p. 100 following the advice of their relatives. FTD had never been suspected. Alzheimer's disease or non specified degenerative dementia was suspected in 2/3 of patients and a psychiatric disorder in 1/3. With the advent of novel pharmacological agents for the treatment of dementing disorders and for research purposes, the identification and accurate differentiation of FTD from Alzheimer's disease and psychiatric disorder is essential. Therefore, the role of multidisciplinary memory clinic is crucial to differentiate FTD from other degenerative dementias.

[Lewy body dementia]. / Démence avec corps de Lewy.

Dementia with Lewy Bodies (DLB) is an entity which now fulfils clinical and neuropathological criteria according to international consensus guidelines (McKeith et al., 1996). It is now possible, in clinical practice, to consider the diagnosis of DLB from the beginning of the management of a demented patient. The clinical, diagnostic and therapeutic aspects of DLB are investigated in a prospective manner. Visual hallucinations, fluctuations and extrapyramidal symptoms seem to have, in association with the progressive cognitive decline, some particularities. However their specificity should be correlated with neuropathological data. Other symptoms, such as repeated falls or syncope, neuroleptic sensitivity, systematized delusions or other modalities of hallucinations, are probably additional arguments giving more predictive value to the association of the major symptoms. The role of neuropsychological patterns at the beginning of the cognitive decline is shown. There are some recent concordant results of functional imaging in DLB. The neuropathological aspects of DLB and the links with Parkinson pathology and especially Alzheimer pathology are emphasized.