Whipple's disease with neurological manifestations - Case report

Whipple's disease (WD) is an uncommon multisystem condition caused by the bacillus Tropheryma whipplei. Central nervous system involvement is a classical feature of the disease observed in 20 to 40% of the patients. We report the case of a 62 yeards old man with WD that developed neurological manifestations during its course, and discuss the most usual signs and symptoms focusing on recent diagnostic criteria and novel treatment regimens.622A34234

Age and hippocampal volume predict distinct parts of default mode network activity

Group comparison studies have established that activity in the posterior part of the default-mode network (DMN) is down-regulated by both normal ageing and Alzheimer’s disease (AD). In this study linear regression models were used to disentangle distinctive DMN activity patterns that are more profoundly associated with either normal ageing or a structural marker of neurodegeneration. 312 datasets inclusive of healthy adults and patients were analysed. Days of life at scan (DOL) and hippocampal volume were used as predictors. Group comparisons confirmed a significant association between functional connectivity in the posterior cingulate/retrosplenial cortex and precuneus and both ageing and AD. Fully-corrected regression models revealed that DOL significantly predicted DMN strength in these regions. No such effect, however, was predicted by hippocampal volume. A significant positive association was found between hippocampal volumes and DMN connectivity in the right temporo-parietal junction (TPJ). These results indicate that postero-medial DMN down-regulation may not be specific to neurodegenerative processes but may be more an indication of brain vulnerability to degeneration. The DMN-TPJ disconnection is instead linked to the volumetric properties of the hippocampus, may reflect early-stage regional accumulation of pathology and might be of aid in the clinical detection of abnormal ageing

Learning, retrieval, and recognition are compromised in aMCI and mild AD: Are distinct episodic memory processes mediated by the same anatomical structures?

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Performance of different episodic memory processes in patients with amnestic mild cognitive impairment (aMCI) and mild Alzheimer's disease (AD) and their anatomical correlates are not completely understood. We evaluated the performance of 48 subjects (17 with aMCI, 15 with mild AD, and 16 controls) on the Rey Auditory Verbal Learning Test (RAVLT). A brain MRI voxel-based morphometry (VBM) analysis was run with the aim of evaluating the correlations between RAVLT and gray matter density. All memory processes were compromised in aMCI and mild AD. Also, the same cerebral structures were involved in all RAVLT stages. Learning and delayed recall were more related to the medial prefrontal cortex and hippocampi, whereas recognition was more related to the thalamic nuclei and caudate nucleus, particularly in the left side. Our findings suggest that these Structures may act as a complex functional system and are involved in the acquisition of new information. (JINS 2010, 16, 205-209.)o TEXTO COMPLETO DESTE ARTIGO, ESTARÁ DISPONÍVEL À PARTIR DE AGOSTO DE 2015.161205209Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP

Lexical semantic memory in amnestic mild cognitive impairment and mild Alzheimer's Disease

Objective: To study lexical semantic memory in patients with amnestic mild cognitive impairment (aMCl), mild Alzheimer's disease (AD) and normal controls. Method: Fifteen mild AD, 15 aMCI, and 15 normal control subjects were included. Diagnosis of AD was based on DSM-IV and NINCDS-ADRDA criteria, and that of aMCl, on the criteria of the International Working Group on Mild Cognitive Impairment, using CDR 0.5 for aMCl and CDR 1 for mild AD. All subjects underwent semantic memory tests (Boston Naming-BNT, CAMCOG Similarities item), Rey Auditory Verbal Learning Test (RAVLT), Mini-Mental Status Examination (MMSE), neuropsychological tests (counterproofs), and Cornell Scale for Depression in Dementia. Data analysis used Mann-Whitney test for intergroup comparisons and Pearson's coefficient for correlations between memory tests and counterproofs (statistical significance level was p < 0.05). Results: aMCl patients were similar to controls on BNT and Similarities, but worse on MMSE and RAVLT. Mild AD patients scored significantly worse than aMCl and controls on all tests. Conclusion: aMCl impairs episodic memory but tends to spare lexical semantic system, which can be affected in the early phase of AD.653A61962

Focal status epilepticus as the first manifestation of paracoccidioidomycosis

We report a 56-year old man with prolonged focal motor status epilepticus as the first clinical manifestation of paracoccidioidomycosis (PCM) and discuss this unusual presentation. We emphasize the need for a comprehensive work-up and increased awareness for central nervous system involvement in PCM, particularly in endemic areas.121737

Whole cortical and default mode network mean functional connectivity as potential biomarkers for mild Alzheimer's disease

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)The search for an Alzheimer's disease (AD) biomarker is one of the most relevant contemporary research topics due to the high prevalence and social costs of the disease. Functional connectivity (PC) of the default mode network (DMN) is a plausible candidate for such a biomarker. We evaluated 22 patients with mild AD and 26 age- and gender-matched healthy controls. All subjects underwent resting functional magnetic resonance imaging (fMRI) in a 3.0 T scanner. To identify the DMN, seed-based FC of the posterior cingulate was calculated. We also measured the sensitivity/specificity of the method, and verified a correlation with cognitive performance. We found a significant difference between patients with mild AD and controls in average z-scores: DMN, whole cortical positive (WCP) and absolute values. DMN individual values showed a sensitivity of 77.3% and specificity of 70%. DMN and WCP values were correlated to global cognition and episodic memory performance. We showed that individual measures of DMN connectivity could be considered a promising method to differentiate AD, even at an early phase, from normal aging. Further studies with larger numbers of participants, as well as validation of normal values, are needed for more definitive conclusions. (C) 2013 Elsevier Ireland Ltd. All rights reserved.22113742Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)FAPESP [2011/17092-0

Coordinated and circumlocutory semantic naming errors are related to anterolateral temporal lobes in mild AD, amnestic mild cognitive impairment, and normal aging

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Naming difficulties are characteristic of Alzheimer s disease (AD) and to a lesser extent, of amnestic mild cognitive impairment (aMCI) patients The association of naming impairment with anterior temporal lobe (ATL) atrophy in Semantic Dementia (SD) could be a tip of the Iceberg effect in which case the atrophy is a marker of more generalized temporal lobe pathology Alternatively, it could reflect the existence of a functional gradient within the temporal lobes wherein more anterior regions provide the basis for greater specificity of representation We tested these two hypotheses in a study of 15 subjects with mild AD 17 with aMCI and 16 aged control subjects and showed that coordinate and circumlocutory semantic error production on the Boston Naming Test was weakly correlated with ATL gray matter density as determined by voxel based morphometry Additionally we investigated whether these errors were benefited by phonemic cues, and similarly to SD, our AD patients had small improvement Because there is minimal gradient of temporal lobe atrophy in AD or MCI and therefore, no basis for a tip of the iceberg effect these findings support the theory of a modest functional gradient in the temporal lobes with the ATLs being Involved in the naming of more specific objects (JINS 2010 16, 1099-1107)o TEXTO COMPLETO DESTE ARTIGO, ESTARÁ DISPONÍVEL À PARTIR DE AGOSTO DE 2015.16610991107Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)FAPESP [2009/02179-2

TRIGEMINO AUTONOMIC CEPHALALGIA AND ARGYLL ROBERTSON PUPIL

66490190

Differences in grey and white matter atrophy in amnestic mild cognitive impairment and mild Alzheimer's disease

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)Grey matter (GM) atrophy has been demonstrated in amnestic mild cognitive impairment (aMCI) and mild Alzheimer's disease (AD), but the role of white matter (WM) atrophy has not been well characterized. Despite these findings, the validity of aMCI concept as prodromal AD has been questioned. We performed brain MRI with voxel-based morphometry analysis in 48 subjects, aiming to evaluate the patterns of GM and WM atrophy amongst mild AD, aMCI and age-matched normal controls. Amnestic mild cognitive impairment GM atrophy was similarly distributed but less intense than that of mild AD group, mainly in thalami and parahippocampal gyri. There were no difference between aMCI and controls concerning WM atrophy. In the mild AD group, we found WM atrophy in periventricular areas, corpus callosum and WM adjacent to associative cortices. We demonstrated that aMCI might be considered a valid concept to detect very early AD pathology, since we found a close proximity in the pattern of atrophy. Also, we showed the involvement of WM in mild AD, but not in aMCI, suggesting a combination of Wallerian degeneration and microvascular ischaemic disease as a plausible additional pathological mechanism for the discrimination between MCI and AD.164468474Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES