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.

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.

[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.

Rilmenidine and vigilance. Review of clinical studies.

In this study, the possible effects of rilmenidine on vigilance are evaluated. Sedation is the most disturbing side effect of alpha 2-agonists, especially during the first weeks of treatment. The level of vigilance was first determined by assessing drowsiness using visual analogue scales and/or by several psychometric tests in four pharmacoclinical studies in healthy subjects or in hypertensive patients: three studies with single administration of rilmenidine (0.5 to 3.0 mg) and one study with repeated administration for three days. These studies were double-blind, Latin-square designed, and controlled versus placebo (in all studies) and versus clonidine (in three studies). Analysis of these results illustrated that after short-term and repeated administration: (1) the effects on vigilance observed with rilmenidine 1 mg did not differ statistically from data observed with placebo; and (2) sedative effects observed with clonidine were significantly greater than with rilmenidine, at equihypotensive doses. Daytime drowsiness was systematically assessed and graded by inciting questioning at each visit in five clinical studies. Ambulatory hypertensive patients were treated with rilmenidine (1 mg per day or 1 mg twice a day). These studies were controlled versus placebo (one study for two weeks, 120 patients; and one study for one month, 126 patients), hydrochlorothiazide (six weeks, 56 patients), clonidine (six weeks, 333 patients), and methyldopa (three months, 157 patients). The results showed that: (1) drowsiness observed with rilmenidine did not differ statistically from that observed with placebo or diuretic; and (2) drowsiness occurred less frequently with rilmenidine than with reference alpha 2-agonists at equihypotensive doses. In conclusion, these results confirm in current clinical use the dissociation already observed in laboratory animals between the antihypertensive effects and the sedative effects and may distinguish rilmenidine among alpha 2-agonists.

Efficacy and safety of rilmenidine for arterial hypertension.

To assess the long-term acceptability and efficacy of rilmenidine (S 3341), patients with placebo-resistant hypertension (diastolic blood pressure [BP] greater than or equal to 95 mm Hg and less than 115 mm Hg) were included in an open 1-year treatment study. Eight examinations allowed treatment adaptation if diastolic BP remained greater than or equal to 90 mm Hg (monotherapy with rilmenidine, 1 or 2 mg/day, followed by the addition of a diuretic, then tritherapy). Three hundred seventeen patients, aged 58.0 +/- 0.7 years, were included. Two hundred sixty-nine were followed for 1 year and 48 withdrew from the trial without any symptom suggesting a withdrawal syndrome: 4 because of adverse effects; 6, lack of efficacy despite triple therapy; 9, intercurrent diseases; 10, noncompliance independent of adverse effects; 18, personal reasons not associated with treatment; and 1, lost to follow-up. On the 12th month, the decrease in supine systolic and diastolic BP reached 25 and 17 mm Hg with monotherapy (n = 150), 26 and 17 mm Hg with double therapy (n = 90) and 20 and 15 mm Hg with triple therapy (n = 29). BP was normalized (diastolic BP less than or equal to 90 mm Hg) on months 6 and 12 in 80 and 84% of the patients, respectively. Monotherapy was maintained in 66 and 60% of these patients, respectively, two-thirds being treated with 1 mg once daily. Adverse effects with monotherapy were mainly observed at the beginning of treatment in 3 to 8%: dry mouth, asthenia, gastralgia, palpitations, drowsiness, insomnia; other adverse effects were rare (1 to 2%).(ABSTRACT TRUNCATED AT 250 WORDS)

Ischaemic myelopathy secondary to disseminated intravascular coagulation in AIDS.

A 39-year-old patient with AIDS presented with a rapidly progressive myelopathy with a partial Brown-Séquard syndrome. He died, 9 weeks after onset of the first neurological signs, from diffuse encephalopathy. Neuropathological examination revealed multiple, usually small, frequently haemorrhagic, infarcts or various ages and numerous fibrin thrombi in medium and small penetrating vessels and capillaries of the brain and spinal cord, characteristic of disseminated intravascular coagulation. There were no inflammatory changes. Immunohistochemical studies for human immunodeficiency virus, cytomegalovirus, varicella zoster virus, herpes simplex virus type 1 and type 2 were negative. Ischaemic spinal cord lesions due to disseminated intravascular coagulation may represent an unusual cause of focal, non-inflammatory, non-tumoral, myelopathic syndrome in AIDS.

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.

[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.

[Cognitive deficits in Parkinson's disease]. / Les altérations cognitives au cours de la maladie de Parkinson.

No clear general view has emerged from the many recent studies devoted to cognitive disorders in Parkinson's disease. Disparity of the populations and methods, as well as interference from motor disorders and possible psychiatric disorders account for the variability of epidemiological data. Subtle cognitive disorders can be found in most patients at the very beginning of the disease. Recent memory is disturbed in free recall tests, but recognition capacities are preserved. Individualization of visuospatial disorders is discussed. Alteration of conceptual capacities is the predominant disorder. Clinically obvious cognitive disorders appear only in some patients after several years of neurological disease. Some risk factors are agreed upon. The bradyphrenia concept is debated, and the term dementia is not accepted by all authors. The major cognitive alterations in Parkinson's disease differ from those observed in Alzheimer dementia, but the latter is more frequent in parkinsonian patients than in the general population. Patients with Parkinson's disease are also exposed to the risk of transient psychotic episodes. Where parkinsonian cognitive pathology is concerned, modern imaging methods are of no help to clinicians. The anatomical and biochemical changes observed in these patients are reviewed, and their responsibility in the genesis of cognitive disorders is discussed: lesions of brainstem nuclei and alterations in their cortical projections on one side, Alzheimer type dementia lesions and Lewys' bodies on the other side. The diverse and inconstant cognitive disorders of patients with Parkinson's disease cannot be explained by lesions of one single structure; they probably result from variable combinations of multiple known or unknown neuronal and biochemical changes.

[Recurrent acute dyskinesia as the sole manifestation of phenytoin poisoning]. / Dyskinésies aigues récidivantes seules manifestations d'un surdosage en phénytoine.

A 20 year-old epileptic female patient was admitted because of recurrent dyskinesias of 3 months duration. She was receiving phenobarbital, phenytoin (PHT) and clonazepam. PHT dosage had been increased 4 months earlier. Clinical examination and CT scan gave normal results. PHT plasmatic levels were 42 micrograms/ml on admission and there were no other symptoms of PHT toxicity. PHT-induced dyskinesias usually occur together with severe encephalopathy and/or clinical or paraclinical evidence of basal ganglia damage and/or other evidence of PHT toxicity, none of which were present here.

[Parkinsonian features of Steele-Richardson-Olszewski syndrome]. / Les signes parkinsoniens du syndrome de Steele-Richardson-Olszewski.

A study of parkinsonian signs was conducted in 29 patients fulfilling Lees' criteria for progressive supranuclear palsy, or Steele-Richardson-Olszewski syndrome, and 2 patients with incomplete ophthalmoplegia but 4 other cardinal features. The patients comprised 17 men and 14 women and their ages at onset ranged from 52 to 77 yrs (mean, 65.1). Parkinsonian features: akinesia, rigidity or resting tremor were present in 29 cases and appeared a mean 0.5 yrs (range 0-3) after onset. In 9 cases (29 p. 100), the parkinsonian features were similar to those of Parkinson's disease, including a resting tremor in 6 cases. The parkinsonian syndrome was initially the sole manifestation in 7 patients, who were diagnosed as having Parkinson's disease. Levodopa was effective in 6 cases, for more than a year in 3. In the other 20 cases (64 p. 100) parkinsonian signs were atypical, usually with a predominantly axial distribution. Levodopa or dopamine agonists showed transient efficacy in 6 cases. In most patients, the treatment with levodopa (n = 27) or bromocriptine (n = 15) was well tolerated. Only two disclosed akinetic fluctuations. The other main clinical features included postural instability with falls (n = 30; mean time after onset 1.8 yrs, range 0-7 yrs); characteristic ophthalmoplegia (n = 29; mean interval 4.4., range 0-18 yrs); intellectual decline and/or frontal signs (n = 22; mean interval 2.4, range 0-8 yrs); axial rigidity (n = 18; mean interval 4.2, range 1-11 yrs); pseudo-bulbar palsy (n = 18; mean interval 3, range 0-8 yrs). An upper limb dystonia was present in an autopsy-proven case.(ABSTRACT TRUNCATED AT 250 WORDS)

[POEMS cured by radical treatment of plasmacytoma]. / POEMS guéri par le traitement radical d'un plasmocytome.

A 53-year-old man presented with POEMS syndrome characterized by a solitary plasmocytoma and an angiofollicular lymph node hyperplasia similar to that observed in Castelmann's disease. Two features were prominent: first, a severe hypertriglyceridemia, which could have been related to autoimmune suppression of lipoprotein lipase activity; second, a marked immunostaining of myelin sheaths with occasional widening of myelin lamellae. These data, added to the complete regression of the neuropathy immediately following surgical removal of the plasmocytoma (with subsequent irradiation) suggested that the paraprotein was responsible for the neuropathy.

[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)

[Münchhausen syndrome. A study of 8 cases of neurologic manifestations]. / Le syndrome de Münchhausen. Etude de 8 cas à expression neurologique.

Eight cases of Münchhausen syndrome were registered within two years in two hospital neurological departments. Summaries of their clinical history were obtained following an enquiry in 58 hospitals of Paris and suburbs. All patients met Asher's description for the emergency presentation, the truculence-evasiveness manner, the luxuriance of tales, the eclecticism of the alleged symptoms, the vehement request of dangerous or painful procedures and the apparent senselessness. The paucity of scars and modesty of peregrinations recorded are discussed, as well as the lack of self-inflicted injury. Ethical implications and traps set by these tricky patients against consistent medical strategies are developed.

[Spinal dural arteriovenous fistulae: exacerbation after myelography with metrizamide]. / Fistules artérioveineuses durales rachidiennes: aggravations après myélographie au métrizamide.

Metrizamide dorsal myelography was performed in two patients with minor to moderate sensorimotor paraparesis. Direct and indirect myelographic signs of spinal arteriovenous aneurysm were seen and spinal cord angiography showed thoracic dural arteriovenous fistulae (AVF) in both cases. Within 24 hours following myelography, clear neurological worsening occurred, associated with cephalalgia, nausea and transient diplopia in one case, leading to paraplegia in a few days. Paraplegia was complete six months after surgery in one case, and had resolved after embolization of fistula in the other patient. The mechanism of neurological worsening may include: substraction of cerebrospinal fluid, sitting position during and after myelography and local increase of metrizamide concentration secondary to impaired resorption caused by the fistula. Water-soluble myelography is of invaluable aid in the diagnosis of dural AVF and must be followed by early angiography, thus allowing prompt therapeutic embolization.

[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.

[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.

[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.

[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.