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A demência com corpúsculos de Lewy (DCL), síndrome com curso flutuante, alucinações visuais, sinais parkinsonianos e hipersensibilidade a antipsicóticos, tem recebido crescente atenção nos últimos anos. Possui, desde 1995, critérios clínicos e neuropatológicos que permitem diagnosticá-la desde o início. A combinação de déficits colinérgico e dopaminérgico requer tratamentos cautelosos. Uso excessivo de medicações dopaminérgicas (L-dopa), antidopaminérgicas (antipsicóticos) ou anticolinérgicas (antipsicóticos ou anti-parkinsonianos) pode complicar o quadro, piorando sintomas cognitivos, psicóticos e extrapiramidais.

Elderly subjects may present stress symptoms due to physical, psychological and social changes during aging process. The aim of this study was to identify stress symptoms in elderly subjects and the coping strategies they used, verifying the relationship between these variables. The Stress Symptoms List (SSL) and the Jalowiec Coping Scale were administered to 41 healthy elderly subjects. Elderly subjects presented stress symptoms with a mean score of 42.8. Although problem-focused coping was predominantly observed, no significant difference was observed between mean SSL scores among elderly subjects who used problem-focused or emotion-focused coping. Although elderly subjects mainly elected problem-focused coping, stress intensity was independent of the coping style, showing that both problem- and emotion-focused coping are associated with similar stress levels.

Objective: Translating and adapting the Cornell scale for depression in dementia to the Portuguese language and verifying the interrater and test-retest reliability of the translated and adapted version., Method: The Cornell scale was translated into Portuguese and back translated into English. Divergences of translation were identified and discussed, resulting in a version which was submitted to a pre-test for cross-cultural adaptation. The final version was administered to a sample of 29 patients with probable AD and to their caregivers., Results: The Cornell Scale presented good interrater (Kappa=0,77; p<0,001) and test-retest reliability (Kappa=0,76; p<0,001). The final version was easy to administer and well understood by the caregivers., Conclusion: The Brazilian version of the Cornell Scale is an instrument with good reliability to evaluate depression in patients with dementia. This tool will contribute to the evaluation and follow-up of depressed patients with dementia in our population and may also be used in multicentric studies with Brazilian population.

The original version of the Disability Assessment for Dementia (DAD) was translated into Portuguese and back translated to English. The divergences of translation were identified and discussed, resulting in a version that was used in a preliminary investigation for cross-cultural adaptation. The final version was administered to 29 patients with mild to moderate probable Alzheimer's disease. The correlation coefficients of DAD were 0.929 and 0.932 for the inter-examiner and test-retest evaluations respectively. The reliability indexes were also high (Kappa 0.72 p<0.001 inter-examiners and 0.85 p<0.001 test-retest). The Brazilian version of DAD was easy to administer and had good reliability to assess the functional status of demented patients. It will contribute to the follow-up of these patients in our population. Moreover, it can be used in transcultural studies on functional abilities in dementia.

Introduction: Cognitive evaluation is usually performed in the assessment of patients with suspected dementia, but is not generally performed in patients with other neurological diseases., Objective: To investigate the relevance of a systematic cognitive examination in patients with different neurological conditions., Method: One-hundred and five patients consecutively attended over a one-year period in a general Neurology outpatient clinic from a public-affiliated hospital, with no complaints of cognitive changes, were submitted to the following cognitive tests: Mini-Mental State Examination (MMSE), digit span (forward and backward), delayed recall of ten simple figures, category fluency and clock drawing. Whenever possible, the cut-off scores were adjusted as function of educational level., Results: Nearly 2/3 of the patients presented impaired performance in at least one of the tests. The MMSE was altered in 20% of the patients. Performance at digit span was impaired in 50.4% of cases (29.5% forward and 20.9% backward), delayed recall in 14.2% of the patients, category fluency in 27.6% and clock drawing in 40.0%., Conclusion: These results reinforce the need of including cognitive evaluation as a routine part of the neurological examination, independently of the presence of specific complaints in this domain.

This panel had the objective of recommending evidence-based guidelines for the clinical diagnosis of Alzheimer's disease (AD) in Brazil. Guidelines from other countries and papers on the diagnosis of AD in Brazil were systematically evaluated in a thorough research of PUBMED and LILACS databases. The panel concluded that dementia diagnosis should be based on the DSM criteria and AD diagnosis, on the McKhann et al. criteria (NINCDS-ADRDA). The recommended auxiliary tests are: blood cell count, blood urea nitrogen, serum levels of creatinine, free-thyroxine, thyroid-stimulant hormone, albumin, hepatic enzymes, vitamin B12 and calcium, serological tests for syphilis and, for those aged less than 60 years, serological tests for HIV. Cerebrospinal fluid examination is recommended in special situations. Computed tomography (or preferentially magnetic resonance imaging, when available) is mandatory and has the main objective of excluding other diseases. SPECT and EEG are optional diagnostic methods.

The educational and cultural heterogeneity of the Brazilian population leads to peculiar characteristics regarding the diagnosis of Alzheimer's disease (AD). This consensus had the objective of recommending evidence-based guidelines for the clinical diagnosis of AD in Brazil. Studies on the diagnosis of AD published in Brazil were systematically evaluated in a thorough research of PUBMED and LILACS databases. For global cognitive evaluation, the Mini-Mental State Examination was recommended; for memory evaluation: delayed recall subtest of CERAD or of objects presented as drawings; attention: trail-making or digit-span; language: Boston naming, naming test from ADAS-Cog or NEUROPSI; executive functions: verbal fluency or clock-drawing; conceptualization and abstraction: similarities from CAMDEX or NEUROPSI; construction: drawings from CERAD. For functional evaluation, IQCODE, or Pfeffer Questionnaire or Bayer Scale for Activities of Daily Living was recommended. The panel concluded that the combined use of cognitive and functional evaluation based on interview with informant is recommended.

We studied the occipital inter-hemispheric coherence of Electroencephalogram (electrodes O1-O2) for alpha band (alpha1--8.0 to 10.0 Hz and alpha2--10.1 to 12.5 Hz) in two groups of healthy individuals (young adults and subjects older than 50 years-old), to assess if there is significant difference between this two age groups. No significant difference in alpha band coherences was found between these two age groups.

Decline of cognitive capacity (DCC) is due to normal physiological aging processes or to pre-dementia stage. Epidemiological studies show that elderly with decline of cognitive capacity have higher risk to develop Alzheimer's disease (AD), especially those with episodic memory deficits. This review presents the most important diagnosis criteria, neuropathological and neuropsychological findings of decline of cognitive capacity during aging.

In this article the authors report two cases of Charles Bonnet syndrome, defined as complex visual hallucinations in patients with low vision, and the patient is aware of the unreal nature of the phenomenon. A great number of cases is misdiagnosed due to lack of direct questioning by the physician. Since the emotional distress caused by this disease, the knowledge of its symptoms is essential in the management of these patients.

Given increasing research into new therapies for dementia, especially for Alzheimer disease, it has become clear that traditional methods of evaluation, centered on cognition, have proved insufficient. Thus, scales for behavioral disturbances and activities of daily living have been added. Nevertheless, some observations of clinical significant changes, as reported by caregivers, could be overlooked. Clinician's impression of global change scales (CIGIC) are based on broader information and may detect more subtle changes. Typically they take into consideration caregiver information, that which is seen by the health professional during the interview, and may also include brief cognitive tests. We present a Brazilian Portuguese version of a widely used scale of this type, specifically designed for Alzheimer disease (ADCS-CGIC), being the result of the recommended method of translation, back-translation and version by panel consensus.

Unlabelled: Mini-metal state examination (MMSE) is a screening test to detect cognitive impairment. The objectives of the present study are to describe some adaptations for use of MMSE in Brazil and to propose rules for its uniform application., Method: We evaluated 433 healthy subjects using the MMSE and verified the possible influence of demographic variables on total scores., Results: Educational level was the main factor that influenced performance, demonstrated by ANOVA: F(4,425) = 100.45, p<0.0001. The median values for educational groups were: 20 for illiterates; 25 for 1 to 4 yrs; 26.5 for 5 to 8 yrs; 28 for 9 to 11 yrs and 29 for higher levels., Conclusion: The MMSE is an excellent screening instrument and definitive rules are necessary for comparison purposes.

Three distinctive clinical presentations can occur in frontotemporal dementia (FTD): disinhibited, apathetic and stereotypic subtypes. Each one shows a specific pattern of clinical, neuropsychological and neuroimaging findings, besides manifesting the core features of this form of dementia. We report three clinical cases, each one an example of a subtype of FTD, that were evaluated by neuropsychological and neuroimaging methods. Even the reported cases being a prototype of a specific subgroup, they can share some features with the others subtypes. According to this, patients with predominantly disinhibited or stereotypic behavior can also show apathy, in much the same way as predominantly apathetic or disinhibited patients can manifest stereotypic ritualistic behavior. The final stage of FTD is generally dominated by apathetic behavior.

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.

Objective: To present the most frequent diagnosis of patients referred to a neurologist and to discuss the importance of this finding for the definition of the curriculum in Neurology., Background: The development of subspecialties of Neurology is interfering in the definition of what should be taught to train a physician or a neurologist. The knowledge of which are the most common neurological diseases may contribute to construct these curricula., Method: The initial diagnosis in 1815 outpatients referred to the neurologic service of an university-affiliated public hospital in São Paulo, Brazil, were analyzed., Results: The most common diagnosis, in decreasing order of frequency were: headache, epilepsy, mental disorders, cerebrovascular disease, head injury, polyneuropathy, vestibular syndrome, spastic crural paraparesis, extrapyramidal syndrome, dementia, intracranial hypertension and facial palsy., Conclusion: The importance of the subspecialties in the curriculum should be related to the frequency of the neurologic diseases in the community.

Report on a 12 years old patient with an arachnoid cyst of posterior cranial fossa and pseudotumor cerebri. This patient is a shunt dependent of his cyst-peritoneal shunt. This association and evolution of this pacient suggest a common and specifical pathogenic mechanism of these two pathologies based in a disturbance of the cerebral fluid circulation.

Thirty patients with dementia defined by DSM-III-R criteria (Alzheimer's disease (22), vascular dementia (3), Parkinson's disease, frontal lobe dementia, possible diffuse Lewy body dementia, normal pressure hydrocephalus and uncertain diagnosis), with scores below 24 points in the Mini-Mental Status Examination and more than 4 years of education were submitted to a neuropsychological evaluation. The scores in the neuropsychological tests were compared to those obtained by thirty normal volunteers paired for age, sex and education. Sensitivity, specificity and accuracy of the tests in the distinction of demented and normal volunteers were determined. The accuracies were calculated using ROC curves. Blessed's information-memory-concentration test showed greatest accuracy, followed by copy of simple figures, delayed memory of 10 figures (after 5 minutes), recognition of 10 figures and verbal fluency test (animals). A linear discriminant function, composed by 6 tests: visual perception, incidental memory, delayed memory (after 5 minutes), drawing of a clock, verbal fluency (animals) and calculation tests, was able to discriminate all controls from patients and only one patient was wrongly classified as normal control. These tests were chosen because they can be applied in less than 10 minutes and are very easy to interpret. This discriminant function must be applied in another group of patients and controls in order to demonstrate its value. When associated to the MMSE it may be useful to discriminate patients with dementia from normal people in epidemiological studies.

A case of neurosyphilis that got worse despite several therapeutical trials with high doses of penicillin is described. The clinical condition was stabilized and cerebrospinal fluid data normalized following treatment with chloramphenicol.

Sixty-two patients with symptomatic neurosyphilis were treated with 20 or 24 megaunits of intravenous penicillin G daily for 15 to 30 days. The mean follow-up time after the treatment was 30 months. Thirteen patients developed new neurological signs after the treatment. Their diagnosis were: general paresis (9), taboparesis (2), tabes dorsalis (1) and meningovascular neurosyphilis (1). After the treatment, thirty-six patients (58.1%) improved, 22 patients (35.5%) were unchanged and 4 patients (6.4%) deteriorated on clinical grounds. In two patients there was a progression to other forms of neurosyphilis. The results of the treatment of these patients with high doses of intravenous penicillin G were not different from the results verified with classical intramuscular penicillin that were reported in the literature, from the clinical standpoint.

Sixty-two patients with symptomatic neurosyphilis were treated with 20 or 24 megaunits of intravenous penicillin G daily for 15 to 30 days. The mean follow-up time after the treatment was 30 months. Forty-one patients had pleocytosis in the CSF before treatment. Six months and twelve or more months later, abnormal cell count was observed in 4 (9.8%) and in 3 patients (7.3%), respectively. The CSF protein level and the titers of Wassermann reaction in the CSF decreased slowly after treatment. The gammaglobulin concentration of the CSF and the immunoglobulin production inside the blood-brain barrier were still increased beyond the first year after treatment. The results of the treatment of these patients with high doses of intravenous penicillin G were not different from the results verified with lesser doses of intramuscular penicillin that were reported in the literature.

IgM antibodies against Treponema pallidum were investigated in the serum and in the CSF of 9 patients with symptomatic neurosyphilis, before the treatment and in several occasions after the treatment. Tests used were the FTA-Abs test, the IgM-solid phase hemadsorption test and an IgM-Elisa test. Titers of reactions decreased after treatment but they were still reactive in the blood and in the CSF during the second year after the treatment.

The authors present a version of the Luria's neuropsychological investigation from an initial battery comprising all the Luria's items, and according to Christensen. It is not an standardized procedure but the investigation of each area of the brain that can be regarded as a supplement to classical neurology. Each of the areas makes a highly specific contribution to ensure the operation of the functional system.