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R Nitrini

Publications and source records attributed to R Nitrini.

At least 19 recordsLinked to original sources
PubMed2005

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.

Aged↗
PubMed2003

14-3-3 protein in the CSF of patients with rapidly progressive dementia.

BACKGROUND: The presence of 14-3-3 protein in the CSF has been described to have high sensitivity and specificity for Creutzfeldt-Jakob disease (CJD). OBJECTIVE: To relate 14-3-3 protein in the CSF with the clinical diagnoses of diseases causing rapidly progressive dementia. METHODS: The authors studied 46 patients with rapidly progressive dementia that was classified into three diagnostic groups: definitive or probable CJD, possible CJD, and other diagnoses. The definitive or probable CJD group comprised 17 patients (3 definitive sporadic, 1 probable iatrogenic, 3 familial, and 10 probable sporadic CJD cases), the possible CJD group was composed of 7 patients, and the group with other diagnoses had 22 patients. Detection of the 14-3-3 protein was done by the immunoblotting method. RESULTS: In the definitive or probable CJD group, the test for 14-3-3 protein in CSF was positive in 14 (82%) cases, whereas 3 patients (1 probable sporadic and 2 familial cases) had negative results. CSF was positive for 14-3-3 protein in three of seven cases with possible CJD (42%). In the group with other diagnoses, three individuals had false-positive results (13%). Their diagnoses were definitive Alzheimer's disease, hypercalcemia, and multiple intracerebral hemorrhages. CONCLUSIONS: The detection of 14-3-3 protein in CSF is a useful in vivo diagnostic test for CJD and, when used in the appropriate clinical context, shows a good correlation to CJD. The presence of the 14-3-3 protein in the CSF reinforces the CJD clinical diagnosis but may not be able to differentiate CJD from other causes of rapidly progressive dementia in everyday clinical practice.

14-3-3 Proteins↗
PubMed2001

Diffusion-weighted MRI in two cases of familial Creutzfeldt--Jakob disease.

Diffusion-weighted magnetic resonance imaging (DWI) has been described as a useful tool for the diagnosis of sporadic Creutzfeldt--Jakob disease (CJD). To our knowledge, DWI abnormalities have not previously been reported in familial CJD. In two patients with familial CJD associated with distinct mutations at codon 183 and at codon 210 of the prion protein gene, DWI showed a high signal in the basal ganglia and in the cerebral cortex. These abnormalities are similar to those described in sporadic CJD. This observation expands the value of DWI for the diagnosis of some forms of familial CJD. It remains to be investigated whether this finding also holds for CJD associated with other mutations of the prion protein gene.

Adult↗
PubMed2001

Elderly Japanese emigrants to Brazil before World War II: I. Clinical profiles based on specific historical background.

OBJECTIVE: To research the demographic and clinical profiles of elderly Japanese emigrants, who arrived in Brazil before World War II, in order to give them appropriate psychogeriatric care. DESIGN: Elderly Japanese immigrants aged 65 years and over, belonging to the Miyagi Association in the São Paulo Metropolitan Area, were targeted. They emigrated from Miyagi Prefecture to Brazil and are now living in the area. We were able to interview 166 respondents. All data were gathered using standardized interview methods covering (a) free interview about the immigration history, (b) demographics, and (c) physical status. RESULTS: Through the free interview, we found their immigration histories, which affected their clinical profiles. The mean age and educational level were 77.5 years and 6.3 years, respectively. Sixty per cent of them immigrated when they were younger than 14. Ninety-four per cent of them still keep Japanese nationality. Fifty-seven per cent of them usually use Japanese, while 10% of them use Portuguese. Although their emigration histories were hard, 76% of them perceived their health as being excellent or relatively good. The percentages of subjects with histories of disease were hypertension, 52.5%; cardiac disease, 20.8%; diabetes mellitus, 24.2%; and hyperlipidemia, 25.0%, which were affected by the Brazilian environment. CONCLUSION: The elderly Japanese who emigrated to Brazil before World War II have a unique historical and demographic background. Their clinical profiles cannot be fully understood without knowing their histories. They definitely need high quality international psychogeriatric care.

Age Distribution↗
PubMed2001

Elderly Japanese emigrants to Brazil before World War II: II. Prevalence of senile dementia.

BACKGROUND: We previously showed the prevalence of dementia in the town of Tajiri (Miyagi Prefecture, Japan), and found it to be 8.0%. The first population-based study on dementia in Brazil (Catanduva) disclosed the prevalence as being 7.1%. To evaluate the effects of environment on development of dementia, elderly Japanese immigrants living in Brazil were examined. Brazil is the country with the largest number of Japanese immigrants. METHODS: All immigrants aged 65 years and over from Miyagi Prefecture, living in the four cities of the São Paulo Metropolitan area were targeted (n = 192). We were able to examine 166 subjects (86.5%). The diagnosis of dementia was based on the DSM-IV with the severity assessed by the CDR (clinical dementia rating) scales. The cognitive ability screening instrument (CASI) was used for neuropsychological assessment. RESULTS: Thirteen subjects were diagnosed with dementia, CDR 1-3, the prevalence being 7.8%. Older subjects suffered more from dementia, and, paradoxically, the more highly educated subjects also suffered more. All the CASI items, except for long-term memory and visual construction, significantly deteriorated in the CDR 0.5 group compared with the CDR 0 group. COMMENTS: The prevalence of dementia was not thought to be affected by environmental factors. A paradoxically higher rate of dementia in the more educated subjects was probably due to the historical problems of the immigrants. Intact CASI item long-term memory in the CDR 0.5 group indicated that suspected dementia patients could maintain this function. This is the first epidemiological study on dementia in elderly Japanese immigrants in Brazil.

Age Distribution↗
PubMed2001

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.

Aged↗
PubMed2001

Prion disease resembling frontotemporal dementia and parkinsonism linked to chromosome 17.

OBJECTIVE: To compare the clinical features of a familial prion disease with those of frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17). BACKGROUND: Prion diseases are not usually considered in the differential diagnosis of FTDP-17, since familial Creutzfeldt-Jakob disease (CJD), the most common inherited prion disease, often manifests as a rapidly progressive dementia. Conversely, FTDP-17 usually has an insidious onset in the fifth decade, with abnormal behavior and parkinsonian features. METHOD: We present the clinical features of 12 patients from a family with CJD associated with a point mutation at codon 183 of the prion protein gene. RESULTS: The mean age at onset was 44.0 +/- 3.7; the duration of the symptoms until death ranged from two to nine years. Behavioral disturbances were the predominant presenting symptoms. Nine patients were first seen by psychiatrists. Eight patients manifested parkinsonian signs. CONCLUSION: These clinical features bear a considerable resemblance to those described in FTDP-17.

Adult↗
PubMed2001

Encoding process in delayed recall impairment and rate of forgetting in Alzheimer's disease.

OBJECTIVE: To assess the role of impaired encoding in learning and in delayed recall disturbances, and to evaluate the rate of forgetting in AD. METHOD: Fifteen AD patients with mild or moderate dementia and 15 normal matched controls were assessed with the Buschke Selective Reminding Test. Delayed recall was evaluated after 30 minutes and after 24 hours. RESULTS: AD patients had a poorer performance across the six trials of the learning phase as well as in both delayed recall evaluations, with no difference between recall at 30 minutes and at 24 hours. CONCLUSION: Performance in the learning phase was as specific and almost as sensitive as the performance in delayed recall for AD diagnosis. Encoding impairment was responsible for poorer learning and rapid displacement of previous learned material in the AD group. Finally, we did not find a higher rate of forgetting in AD patients.

Aged↗
PubMed2001

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.

Adult↗
PubMed2001

Involuntary hand levitation associated with parietal damage: another alien hand syndrome.

The alien hand syndrome (AHS) usually consists of an autonomous motor activity perceived as an involuntary and purposeful movement, with a feeling of foreignness of the involved limb, commonly associated with a failure to recognise ownership of the limb in the absence of visual clues. It has been described in association to lesions of the frontal lobes and corpus callosum. However, parietal damage can promote an involuntary, but purposeless, hand levitation, which, sometimes, resembles AHS. In the present study, four patients (cortico-basal ganglionic degeneration - n=2; Alzheimer's disease - n=1 and parietal stroke - n=1) who developed alien hand motor behaviour and whose CT, MRI and/or SPECT have disclosed a major contralateral parietal damage or dysfunction are described. These results reinforce the idea that parietal lobe lesions may also play a role in some patients with purposeless involuntary limb levitation, which is different from the classic forms of AHS.

Aged↗
PubMed2001

Familial Creutzfeldt-Jakob disease associated with a point mutation at codon 210 of the prion protein gene.

Creutzfeldt-Jakob disease (CJD), the most known human prion disease, is usually sporadic but approximately 15% of the cases are familial. To date, seven CJD cases with codon 210 mutation (GTT to ATT) have been reported in the literature. We describe a case of a 57 year-old woman who presented gait disturbances and rapidly progressive dementia, leading to death four months after onset. Electroencephalogram revealed periodic activity, diffusion-weighted magnetic resonance imaging showed hypersignal in basal ganglia, and test for 14-3-3 protein was strongly positive in the CSF. The complete prion protein gene coding region was sequenced after PCR amplification, showing a point mutation in codon 210. This is the first case of CJD with codon 210 mutation diagnosed in Brazil. We emphasize the role of genetic search for prion protein gene mutation, even in patients presenting clinical features resembling sporadic CJD.

Codon↗
PubMed2001

[Clinical characterization of vascular dementia: retrospective evaluation of an outpatient sample].

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.

Aged↗
PubMed2001

[Clinical subtypes of frontotemporal dementia].

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.

Dementia↗
PubMed2000

The history of tabes dorsalis and the impact of observational studies in neurology.

It is common for a particular aspect of scientific knowledge to undergo a great advance in a brief period of time after the discovery of new investigational procedures that broaden research horizons. Knowledge of neurosyphilis increased markedly during the second half of the 19th century. As revealed by the example of tabes dorsalis, this progress was not related to new research methods but instead to the impetus of careful clinical observations.

History, 19th Century↗
PubMed2000

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.

Aged↗
PubMed2000

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.

Age of Onset↗
PubMed1999

Increased apolipoprotein B serum concentration in Alzheimer's disease.

OBJECTIVE: To investigate the lipoprotein profile in a group of Alzheimer's disease (AD) patients. PATIENTS AND METHODS: Twenty-four patients with AD and 32 elderly controls were evaluated. Fasting blood samples were obtained for determination of total VLDL, HDL and LDL cholesterol, lipoprotein (a), triglycerides, apolipoprotein A1 and apolipoprotein B. RESULTS: Significantly higher levels of apolipoprotein B were found in AD patients (P = 0.004), whereas the concentration of lipoprotein (a) and plasma lipids was not statistically different. Apo B levels were similar between AD patients with or without leukoaraiosis on CT scan. CONCLUSION: AD patients had high serum concentration of apolipoprotein B. This finding suggests that apolipoprotein E may not be the single factor in lipid metabolism to play a role in AD pathogenesis.

Aged↗