Combination of white matter hyperintensities and Aß burden is related to cognitive composites domain scores in subjective cognitive decline: the FACEHBI cohort.

BACKGROUND: To explore whether the combination of white matter hyperintensities (WMHs) and amyloid-beta (Aß) deposition is associated with worse cognitive performance on cognitive composites (CCs) domain scores in individuals with subjective cognitive decline (SCD). METHODS: Two hundred participants from the FACEHBI cohort underwent structural magnetic resonance imaging (MRI), 18F-florbetaben positron emission tomography (FBB-PET), and neuropsychological assessment. WMHs were addressed through the Fazekas scale, the Age-Related White Matter Changes (ARWMC) scale, and the FreeSurfer pipeline. Eight CCs domain scores were created using the principal component analysis (PCA). Age, sex, education, and apolipoprotein E (APOE) were used as adjusting variables. RESULTS: Adjusted multiple linear regression models showed that FreeSurfer (B - .245; 95% CI - .1.676, - .393, p = .016) and ß burden (SUVR) (B - .180; 95% CI - 2.140, - .292; p = .070) were associated with face-name associative memory CCs domain score, although the latest one was not statistically significant after correction for multiple testing (p = .070). There was non-significant interaction of these two factors on this same CCs domain score (p = .54). However, its cumulative effects on face-name associative performance indicated that those individuals with either higher WMH load or higher Aß burden showed the worst performance on the face-name associative memory CCs domain score. CONCLUSIONS: Our results suggest that increased WMH load and increased Aß are independently associated with poorer episodic memory performance in SCD individuals, indicating a cumulative effect of the combination of these two pathological conditions in promoting lower cognitive performance, an aspect that could help in terms of treatment and prevention.

Longitudinal Course of Agitation and Aggression in Patients with Alzheimer's Disease in a Cohort Study: Methods, Baseline and Longitudinal Results of the A3C Study.

BACKGROUND: To present methodology, baseline results and longitudinal course of the Agitation and Aggression in patients with Alzheimer's Disease Cohort (A3C) study. OBJECTIVES: The central objective of A3C was to study the course, over 12 months of clinically significant Agitation and Aggression symptoms based on validated measures, and to assess relationships between symptoms and clinical significance based on global ratings. DESIGN: A3C is a longitudinal, prospective, multicenter observational cohort study performed at eight memory clinics in France, and their associated long-term care facilities. SETTING: Clinical visits were scheduled at baseline, monthly during the first 3 months, at 6 months, at 9 months and at 12 months. The first three months intended to simulate a classic randomized control trial 12-week treatment design. PARTICIPANTS: Alzheimer's Disease patients with clinically significant Agitation and Aggression symptoms lived at home or in long-term care facilities. MEASUREMENTS: Clinically significant Agitation and Aggression symptoms were rated on Neuropsychiatric Inventory (NPI), NPI-Clinician rating (NPI-C) Agitation and Aggression domains, and Cohen Mansfield Agitation Inventory. Global rating of agitation over time was based on the modified Alzheimer's Disease Cooperative Study-Clinical Global Impression of Change. International Psychogeriatric Association "Provisional Diagnostic Criteria for Agitation", socio-demographics, non-pharmacological approaches, psychotropic medication use, resource utilization, quality of life, cognitive and physical status were assessed. RESULTS: A3C enrolled 262 AD patients with a mean age of 82.4 years (SD ±7.2 years), 58.4% women, 69.9% at home. At baseline, mean MMSE score was 10.0 (SD±8.0), Cohen Mansfield Agitation Inventory score was 62.0 (SD±15.8) and NPI-C Agitation and Aggression clinician severity score was 15.8 (SD±10.8). According to the International Psychogeriatric Association agitation definition, more than 70% of participants showed excessive motor activity (n=199, 76.3%) and/or a verbal aggression (n=199, 76.3%) while 115 (44.1%) displayed physical aggression. The change of the CMAI score and the NPI-C Agitation and Aggression at 1-year follow-up period was respectively -11.36 (Standard Error (SE)=1.32; p<0.001) and -6.72 (SE=0.77; p<0.001). CONCLUSION: Little is known about the longitudinal course of clinically significant agitation symptoms in Alzheimer's Disease about the variability in different outcome measures over time, or the definition of a clinically meaningful improvement. A3C may provide useful data to optimize future clinical trials and guide treatment development for Agitation and Aggression in Alzheimer's Disease.

Guidance for the Prevention of the COVID-19 Epidemic in Long-Term Care Facilities: A Short-Term Prospective Study.

BACKGROUND: Guidance aiming at limiting the entry and spread of the COVID-19 have been widely communicated to Long-term Care Facilities (LTCFs). However, no clinical research has investigated their relevance. OBJECTIVE: Our objective was to compare the guidance applied for the prevention of the COVID-19 epidemic between the LTCFs having been contaminated by COVID-19 and LTCFs having not been contaminated. METHODS: A questionnaire was sent and systematically accompanied by phone call to the 132 LTCFs of Haute-Garonne (Occitania region, South-West of France). The questionnaire focused on the preventive measures implemented before March 23, 2020 (first LTCFs contaminated in this area). The questionnaire focused on physician support, implementation of usual guidance (eg, masks, hydro-alcoholic solute used), training on hygiene, containment in residents' rooms and other distancing measures, use of temporary workers, compartmentalization within zones of residents and staff and a self-assessment analogic scale on the quality of the application of the preventive measures. We compared implementation of the guidance between the LTCFs with at least one case of COVID-19 among residents and/or health care professionals and LTCFs without COVID-19 case (between March 23rd and May 6th). RESULTS: 124 LTCFs participated (93.9%). 30 LTCFs (24.19%) were contaminated with COVID-19. Large heterogeneity of the application of the guidance was observed. Public LTCFs (OR= 0.39 (0.20-0.73), LTCFs which organized staff compartmentalization within zones (OR= 0.19 (0.07-0.48)), and LTCF with a staff who self-assessed a higher quality implementation of the preventive measures (OR= 0.65 (0.43-0.98)) were significantly more likely to avoid contamination by the COVID-19 outbreak. CONCLUSION: Our study supports the relevance of guidance to prevent the entry of COVID-19, in particular the staff compartmentalization within zones, as well as the perception of the staff regarding the quality of implementation of those measures in LTCFs.

Correlations between plasma and PET beta-amyloid levels in individuals with subjective cognitive decline: the Fundació ACE Healthy Brain Initiative (FACEHBI).

BACKGROUND: Peripheral biomarkers that identify individuals at risk of developing Alzheimer's disease (AD) or predicting high amyloid beta (Aß) brain burden would be highly valuable. To facilitate clinical trials of disease-modifying therapies, plasma concentrations of Aß species are good candidates for peripheral AD biomarkers, but studies to date have generated conflicting results. METHODS: The Fundació ACE Healthy Brain Initiative (FACEHBI) study uses a convenience sample of 200 individuals diagnosed with subjective cognitive decline (SCD) at the Fundació ACE (Barcelona, Spain) who underwent amyloid florbetaben(18F) (FBB) positron emission tomography (PET) brain imaging. Baseline plasma samples from FACEHBI subjects (aged 65.9 ± 7.2 years) were analyzed using the ABtest (Araclon Biotech). This test directly determines the free plasma (FP) and total plasma (TP) levels of Aß40 and Aß42 peptides. The association between Aß40 and Aß42 plasma levels and FBB-PET global standardized uptake value ratio (SUVR) was determined using correlations and linear regression-based methods. The effect of the APOE genotype on plasma Aß levels and FBB-PET was also assessed. Finally, various models including different combinations of demographics, genetics, and Aß plasma levels were constructed using logistic regression and area under the receiver operating characteristic curve (AUROC) analyses to evaluate their ability for discriminating which subjects presented brain amyloidosis. RESULTS: FBB-PET global SUVR correlated weakly but significantly with Aß42/40 plasma ratios. For TP42/40, this observation persisted after controlling for age and APOE ε4 allele carrier status (R2 = 0.193, p = 1.01E-09). The ROC curve demonstrated that plasma Aß measurements are not superior to APOE and age in combination in predicting brain amyloidosis. It is noteworthy that using a simple preselection tool (the TP42/40 ratio with an empirical cut-off value of 0.08) optimizes the sensitivity and reduces the number of individuals subjected to Aß FBB-PET scanners to 52.8%. No significant dependency was observed between APOE genotype and plasma Aß measurements (p value for interaction = 0.105). CONCLUSION: Brain and plasma Aß levels are partially correlated in individuals diagnosed with SCD. Aß plasma measurements, particularly the TP42/40 ratio, could generate a new recruitment strategy independent of the APOE genotype that would improve identification of SCD subjects with brain amyloidosis and reduce the rate of screening failures in preclinical AD studies. Independent replication of these findings is warranted.

The ROSAS Cohort: A Prospective, Longitudinal Study of Biomarkers for Alzheimer's Disease. Strategy, Methods and Initial Results.

OBJECTIVE: The aims of the Research Of biomarkers in Alzheimer's diseaSe (ROSAS) study were to determine the biofluid and imaging biomarkers permitting an early diagnosis of Alzheimer's disease and better characterisation of cognitive and behavioural course of the pathology. This paper outlines the overall strategy, methodology of the study, baseline characteristics of the population and first longitudinal results from the ROSAS cohort. METHODS: Longitudinal prospective monocentric observational study performed at the Alzheimer's disease Research centre in Toulouse. A total of 387 patients were studied and analyzed in 3 groups: 184 patients with dementia of Alzheimer's type, 96 patients with memory disorders without dementia (Mild Cognitive Impairment) and 107 patients without abnormal memory tests (control group), and were followed up during 4 years. Patient's sociodemographic characteristics, risk factors, medical conditions, previous and current medications, neuropsychological assessment and overall cognitive status were recorded. Blood and urine samples were collected at every year, Magnetic Resonance Imaging were performed at inclusion, after one year of follow-up and at the end of the study. RESULTS: At baseline, three different groups of the cohort differed interestingly in age, level of education, and in percentage of ApoEε4 carriers whereas the history of cardiovascular and endocrine pathologies were similar among the groups. During the follow-up period (3-4 years) 42 mild cognitive impairment patients (43.8%) progressed to dementia, 7 controls progressed into mild cognitive impairment and 1 patient in the control group converted from mild cognitive impairment group to dementia of Alzheimer's type group. During the first year of follow up, the incidence of progression from mild cognitive impairment to dementia of Alzheimer's type was 12.7 per 100, during the second year 33.9 per 100 and 46.7 per 100 for the third year. CONCLUSION: This paper presents the baseline characteristics of the unique French prospective monocenter study in which the natural course of dementia of Alzheimer's type was evaluated. Future analysis of blood and urine samples collection from the ROSAS study will permit to identify possible biofluid biomarkers predicting the early stages of the dementia of Alzheimer's type and risk of progression from Mild Cognitive Impairment to Alzheimer's disease.

FACEHBI: A Prospective Study of Risk Factors, Biomarkers and Cognition in a Cohort of Individuals with Subjective Cognitive Decline. Study Rationale and Research Protocols.

BACKGROUND: Long-term longitudinal studies with multimodal biomarkers are needed to delve into the knowledge of preclinical AD. Subjective cognitive decline has been proposed as a risk factor for the development of cognitive impairment. Thus, including individuals with SCD in observational studies may be a cost-effective strategy to increase the prevalence of preclinical AD in the sample. OBJECTIVES: To describe the rationale, research protocols and baseline characteristics of participants in the Fundació ACE Healthy Brain Initiative (FACEHBI). DESIGN: FACEHBI is a clinical trial (EudraCT: 2014-000798-38) embedded within a long-term observational study of individuals with SCD. SETTING: Participants have been recruited at the memory clinic of Fundació ACE (Barcelona) from two different sources: patients referred by a general practitioner and individuals from an Open House Initiative. PARTICIPANTS: 200 individuals diagnosed with SCD with a strictly normal performance in a comprehensive neuropsychological battery. MEASUREMENTS: Individuals will undergo an extensive neuropsychological protocol, risk factor assessment and a set of multimodal biomarkers including florbetaben PET, structural and functional MRI, diffusion tensor imaging, determination of amyloid species in plasma and neurophthalmologic assessment with optical coherence tomography. RESULTS: Two hundred individuals have been recruited in 15 months. Mean age was 65.9 years; mean MMSE was 29.2 with a mean of 14.8 years of education. CONCLUSIONS: FACEHBI is a long-term study of cognition, biomarkers and lifestyle that has been designed upon an innovative symptom-based approach using SCD as target population. It will shed light on the pathophysiology of preclinical AD and the role of SCD as a risk marker for the development of cognitive impairment.

Genome-wide significant risk factors for Alzheimer's disease: role in progression to dementia due to Alzheimer's disease among subjects with mild cognitive impairment.

Few data are available concerning the role of risk markers for Alzheimer's disease (AD) in progression to AD dementia among subjects with mild cognitive impairment (MCI). We therefore investigated the role of well-known AD-associated single-nucleotide polymorphism (SNP) in the progression from MCI to AD dementia. Four independent MCI data sets were included in the analysis: (a) the German study on Aging, Cognition and Dementia in primary care patients (n=853); (b) the German Dementia Competence Network (n=812); (c) the Fundació ACE from Barcelona, Spain (n=1245); and (d) the MCI data set of the Amsterdam Dementia Cohort (n=306). The effects of single markers and combined polygenic scores were measured using Cox proportional hazards models and meta-analyses. The clusterin (CLU) locus was an independent genetic risk factor for MCI to AD progression (CLU rs9331888: hazard ratio (HR)=1.187 (1.054-1.32); P=0.0035). A polygenic score (PGS1) comprising nine established genome-wide AD risk loci predicted a small effect on the risk of MCI to AD progression in APOE-ɛ4 (apolipoprotein E-ɛ4) carriers (HR=1.746 (1.029-2.965); P=0.038). The novel AD loci reported by the International Genomics of Alzheimer's Project were not implicated in MCI to AD dementia progression. SNP-based polygenic risk scores comprising currently available AD genetic markers did not predict MCI to AD progression. We conclude that SNPs in CLU are potential markers for MCI to AD progression.

Follow-up of loci from the International Genomics of Alzheimer's Disease Project identifies TRIP4 as a novel susceptibility gene.

To follow-up loci discovered by the International Genomics of Alzheimer's Disease Project, we attempted independent replication of 19 single nucleotide polymorphisms (SNPs) in a large Spanish sample (Fundació ACE data set; 1808 patients and 2564 controls). Our results corroborate association with four SNPs located in the genes INPP5D, MEF2C, ZCWPW1 and FERMT2, respectively. Of these, ZCWPW1 was the only SNP to withstand correction for multiple testing (P=0.000655). Furthermore, we identify TRIP4 (rs74615166) as a novel genome-wide significant locus for Alzheimer's disease risk (odds ratio=1.31; confidence interval 95% (1.19-1.44); P=9.74 × 10(-)(9)).

ATP5H/KCTD2 locus is associated with Alzheimer's disease risk.

To identify loci associated with Alzheimer disease, we conducted a three-stage analysis using existing genome-wide association studies (GWAS) and genotyping in a new sample. In Stage I, all suggestive single-nucleotide polymorphisms (at P<0.001) in a previously reported GWAS of seven independent studies (8082 Alzheimer's disease (AD) cases; 12 040 controls) were selected, and in Stage II these were examined in an in silico analysis within the Cohorts for Heart and Aging Research in Genomic Epidemiology consortium GWAS (1367 cases and 12904 controls). Six novel signals reaching P<5 × 10(-6) were genotyped in an independent Stage III sample (the Fundació ACE data set) of 2200 sporadic AD patients and 2301 controls. We identified a novel association with AD in the adenosine triphosphate (ATP) synthase, H+ transporting, mitochondrial F0 (ATP5H)/Potassium channel tetramerization domain-containing protein 2 (KCTD2) locus, which reached genome-wide significance in the combined discovery and genotyping sample (rs11870474, odds ratio (OR)=1.58, P=2.6 × 10(-7) in discovery and OR=1.43, P=0.004 in Fundació ACE data set; combined OR=1.53, P=4.7 × 10(-9)). This ATP5H/KCTD2 locus has an important function in mitochondrial energy production and neuronal hyperpolarization during cellular stress conditions, such as hypoxia or glucose deprivation.

GAB2 gene does not modify the risk of Alzheimer's disease in Spanish APOE 4 carriers.

OBJECTIVES: The genetic basis of Alzheimer's disease (AD) is being analyzed in multiple whole genome association studies (WGAS). The GAB2 gene has been proposed as a modifying factor of APOE epsilon 4 allele in a recent case-control WGAS conducted in the US. Given the potential application of these novel results in AD diagnostics, we decided to make an independent replication to examine the GAB2 gene effect in our series. DESIGN: We are conducting a multicenter population-based study of AD in Spain. PARTICIPANTS: We analyzed a total of 1116 Spanish individuals. Specifically, 521 AD patients, 475 controls from the general population and 120 neurologically-normal elderly controls (NNE controls). METHODS: We have genotyped GAB2 (rs2373115 G/T) and APOE rs429358 (SNP112)/rs7412 (SNP158) polymorphisms using real time-PCR technologies. RESULTS: As previously reported in Spain, APOE epsilon 4 allele was strongly associated with AD in our series (OR=2.88 [95% C.I. 2.16- 3.84], p=7.38E-11). Moreover, a large effect for epsilone 4/epsilone 4 genotype was also observed (OR=14.45 [95% C.I., 3.34-125.2], p=1.8E-6). No difference between the general population and the NNE controls series were observed for APOE genotypes (P > 0.61). Next, we explored GAB2 rs2373115 SNP singlelocus association using different genetic models and comparing AD versus controls or NNE controls. No evidence of association with AD was observed for this GAB2 marker (p > 0.17). To evaluate GAB2-APOE genegene interactions, we stratified our series according to APOE genotype and case-control status, in accordance with the original studies. Again, no evidence of genetic association with AD was observed in any strata of GAB2-APOE loci pair (p > 0.34). CONCLUSION: GAB2 rs2373115 marker does not modify the risk of Alzheimer's disease in Spanish APOE epsilon 4 carriers.

Paquimeningitis hipertrófica.Revisión histórica a propósito de dos casos y relación patogénicacon el síndrome Tolosa-Hunt y el pseudotumor orbitario / Hypertrophic pachymeningitis. A review of the histories of two cases and the pathological relationship with the Tolosa-Hunt syndrome and the orbital pseudotumor

Introducción. La paquimeningitis hipertrófica es una entidad infrecuente que origina un engrosamiento de la duramadre y cuya patogenia permanece sin aclarar. Presentamos dos nuevos casos de etiología indeterminada. Casos clínicos. Caso 1. Varón de 53 años que presenta desde febrero de 1981 cefalea occipital, acúfenos e hipoacusia derecha. Ingresa en octubre del mismo año por aumento de su cefalea, dolor periorbitario, disgeusia y parálisis facial periférica ipsilateral. En diciembre presenta convulsiones tonicoclónicas generalizadas y parálisis del VII y XI par derechos y del IX, X y XII izquierdos. En febrero de 1982 inicia neuralgia trigeminal derecha. Reingresa en noviembre de 1983 por cefalea continua con vómitos y trastorno de conducta. La tomografía computarizada mostró imágenes de alta atenuación en duramadre posterior, desestimándose por neurocirugía la biopsia. En marzo de 1985 fallece. El estudio necrópsico mostró una paquimeningitis hipertrófica. Caso 2. Paciente de 62 años que consulta en noviembre de 1995 por hipoacusia derecha de seis meses de evolución, a la que se añaden progresivamente parálisis ipsilaterales de los pares craneales II, IV, VI, VII y VIII, sin otras alteraciones en la exploración física. La analítica y los estudios serológicos fueron normales. La resonancia magnética craneal mostró una lesión infiltrativa extraparenquimatosa en fosa craneal media. Se decidió biopsia tras no mejorar clínicamente con tratamiento corticosteroideo. La anatomía patológica demostró una paquimeningitis hipertrófica. Se inició tratamiento con ciclofosfamida en forma de pulsos mensuales, permaneciendo estable hasta la fecha. Conclusión. Con estos dos nuevos casos queremos establecer una relación patogénica con el síndrome Tolosa-Hunt y el pseudotumor orbitario, así como reflejar el papel del tratamiento inmunosupresor en el control evolutivo de la paquimeningitis hipertrófica (AU)

Mitral papillary fibroelastoma as a cause of cardiogenic embolic stroke: report of two cases and review of the literature.

UNLABELLED: Papillary fibroelastoma (PFE) is a rare benign tumour that attaches to the endocardial surface, mostly on cardiac valves. Though usually asymptomatic, it can be the source of several complications. To date, 49 cases have been reported of embolic stroke with a PFE as the probable origin. CASE REPORTS: (i) a 39-year-old male presented with ischemic embolic stroke; the presence of a PFE was assessed by means of transoesophageal echocardiography and confirmed by pathological findings; (ii) a 32-year-old woman presented with sudden onset of left hemiparesis; a cardiogenic embolic stroke was suspected, and a diagnosis of PFE was made based on echocardiographic and pathological findings. In both cases, surgical excision of the tumours was performed with no recurrences at follow-up. Two mechanisms can explain the formation of emboli in PFE: dislodgement of the tumour leaves or fibrin-platelet aggregation on the endocardial surface of these leaves. Transthoracic echocardiography may lead to the suspicion of a PFE, but transoesophageal echocardiography is required for confirmation. Prompt surgical excision is indicated in most cases. Anticoagulation is only recommended in situations of high surgical risk and during the wait for surgery.

[Hypertrophic pachymeningitis. A review of the histories of two cases and pathological relationship with the Tolosa-Hunt syndrome and the orbital pseudotumor]. / Paquimeningitis hipertrófica. Revisión histórica a propósito de dos casos y relación patogénica con el síndrome Tolosa-Hunt y el pseudotumor orbitario.

INTRODUCTION: Hypertrophic pachymeningitis is an infrequent condition which starts with a thickening of the dura mater and whose pathogenesis is unknown. We present two new cases of unknown aetiology. CLINICAL CASE: Case 1. A 53 year old man complained of occipital headache, tinnitus and deafness since February 1981. In October 1981 he was admitted to hospital with a worse headache, perio-orbital pain, dysgeusia and ipsilateral peripheral facial palsy. In December he had generalized tonic-clonic seizures and paralysis of the VII and XI right cranial nerves and IX, X and XII left cranial nerves. In February 1982 he developed right trigeminal neuralgia. He was readmitted in November 1983 with continuous headache, vomiting and a behavior disorder. On CT there was marked attenuation of the posterior dura mater, which the neurosurgical department considered unsuitable for biopsy. He died in March 1985. On necropsy there was hypertrophic pachymeningitis. Case 2. A 62 year old patient consulted in November 1995 complaining of right hypoacusia for the past six months, progressively accompanied by ipsilateral paralysis of the II, IV, VI, VII and VIII cranial nerves but with no other alterations on physical examination. Analytical and serological investigations were normal. Cranial MR showed an extraparenchymatous infiltrating lesion in the middle cranial fossa. Biopsy was decided on when no clinical improvement was seen with corticosteroid treatment. The pathologist reported hypertrophic pachymeningitis. Treatment was started with cyclophosphamide in monthly doses and the condition has remained stable to date. CONCLUSION: With these two cases we wish to establish a pathogenic relation between the Tolosa-Hunt syndrome and orbital pseudotumor and show the role played by immunosuppressive treatment in the control of hypertrophic pachymeningitis.

[Progressive multifocal leukoencephalopathy in elderly immunocompetent patients. Report of 2 cases]. / Leucoencefalopatía multifocal progresiva en pacientes ancianos inmunocompetentes. A propósito de dos casos.

INTRODUCTION: Progressive multifocal leukoencephalopathy is a disorder which is rare in immunocompetent patients. OBJECTIVES: We report the cases of two elderly patients with serology, in one case positive for hepatitis C, and in the other with anti-DNA antibodies, and discuss the part these might play in causing progressive multifocal leukoencephalopathy. CLINICAL CASES: Case 1. An 86 year old man had been found on serology investigations to be positive for hepatitis C virus. In November 1996 he complained of dysarthria and left hemi-negligence following an accidental fall. Since his clinical condition became worse he was admitted to hospital for further investigation. On neuroimaging studies the intracerebral lesions were increased. The only other finding confirmed was that of positive serology for hepatitis C virus. The patient deteriorated progressively and died 50 days after admission. Case 2. A 70 year old woman began to show progressive cognitive impairment and left hemiparesia in June 1996. Se was investigated in another centre and provisionally diagnosed as having vasculitis of the CNS, in view of her positive anti-DNA antibody and right frontoparietal hypodense lesion. Treatment had been started with corticosteroids. She was admitted to our hospital when her neurological deficits worsened. The immunological alterations were confirmed. On MRI the lesions in the white matter were seen to have progressed. The patient slowly improved. She was discharged from hospital in February 1997 in a semiconscious state, able to follow persons and things with her eyes, with global aphasia and with spastic tetraparesia which was mainly left-sided. She remains stable. CONCLUSION: Progressive multifocal leukoencephalopathy is a condition which should be remembered when dealing with immunocompetent patients.

[Intraventricular hemorrhage due to the rupture of atherosclerotic dissecting aneurysm of the middle cerebral artery]. / Hemorragia intraventricular por rotura de aneurisma disecante ateroscleroso de la arteria cerebral media.

INTRODUCTION: We present a case of fusiform intracranial aneurysm where, apart from the unusual site, we draw attention to the form of clinical presentation, namely intraventricular haemorrhage. Clinical case. A 68 year-old-man with a history of smoking, hyperuricemia with seizures of gout treated with colchicine and allopurinol, and hypertension treated with captopril. Nine years previously he had a right capsulothalamic haematoma and presented (as a sequela of this) a left sensomotor deficit, with a good functional level. In December 1998 he was admitted for sudden onset of headache and deterioration of consciousness. He had right limb movements which were typical of decerebration and made intubation and mechanical ventilation necessary. Cerebral CT, with angiographic sequences, showed blood in the lateral ventricles and III ventricle, with ventricular dilation and a fusiform aneurysm of the left middle cerebral artery. In view of the neurological state of the patient, treatment of the aneurysm was postponed. After initial improvement, which permitted extubation, tetraparesia (predominantly right) and a pseudobulbar syndrome were seen. The patient had repeated respiratory infections and died from sepsis caused by Pseudomona aeruginosa (of respiratory origin) three months after admission. CONCLUSIONS: Fusiform intracranial aneurysms form 9% of all aneurysms. Localization to the middle cerebral artery is infrequent, the basilar trunk and internal carotid artery are commoner sites. In our case angio-CT was a useful non-invasive neuro-radiological technique.

[Stroke: a stranger among the people]. / Ictus: un desconocido para la población.

INTRODUCTION: Lack of awareness of warning signs of stroke is a factor that contribute to late patient arrival to the emergency department. OBJECTIVE: The goal of this pilot study was to determine the baseline knowledge of stroke among the population (terminology, signs-symptoms, risk factors and attitude) prior to educational campaigns. PATIENTS AND METHODS: A population-based interview using closed-ended questions was conducted by neurologists among 100 users of the Vall d'Hebron's Primary Health Center, randomly sampled. RESULTS: In our population a 9% unknowns totally the disease, of the remainder, 42% has a good knowledge of signs-symptoms and 46% of risk factors. Only 22% of the sample has good global knowledge of the disease. If suffering a stroke this population should seek medical attention through 911 (46.2%) or come directly to the emergency department (50.5%). If symptoms were gone away, transient ischemic attack (TIA), appears a trend to contact primary physicians (59.3%). Respondents aged > 65 years were less likely to recognize symptoms (p = 0.001) and to consider stroke as an emergency. Respondents with an affected relative (50.5%) tend to locate more exactly the disease at the brain (p = 0.05) and to arrive earlier to the emergency department (p = 0.045), than those with non-affected relatives. CONCLUSIONS: Less than a quarter of our population have a good knowledge of the disease. Stroke is considered an emergency unlike TIA. The information about stroke is theoretically associated with early presentation to the emergency department. These results permit a redesign of the questionnaire to conduct a second phase of the study and generalize them for the Spanish population.