Spelling tasks and Alzheimer's disease staging.

Reading, writing and oral spelling were evaluated in 30 normal elderly individuals and 28 patients with probable Alzheimer's disease (AD). Significant differences between control and AD groups were found in all tasks. Oral spelling was the most impaired function in AD and the only one which differentiated mild and moderate dementia cases. This task is very sensitive to the disease's effects on working memory and may be included in batteries aimed at staging AD.

Incidence of dementia in a community-dwelling Brazilian population.

The authors report the incidence of dementia in a community-dwelling Brazilian population. In 1997, 1656 individuals aged 65 years or more, the majority being of very low educational level, were screened at their homes in Catanduva, Brazil, and dementia was diagnosed in 118 cases. The remaining 1538 individuals were rescreened 3.25 years later applying a health questionnaire, the Mini-Mental State Examination (MMSE) and the Pfeffer Functional Activities Questionnaire (PFAQ). According to PFAQ and MMSE scores, selected subjects were submitted to clinical, neurologic, and cognitive evaluations. The subjects diagnosed with dementia underwent laboratory tests and brain computed tomography. A total of 1119 individuals were rescreened and 50 incident cases of dementia (28 with Alzheimer disease [AD]) were identified. The incidence rate of dementia was 13.8 and of AD was 7.7 per 1000 person-years for individuals aged 65 years or older. The incidence rates of dementia almost doubled with every 5 years of age. There was no difference according to gender, but women had a higher incidence of dementia, predominantly AD, in very old age. There was a trend for higher incidence of dementia in illiterates (p = 0.07), but multivariate analysis disclosed significant association only between age and higher incidence of dementia. The incidence rates of dementia in this Brazilian community are comparable to those reported in Western and Asian studies.

Primary progressive aphasia: analisys of 16 cases.

Primary progressive aphasia (PPA) is an intriguing syndrome, showing some peculiar aspects that differentiate it from classical aphasic pictures caused by focal cerebral lesions or dementia. The slow and progressive deterioration of language occurring in these cases provides an interesting model to better understand the mechanisms involved in the linguistic process. We describe clinical and neuroimaging aspects found in 16 cases of PPA. Our patients underwent language and neuropsychological evaluation, magnetic resonance imaging (MRI) and single photon emission computerized tomography (SPECT). We observed a clear distinction in oral expression patterns; patients were classified as fluent and nonfluent. Anomia was the earliest and most evident symptom in both groups. Neuroimaging pointed to SPECT as a valuable instrument in guiding the differential diagnosis, as well as in making useful clinical and anatomical correlations. This report and a comparison to literature are an attempt to contribute to a better understanding of PPA.

[Clinical characterization of vascular dementia: retrospective evaluation of an outpatient sample]. / Caracterização clínica da demência vascular: avaliação retrospectiva de uma amostra de pacientes ambulatoriais.

OBJECTIVE: to analyze the clinical features and associated morbidity in a group of patients with vascular dementia (VD). METHODS: we retrospectively evaluated 25 patients with diagnosis of VD, based on the State of California Alzheimers Disease Diagnostic and Treatment Centers (ADDTC) criteria. Clinical and neuroimaging data and laboratory test results were obtained for the characterization of the sample. RESULTS: the mean age was 68.7 +/- 14.6 years (64.0% men), with mean educational level of 5.2 +/- 4.4 years. Sudden onset of symptoms was observed in 48.0% of patients and stepwise deteriorating and fluctuating courses being observed in 4.0% and 16% respectively. Focal neurologic deficits were the first symptom in 48.0%, with focal deficits being observed in 80% on examination. The main morbidity were: hypertension (92.0%); hypercholesterolemia (64.0%); coronary heart disease (40.0%); smoking (40.0%); hypertriglyceridemia (36.0%); diabetes mellitus (32.0%); Chagas' disease (8.0%). CONCLUSIONS: we observed strong association between VD and hypertension and hypercholesterolemia. The observation of two patients presenting Chagas' disease suggests that this endemic condition may be considered a possible regional risk factor.

No evidence of association between the D10S1423 locus and Alzheimer disease in Brazilian patients.

In a genome survey for Alzheimer's disease (AD), Zubenko et al. (1998) reported that the 234bp allele of the D10S1423 locus was more frequent among AD cases than in controls. We have analyzed this polymorphic locus in patients and healthy controls and observed that the 226bp allele is the most frequent allele in the D10S1423 locus in Brazilian AD patients. However, no statistically significant association between any D10S1423 allele was observed in AD patients as well as in controls.

Analysis of the disease risk locus DXS1047 polymorphism in Brazilian Alzheimer patients.

Alzheimer's disease (AD) is a disorder characterized by a progressive deterioration in memory and other cognitive functions. Four genes associated with early onset AD have been identified but familial AD is rare. The majority of late onset AD (LOAD) is caused by a complex inheritance with several genes interacting with environmental factors. The epsilon4 allele of the apolipoprotein E (APOE) gene has been reported worldwide as a risk factor associated with LOAD. The short variant of a polymorphism in the transcriptional region of the serotonin transporter gene (5-HTTLPR) was analyzed in several psychiatric conditions and found to be more frequently associated with European and Brazilian LOAD patients. Recently, allelic associations with LOAD were reported for five other loci, the most significant for one X-linked 202-bp allele, at the DXS1047 locus. We have analyzed this locus in Brazilian LOAD patients and observed that the 202-bp allele was not significantly more frequent among patients. In contrast, two other alleles (200 bp and 208 bp) were less frequent among AD male patients than in controls, confirming the importance of replicating association studies in different populations.

[Malignant hyperthermia susceptibility in 3 patients with malignant neuroleptic syndrome]. / Susceptibilidade à hipertermia maligna em três pacientes com síndrome maligna por neurolépticos.

Hyperthermia, skeletal muscle rigidity, rhabdomyolysis, acidosis and multiple system insufficiency characterize malignant hyperthermia. Anaesthetic malignant hyperthermia follows halogenated volatile agents and/or depolarizing muscle relaxants utilization. Diagnosis is based on in vitro muscle contracture in response to halothane and/or caffeine exposure. Neuroleptic malignant syndrome affects patients taking neuroleptic drugs; clinical findings include hyperthermia, extrapyramidal rigidity, acidosis, neurovegetative instability and neurological signs. We report three neuroleptic malignant syndrome patients with positive muscle contracture tests which shows that muscle from neuroleptic malignant syndrome patients may in some instances show alterations similar to those of anaesthetic malignant hyperthermia.

SPECT in Alzheimer's disease: features associated with bilateral parietotemporal hypoperfusion.

OBJECTIVE: To investigate why bilateral parietotemporal hypoperfusion, the typical SPECT pattern of Alzheimer's disease (AD), occurs in some but not in all patients with probable AD. METHODS: We reviewed the SPECT scans of 220 patients presenting with cognitive impairment. Among them, 104 patients fulfilled NINCDS-ADRDA criteria for probable AD, 48 (32 women) with early onset (before age of 65) and 56 (40 women) with late onset of the symptoms. Dementia severity was assessed by the Mini-Mental State Examination. The SPECT scans were classified by visual inspection blind to clinical diagnoses. RESULTS: Bilateral parietotemporal hypoperfusion was more frequent in patients with severe AD, in those with early onset of the symptoms, and in men. Duration of symptoms, type of gamma-camera or radiopharmaceutical agent used were not associated with this SPECT pattern. CONCLUSION: These findings may be useful in the clinical setting and point to heterogeneity of AD according to age at onset.

Neurophysiological characteristics of Lafora's Disease

Avaliamos as características neurofisiológicas (EEG e potenciais evocados) de 2 pacientes na fase III da doença de Lafora, No EEG, desorganizaçäo da atividade de base foi associada a descargas epileptiformes difusas e bilaterais, com acentuaçäo em regiöes pccipitais onde também ocorreram descargas isoladas nos exames de ambos os pacientes. Resposta fotoconvulsiva foi evidenciada nos 2 casos e, em 1 deles, foram registradas descargas occipitais seguindo a estimulaçäo fótica com baixa frequência (1-3 flashes/s) correspondendo a grande amplitude de N2-P2 na resposta evocada visual por flash