What are the preferences of patients attending a memory clinic for disclosure of Alzheimer's disease?

BACKGROUND/AIMS: This report shares and discusses the collected personal preferences of patients attending a memory clinic for disclosure of a potential Alzheimer's disease (AD) diagnosis. METHODS: In this prospective study of outpatients attending a single memory clinic over a 6-year period (March 2004-October 2010), doctors collected their patients' wishes (willingness to be informed, motivation, presence of the family) through a standardized procedure. RESULTS: Of the 1005 patients questioned throughout the study period-with a final diagnosis of dementia for 480 of them-858 (85.3%) wished to be informed of an AD diagnosis, whereas 72 (7.2%) did not and 75 (7.5%) were not sure. Older age and reduced cognitive functioning were independently associated with a preference to not be informed of a potential AD diagnosis. CONCLUSION: Our study provides evidence of the willingness of most patients to know the truth vis-à-vis AD and also offers some insight into their motivations.

[Increase of serum procalcitonin levels during a neuroleptic malignant syndrome]. / Augmentation de la concentration sérique de la procalcitonine au cours d'un syndrome malin des neuroleptiques.

When fever occurs in a patient treated with a neuroleptic, the diagnosis of a neuroleptic malignant syndrome is difficult to differentiate to that of an infectious event. Among inflammation biomarkers of inflammation, serum procalcitonin levels increase both quickly and specifically during a bacterial infection. We report the first case of a neuroleptic malignant syndrome associated with a significant increase of serum procalcitonin levels, without concomitant septic syndrome. The neuroleptic malignant syndrome might be a non-infectious clinical situation associated with an increased serum procalcitonin concentration.

Early detection of patients in the pre demented stage of Alzheimer's disease: the Pre-Al Study.

OBJECTIVES: The aim of the Pre-Al study is to evaluate and compare the predictive value of different tools for an early identification of Alzheimer's disease. DESIGN AND PARTICIPANTS: Patients coming for consultation to memory clinics without dementia were included if they had an objective memory or attention trouble assessed by a MMSE score > 25 (with at least one missing item at the words recall) and / or an Isaac set test score < 28. All were examined by a neuropsychological battery (Free and Cued Selective Reminding Test, digit ordering test, WAIS-R digit symbol, Trail making test, Benton visual retention test, verbal fluency, confrontation naming and Baddeley's double task test). A subpopulation received an MRI and SPECT assessment. RESULTS AND DISCUSSION: 251 patients were included (mean age: 72.0 years; mean education duration: 10.9 years). Validation of the predictive tests will be based on the comparison of these tests in patients developing dementia and others, after a follow-up of at least 3 years. This paper presents methodology of the study and the population description.

[Hallucinations and dementia. Prevalence, clinical presentation and pathophysiology]. / Hallucinations et démences. Prévalence, sémiologie et pathophysiologie.

Hallucinations are a common feature of certain degenerative diseases with a risk of dementia such as Alzheimer's disease, Lewy body dementia, and Parkinson's disease. Obtaining valid epidemiological data is nevertheless quite difficult because of methodological problems. As a rule, hallucinations are more prevalent in Lewy body disease than Parkinson's disease or Alzheimer's disease. The prevalence in parkinsonian dementia is about the same as in Lewy body disease. Complex visual hallucinations predominate, auditory or tactile hallucinations are more exceptional. Minor forms (illusions, sensation of presence) are also observed. Recurrence is common, mainly in the evening or at night. Patients with advanced mental impairment generally take the hallucinations for reality. The hallucinations can be associated with psychological and behavioral disorders such as delusionnal idea or identification disorders. It is important to search for other causes of hallucinations such as drugs, ocular disorders, or depression, but many of these disorders are common comorbidities in elderly patients with degenerative disease. There is no unique model fitting all the hypothesized pathogenic mechanisms. Complex visual hallucinations most likely arise from abnormal activation of the extra-striat temporal associative regions, but only hypothetical mechanisms have been proposed. Genetic studies and functional imaging have not provided convincing evidence. Current focus is placed on an imbalance between deficient cholinergic transmission and preserved or augmented monoaminergic transmission at the cortical level, but other neurotransmission systems could be involved. The dream dysregulation mechanism proposed in Parkinson's disease cannot be generalized. The link between cognitive disorders and hallucination is also poorly understood: hallucinations are associated with more severe cognitive impairments or more rapid cognitive deline in Parkinson's disease and Alzheimer's disease, but the association with specific cognitive disorders remains to be fully explored.

[Memory clinics: value and limitations]. / Les consultations de la mémoire. Intérêts et limites.

CONCEPT: Memory clinics have developed since the early 1980s. Their main objective is the diagnosis of dementia for early multiple disciplinary management. INTEREST: Memory clinics serve an expert center, allowing early diagnosis in the predementia phase and a precise diagnosis of the cause. Memory clinics have revealed the frequency of psychiatric disorders, particularly depression and anxiety, leading to memory complaints. They provide a significant contribution for follow-up of dementia patients, announcement of the diagnosis, initiation of new treatments, prevention of "emergency hospitalizations" as well as postponement or preparation of institutionalization. They also have an educative role, promoting better use of psychotropics and a better understanding of management approaches and incidents observed during the disease course. Clinical research, which is not always sufficiently supported, is one of their important responsibilities. LIMITATIONS: The basic limitation for memory centers is the problem of the availability of qualified personnel. The lack of sufficient interest in truly early screening for Alzheimer's disease remains a problem to be solved.

Hallucinations in Parkinson's disease: prevalence, phenomenology and risk factors.

Hallucinations, mainly of a visual nature, are considered to affect about one-quarter of patients with Parkinson's disease. They are commonly viewed as a side-effect of antiparkinsonian treatment, but other factors may be involved. The aim of this study was to determine the phenomenology, prevalence and risk factors of hallucinations in Parkinson's disease. Two-hundred and sixteen consecutive patients fulfilling clinical criteria for Parkinson's disease were studied. Demographic and clinical variables were recorded, including motor and cognitive status, depressive symptoms and sleep-wake disturbances. Patients with and without hallucinations were compared using non-parametric tests, and logistic regression was applied to significant data. Hallucinations had been present during the previous 3 months in 39.8% of the patients, and fell into three categories: minor forms, consisting of a sensation of a presence (person), a sideways passage (commonly of an animal) or illusions were present in 25.5% of the patients (an isolated occurrence in 14.3%), formed visual hallucinations were present in 22.2% (isolated in 9.3%) and auditory hallucinations were present in 9.7% (isolated in 2.3%). Patients with minor hallucinations had a higher depression score than non-hallucinators but did not differ in other respects. Logistic regression analysis identified three factors independently predictive of formed visual hallucinations: severe cognitive disorders, daytime somnolence and a long duration of Parkinson's disease. These findings indicate that, when minor hallucinations are included, the total prevalence is much higher than previously reported. A simple side-effect of dopaminergic treatment is not sufficient to explain the occurrence of all visual hallucinations. The main risk factor in treated patients is cognitive impairment, although sleep-wake cycle disturbances, and possibly other factors related to the duration of the disease, act as cofactors.

Neuropsychological prediction of dementia in Parkinson's disease.

OBJECTIVE: To identify neuropsychological characteristics predictive of later dementia in Parkinson's disease. METHODS: A comprehensive neuropsychological test battery was administered to a cohort of 89 initially non-demented patients with Parkinson's disease consecutively enrolled at a specialised Parkinson's disease clinic. They were reassessed after a mean of 3.5 years for the diagnosis of dementia. The Cox proportional hazards model was used to identify baseline characteristics predictive of dementia. RESULTS: Only four of the baseline clinical characteristics of Parkinson's disease and neuropsychological variables remained independently linked to subsequent development of dementia: the age of onset of Parkinson's disease (>60 years; relative risk (RR) 4.1, 95% confidence interval (95% CI) 1.8-24.0, p<0.03), the picture completion subtest of the Wechsler adult intelligence scale (score<10; RR 4.9, 95% CI 1.0-24.1, p<0.02), the interference section of the Stroop test (score<21; RR 3.8, p=0.08), and a verbal fluency task (score<9; RR 2.7, 95% CI 0.8-9.1, p=0.09). Depressive symptoms and the severity of motor impairment were not predictive of dementia. CONCLUSION: These features are different from the neuropsychological characteristics predictive of Alzheimer's dementia in healthy elderly people (mainly memory and language performance). They are in keeping with the well known specificity of the impairments in Parkinson's disease for visuospatial abilities and difficulties in inhibiting irrelevant stimuli. It is postulated that the composite nature of the picture completion subtest, involving several cognitive abilities impaired in Parkinson's disease, explains its sensitivity.

Mapping of a complicated familial spastic paraplegia to locus SPG4 on chromosome 2p.

Autosomal dominant familial spastic paraplegia (AD-FSP) is a degenerative disorder of the central motor system characterised by progressive spasticity of the lower limbs. AD-FSP has been divided into pure and complicated forms. Pure AD-FSP is genetically heterogeneous; three loci have been mapped to chromosomes 14q (SPG3), 2p (SPG4), and 15q (SPG6), whereas no loci responsible for complicated forms have been identified to date. Here we report linkage to the SPG4 locus in a three generation family with AD-FSP complicated by dementia and epilepsy. Assuming that both forms of AD-FSP are caused by mutations involving the same FSP gene, analysis of recombination events in this family positions the SPG4 gene within a 0 cM interval flanked by loci D2S2255 and D2S2347.

[Memory clinics: long-term follow-up]. / Consultations de mémoire: suivi à long-terme.

Follow-up of patients in memory units raises problems of structure, team, diseases, evaluation and organization. Structures could be within the hospital, for early diagnosis, or outside hospital for prevention. The medical team is organized around the neurologist, must integrate different medical specialists (neurologist, psychiatrist, geriatrician) and be composed minimally of a clinician and a psychologist. Diseases range from memory complaint to very serious diseases such as Alzheimer's, vascular or post-traumatic dementia. Therapy includes cognitive stimulation and drug clinical trials. Evaluation requires medico-psycho-social analysis of the patient and his/her family. Memory consulting units could be integrated in a caring network organised in a modular fashion or like a geriatric hospital.

[Neuropsychological testing of 5 patients with Parkinson's disease before and after neuron graft]. / Examen neuropsychologique de 5 patients Parkinsoniens avant et après greffe neuronale.

Five patients with idiopathic PD were followed by neuropsychological tests after brain fetal neuronal transplantation. The following tests were used in order to assess memory as well as visuospatial and frontal functions: MMSE, Mattis Scale, Wisconsin Card Sorting Test, Stroop task, word fluency tasks, 15-objects test, WAIS-R (Digit span, Arithmetic, Block design, Pictures completion, Pictures arrangement), learning of 15 words of Rey, WMS-R (Logical memory) and Visual memory of L. Israël. The same tests were performed before, then one year following the transplantation. Pooled data did not show any significant difference between pre and post-operative tests. Individual results varied among patients: 2 remained unchanged, 1 had a pathological deterioration which increased after one year, 1 had some frontal symptoms whereas the last patient improved. Our data confirm that this surgical procedure do not induce permanent neuropsychological deficits, but do not indicate at the present time any clear effect of dopamine reinnervation on cognitive functions.

[Isoform 4 of apolipoprotein E and Alzheimer disease. Specificity and clinical study]. / Isoforme 4 de l'apolipoprotéine E et maladie d'Alzheimer. Spécificité et étude clinique.

We studied apolipoprotein E (apoE) phenotype in 113 patients with possible and probable Alzheimer's disease (AD), 49 patients with Parkinson's disease (including 11 patients with dementia) and 23 patients with mixed and vascular dementia. Normal controls were 498 young, healthy blood donors previously recorded. All patients were assayed for blood lipid parameters. All AD patients underwent a neuropsychological evaluation (including a mini-mental status and 5 subtests of Cole and Dastoor hierarchic dementia scale) and a detailed interrogation of them and their caregivers about their familial and personal medical history. The recorded data included age at onset, clinical subtype (i.e. amnesic or aphaso-apraxic), occurrence of fits, cases of probable dementia in relatives, and ages of their parents at death. There was a significant association between the fourth isoform of apoE and AD, as in previous works. We did not found such an association for PD patients (even with dementia) nor mixed and vascular demented patients. We failed to find any association between any clinical characteristic of the patients and the biological subgroups defined by the number of epsilon 4 alleles, except with regard for the age of onset. Surprisingly, the mothers of epsilon 4 bearers had a significantly longer life than mothers of other patients. We failed to found any significant difference of apoE2 isoform frequency between AD patients and controls. AD patients had higher levels of cholesterol and apoAl than did MP and mixed and vascular demented patients. ApoAl level is known to constitute a protective factor against coronary heart disease, which is usually increased by the presence of apoE-epsilon 4.(ABSTRACT TRUNCATED AT 250 WORDS)

Mini-Mental Parkinson: first validation study of a new bedside test constructed for Parkinson's disease.

We have developed a brief screening test aimed at identifying cognitive disorders in Parkinson's disease. The Mini-Mental Parkinson derives from the Mini-Mental State Examination of Folstein. It includes seven ordered subsections, with a total score of 32. A pilot study was conducted in 50 community-dwelling parkinsonian patients, in order to establish its metrological qualities. Comparisons were made with a neuropsychological battery including several tests widely used in the assessment of specific cognitive disorders in Parkinson's disease. The correlations between the Mini-Mental Parkinson and each component of this battery were substantial, especially for the performance subtests of the WAIS-R (r = 0.62 to 0.72), the Stroop test (r = 0.65) the 15-objects test (r = 0.64), the word fluency (r = 0.63) and the Odd Man Out test (r = 0.61). The validity of each subtest of the Mini-Mental Parkinson was adequate except for one, based on a word choice, which requires a modification in French before definitive use. The test-retest reliability was high (r = 0.84). There was a significant difference in the mean scores in cases with confusional event (22.4), even without current signs of dementia, compared with patients with no such history (27.2). In conclusion, this brief test is suitable for assessment of parkinsonian patients.

A prospective study of patients with CT detected pallidal calcifications.

In a prospective study pallidal calcification was detected in 30 of 1478 (2%) adult patients, on CT brain scans. In 8 cases (26%), the calcifications were detected either years after, or during the course of, conditions known to cause basal ganglia calcification, including AIDS in four cases. Eight patients (three with AIDS) had disturbances of calcium and phosphorus metabolism. It was concluded that: a) pallidal calcification is not uncommon and aetiological factors may be recognised more often than previously reported; b) AIDS is emerging as a significant cause of pallidal calcification in young adults, and c) in AIDS and other conditions, abnormal calcium and phosphate metabolism may act in conjunction with local vascular changes.

Prevalence of apolipoprotein E phenotypes in ischemic cerebrovascular disease. A case-control study.

BACKGROUND AND PURPOSE: Apolipoprotein E polymorphism may influence the early development of coronary artery disease. We investigated the putative role of apolipoprotein E phenotypes in cerebral infarction. METHODS: The apolipoprotein E phenotypes of 69 patients (mean +/- SD age, 72 +/- 11 years) who had suffered completed stroke or a transient ischemic attack and 68 sex- and age-matched control subjects free of cerebrovascular disease were determined by isoelectric focusing. The relative frequency of the apolipoprotein E phenotypes in the general population was estimated in 498 healthy blood donors (mean age, 37 years). RESULTS: The prevalences of hypertension, diabetes mellitus, obesity, and intermittent claudication were significantly higher in patients than in control subjects. Serum lipid and apolipoprotein B concentrations and the composition of very low density lipoproteins were not significantly different between patients and control subjects. Apolipoprotein A-I and E levels were significantly lower in patients. Cholesterol levels were higher in male patients than in male control subjects (5.10 +/- 1.46 versus 4.41 +/- 0.80 mmol/L; p = 0.036), and the ratio of apolipoprotein A-I to B was lower (0.77 +/- 0.29 versus 1.03 +/- 0.37; p < 0.001). The E3/E3 phenotype was more frequent in control subjects (85%) than in patients (72.5%; p < 0.05) and healthy blood donors (64%; p < 0.02). The E3/E2 phenotype was more frequent in patients (10.1%) than in control subjects (1.4%; p < 0.05). A stepwise logistic regression showed that the presence of stroke was significantly related to high blood pressure (p < 0.0001), low apo E levels (p < 0.008), obesity (p < 0.041), the apo E phenotype (p < 0.05), and diabetes mellitus (p < 0.05). CONCLUSIONS: The E3/E3 phenotype may protect against early vascular morbidity, and the epsilon 2 gene may be a risk factor for cerebrovascular morbidity, possibly related to diabetes, hypertension, and/or obesity.

[Specialized memory consultation. Value and evaluation after 2 years of functioning]. / Consultation spécialisée de la mémoire. Intérêt et bilan de 2 années de fonctionnement.

Disorders of memory are a frequent cause of consultation and are observed in dementias, in some depressive syndromes and in normal ageing. The specialized memory consultation is based on two successive examinations: one by a neurologist, the other by a psychologist, using standardized batteries of tests. In 100 consecutive subjects, 3 main groups of about 20 to 30 individuals each could be identified: dementia syndromes, psychiatric disorders and age-related disorders of memory. The remaining subjects had various diseases. In subjects with memory complaints a psychometric evaluation performed by a team of specialists seems to be the only means of refining the diagnosis enough for a personalized management. In addition, the specialized consultation team acquires the knowledge that will help it, in the future, to lay down the bases of medical prevention of pathological cerebral ageing.