RESUMO
Changes in hippocampal subfield volumes (HSV) along the Alzheimer's disease (AD) continuum have been scarcely investigated to date in elderly subjects classified based on the presence of ß-amyloid aggregation and signs of neurodegeneration. We classified patients (either sex) with mild dementia compatible with AD (n = 35) or amnestic mild cognitive impairment (n = 39), and cognitively unimpaired subjects (either sex; n = 26) using [11 C]PIB-PET to assess ß-amyloid aggregation (A+) and [18 F]FDG-PET to account for neurodegeneration ((N)+). Magnetic resonance imaging-based automated methods were used for HSV and white matter hyperintensity (WMH) measurements. Significant HSV reductions were found in A+(N)+ subjects in the presubiculum/subiculum complex and molecular layer, related to worse memory performance. In both the A+(N)+ and A+(N)- categories, subicular volumes were inversely correlated with the degree of Aß deposition. The A-(N)+ subgroup showed reduced HSV relative to the A-(N)- subgroup also in the subiculum/presubiculum. Combining all (N)- subjects, HSV were lower in subjects presenting significant cognitive decline irrespective of A+/A- classification (controlling for WMH load); these between-group differences were detected again in the presubiculum, but also involved the CA4 and granular layer. These findings demonstrate that differential HSV reductions are detectable both in (N)+ and (N)- categories along the AD continuum, and are directly related to the severity of cognitive deficits. HSV reductions are larger both in A+(N)+ and A+(N)- subjects in direct proportion to the degree of Aß deposition. The meaningful HSV reductions detected in the A-(N)+ subgroup highlights the strength of biomarker-based classifications outside of the classical AD continuum.
Assuntos
Doença de Alzheimer/patologia , Peptídeos beta-Amiloides/análise , Disfunção Cognitiva/patologia , Hipocampo/patologia , Neuroimagem , Tomografia por Emissão de Pósitrons , Agregados Proteicos , Idoso , Idoso de 80 Anos ou mais , Doença de Alzheimer/diagnóstico por imagem , Doença de Alzheimer/metabolismo , Compostos de Anilina , Atrofia , Biomarcadores , Radioisótopos de Carbono , Disfunção Cognitiva/diagnóstico por imagem , Disfunção Cognitiva/metabolismo , Feminino , Hipocampo/química , Hipocampo/diagnóstico por imagem , Humanos , Imageamento por Ressonância Magnética , Masculino , Tamanho do Órgão , Compostos Radiofarmacêuticos , Tiazóis , Substância Branca/diagnóstico por imagemRESUMO
The short-term memory binding (STMB) test involves the ability to hold in memory the integration between surface features, such as shapes and colours. The STMB test has been used to detect Alzheimer's disease (AD) at different stages, from preclinical to dementia, showing promising results. The objective of the present study was to verify whether the STMB test could differentiate patients with distinct biomarker profiles in the AD continuum. The sample comprised 18 cognitively unimpaired (CU) participants, 30 mild cognitive impairment (MCI) and 23 AD patients. All participants underwent positron emission tomography (PET) with Pittsburgh compound-B labelled with carbon-11 ([11C]PIB) assessing amyloid beta (Aß) aggregation (A) and 18fluorine-fluorodeoxyglucose ([18F]FDG)-PET assessing neurodegeneration (N) (A-N- [n = 35]); A+N- [n = 11]; A+ N+ [n = 19]). Participants who were negative and positive for amyloid deposition were compared in the absence (A-N- vs. A+N-) of neurodegeneration. When compared with the RAVLT and SKT memory tests, the STMB was the only cognitive task that differentiated these groups, predicting the group outcome in logistic regression analyses. The STMB test showed to be sensitive to the signs of AD pathology and may represent a cognitive marker within the AD continuum.
Assuntos
Doença de Alzheimer , Disfunção Cognitiva , Doença de Alzheimer/diagnóstico por imagem , Peptídeos beta-Amiloides/metabolismo , Encéfalo/diagnóstico por imagem , Disfunção Cognitiva/diagnóstico por imagem , Humanos , Memória de Curto Prazo , Tomografia por Emissão de PósitronsRESUMO
OBJECTIVES: To assess the effect of withdrawal of the antiparkinsonian drug regimen administration on patients with PD and its relation to pain. METHODS: The sample included 22 men and 12 women who were candidates for neurosurgery to control motor signs and symptoms treated with L-dopa as a drug, alone or in combination with others (Cholinergic Antagonists; Dopamine Agents). All of them were examined at two different moments, with and without medication, and analyzed for painful symptoms. The Hoehn and Yahr scale was used for functional staging of the disease. Pain intensity was assessed by using the numerical verbal scale. RESULTS: The mean pain intensity among those on medication {2.17±0.39 (SE)} was significantly lower than in the abstinence group {4.2±0.59 (SE), p=0.006, Wilcoxon}, which corresponded to the increase in the total functional staging score from 93 to 111, respectively. CONCLUSION: The interruption of the administration of specific medications in patients with Parkinson's disease caused, or increased the intensity of, painful discomfort correlated with the intensity of functional impairment. This effect was also observed in women, but it was statistically relevant only for men. The results suggest that pain may be a "red flag" that points to the need for a therapeutic drug review when its presence or worsening is detected.
Assuntos
Doença de Parkinson , Antiparkinsonianos/efeitos adversos , Feminino , Humanos , Levodopa/efeitos adversos , Masculino , Dor/tratamento farmacológico , Dor/etiologia , Doença de Parkinson/tratamento farmacológicoRESUMO
It is a common pathway for patients with the behavioral variant of frontotemporal dementia (bvFTD) to be first misdiagnosed with a primary psychiatric disorder, a considerable proportion of them being diagnosed with bipolar disorder (BD). Conversely, not rarely patients presenting in late life with a first episode of mania or atypically severe depression are initially considered to have dementia before the diagnosis of late-onset BD is reached. Beyond some shared features that make these conditions particularly prone to confusion, especially in the elderly, the relationship between bvFTD and BD is far from simple. Patients with BD often have cognitive complaints as part of their psychiatric disorder but are at an increased risk of developing dementia, including FTD. Likewise, apathy and disinhibition, common features of depression and mania, respectively, are among the core features of the bvFTD syndrome, not to mention that depression may coexist with dementia. In this article, we take advantage of the current knowledge on the neurobiology of these two nosologic entities to review their historical and conceptual interplay, highlighting the clinical, genetic and neuroimaging features that may be shared by both disorders or unique to each of them.
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BACKGROUND: The functional capacity of elderly individuals with Alzheimer disease (AD) progressively declines. OBJECTIVE: To verify the influence of sociodemographic, clinical, staging, mobility, and postural and cognitive balance data on the impairment of the functional capacity of elderly individuals with AD. METHODS: This observational, analytical, cross-sectional study was performed at the Physiotherapy Department of the Universidade Federal do Rio Grande do Norte, Natal, Rio Grande do Norte, Brazil. The study consisted of forty elderly individuals aged ≥60 years old with mild or moderate AD, who could ambulate independently. The instruments used included a questionnaire to assess sociodemographic and anthropometric data; the Mini-Mental Health State Examination (MMSE); the Clinical Dementia Rating (CDR); a clock drawing test (CDT); a verbal fluency test (VFT); the Timed Up and Go Test (TUG); and the Clinical Test of Sensory Organization and Balance (CTSIB). Simple descriptive analyses, Mann-Whitney test, Spearman's correlation test, linear regression modeling, and prediction equation (p<0.05, 95% confidence interval [95%CI]) were performed. RESULTS: Fifteen linear regression models were generated, with the final model chosen for analysis. The variables assumed in that model were CDR, MMSE score, and condition 3 of the CTSIB, which explained 60.1% of the outcome. CONCLUSIONS: Impairment of functional capacity in elderly individuals with AD was influenced by disease progression, which was due to cognitive deficits and deficits in postural balance, which are related to the inaccuracy of the somatosensory system in performing sensory integration.
INTRODUÇÃO: A capacidade funcional de idosos com doença de Alzheimer (DA) sofrerá prejuízo progressivo. OBJETIVO: O presente estudo visou verificar a influências de dados sociodemográficos, clínicos, de estadiamento, mobilidade, equilíbrio postural e cognitivos no prejuízo da capacidade funcional de idosos com DA. MÉTODOS: Trata-se de um estudo observacional, analítico e transversal, realizado no Departamento de Fisioterapia da Universidade Federal do Rio Grande do Norte, em Natal, Rio Grande do Norte, Brasil. O estudo contou com a participação de 40 idosos com idade igual ou superior a 60 anos com DA leve ou moderada, com deambulação independente. Os instrumentos utilizados incluíram um questionário para avaliação de dados sociodemográficos e antropométricos; o Mini-Exame de Estado de Saúde Mental (MEEM); a Avaliação Clínica da Demência (CDR); o Teste do desenho do Relógio (TDR); o Teste de Fluência Verbal (TFV); o Timed Up and Go Test (TUG); e o Clinical Test of Sensory Organization and Balance (CTSIB). Foram realizadas análises descritivas simples, teste de Mann-Whitney, teste de correlação de Spearman, modelo de regressão linear e equação de predição (p<0,05 e intervalo de 95% [IC95%]). RESULTADOS: Foram gerados quinze modelos de regressão linear e foi escolhido o último para a análise. As variáveis assumidas nesse modelo citado foram: CDR, MEEM e condição três do CTSIB, que explicam 60,1% do desfecho. CONCLUSÕES: Pode-se concluir que o prejuízo da capacidade funcional de idosos com DA é influenciado pelo avançar da doença, pelos déficits cognitivo e de equilíbrio postural, sendo esse mais relacionado à imprecisão do sistema somatossensorial em realizar a integração sensorial.
RESUMO
BACKGROUND: The purpose of this study was to determine whether whole-body MRI (WBMRI) with diffusion-weighted sequences, which is free of ionizing radiation, can perform as well as traditional methods when used alone for staging or follow-up of pediatric cancer patients. METHODS: After obtaining approval from our institutional research ethics committee and appropriate informed consent, we performed 34 examinations in 32 pediatric patients. The examinations were anonymized and analyzed by two radiologists with at least 10 years' experience. RESULTS: The sensitivity and specificity findings, respectively, were as follows: 100% and 100% for primary tumor; 100% and 86% for bone metastasis; 33% and 100% for lung metastasis; 85% and 100% for lymph node metastasis; and 100% and 62% for global investigation of primary or secondary neoplasias. We observed excellent interobserver agreement for WBMRI and excellent agreement with standard staging examination results. CONCLUSIONS: Our results suggest that pediatric patients can be safely imaged with WBMRI, although not as the only tool but in association with low-dose chest CT (for subcentimeter pulmonary nodules). However, additional exams with ionizing radiation may be necessary for patients who tested positive to correctly quantify and locate the lesions.
Assuntos
Neoplasias/patologia , Adolescente , Criança , Pré-Escolar , Estudos Transversais , Imagem de Difusão por Ressonância Magnética/métodos , Feminino , Humanos , Masculino , Estadiamento de Neoplasias/métodos , Estudos Prospectivos , Sensibilidade e Especificidade , Imagem Corporal Total/métodosRESUMO
BACKGROUND: Studies of elderly subjects using biomarkers that are proxies for Alzheimer's disease (AD) pathology have the potential to document meaningful relationships between cognitive performance and biomarker changes along the AD continuum. OBJECTIVE: To document cognitive performance differences across distinct AD stages using a categorization based on the presence of PET-assessed amyloid-ß (Aß) burden and neurodegeneration. METHODS: Patients with mild dementia compatible with AD (nâ=â38) or amnestic mild cognitive impairment (aMCI; nâ=â43) and a cognitively unimpaired group (nâ=â27) underwent PET with Pittsburgh compound-B (PiB) assessing Aß aggregation (A+) and [18F]FDG-PET assessing neurodegeneration ((N)+). Cognitive performance was assessed with verbal and visual episodic memory tests and the Mini-Mental State Examination. RESULTS: The A+(N)+ subgroup (nâ=â32) showed decreased (pâ<â0.001) cognitive test scores compared to both A+(N)-(nâ=â18) and A-(N)-(nâ=â49) subjects, who presented highly similar mean cognitive scores. Despite its modest size (nâ=â9), the A-(N)+ subgroup showed lower (pâ<â0.043) verbal memory scores relative to A-(N)-subjects, and trend lower (pâ=â0.096) scores relative to A+(N)-subjects. Continuous Aß measures (standard uptake value ratios of PiB uptake) were correlated most significantly with visual memory scores both in the overall sample and when analyses were restricted to dementia or (N)+ subjects, but not in non-dementia or (N)-groups. CONCLUSION: These results demonstrate that significant Aß-cognition relationships are highly salient at disease stages involving neurodegeneration. The fact that findings relating Aß burden to memory performance were detected only at (N)+ stages, together with the similarity of test scores between A+(N)-and A-(N)-subjects, reinforce the view that Aß-cognition relationships during early AD stages may remain undetectable unless substantially large samples are evaluated.
Assuntos
Peptídeos beta-Amiloides/metabolismo , Disfunção Cognitiva/diagnóstico por imagem , Idoso , Idoso de 80 Anos ou mais , Doença de Alzheimer/diagnóstico por imagem , Compostos de Anilina , Cognição , Feminino , Fluordesoxiglucose F18 , Humanos , Masculino , Memória Episódica , Testes Neuropsicológicos , Tomografia por Emissão de Pósitrons , TiazóisRESUMO
SUMMARY OBJECTIVES: To assess the effect of withdrawal of the antiparkinsonian drug regimen administration on patients with PD and its relation to pain. METHODS: The sample included 22 men and 12 women who were candidates for neurosurgery to control motor signs and symptoms treated with L-dopa as a drug, alone or in combination with others (Cholinergic Antagonists; Dopamine Agents). All of them were examined at two different moments, with and without medication, and analyzed for painful symptoms. The Hoehn and Yahr scale was used for functional staging of the disease. Pain intensity was assessed by using the numerical verbal scale. RESULTS: The mean pain intensity among those on medication {2.17±0.39 (SE)} was significantly lower than in the abstinence group {4.2±0.59 (SE), p=0.006, Wilcoxon}, which corresponded to the increase in the total functional staging score from 93 to 111, respectively. CONCLUSION: The interruption of the administration of specific medications in patients with Parkinson's disease caused, or increased the intensity of, painful discomfort correlated with the intensity of functional impairment. This effect was also observed in women, but it was statistically relevant only for men. The results suggest that pain may be a "red flag" that points to the need for a therapeutic drug review when its presence or worsening is detected.
Assuntos
Humanos , Masculino , Feminino , Doença de Parkinson/tratamento farmacológico , Dor/etiologia , Dor/tratamento farmacológico , Levodopa/efeitos adversos , Antiparkinsonianos/efeitos adversosRESUMO
Neuroplasticity can be conceptualized as an intrinsic property of the brain that enables modification of function and structure in response to environmental demands. Neuroplastic strengthening of synapses is believed to serve as a critical mechanism underlying learning, memory, and other cognitive functions. Ex vivo work investigating neuroplasticity has been done on hippocampal slices using high frequency stimulation. However, in vivo neuroplasticity in humans has been difficult to demonstrate. Recently, a long-term potentiation-like phenomenon, a form of neuroplastic change, was identified in young adults by differences in visual evoked potentials (VEPs) that were measured before and after tetanic visual stimulation (TVS). The current study investigated whether neuroplastic changes in the visual pathway can persist in older adults. Seventeen healthy subjects, 65 years and older, were recruited from the community. Subjects had a mean age of 77.4 years, mean education of 17 years, mean MMSE of 29.1, and demonstrated normal performance on neuropsychological tests. 1Hz checkerboard stimulation, presented randomly to the right or left visual hemi-field, was followed by 2min of 9Hz stimulation (TVS) to one hemi-field. After 2min of rest, 1Hz stimulation was repeated. Temporospatial principal component analysis was used to identify the N1b component of the VEPs, at lateral occipital locations, in response to 1Hz stimulation pre- and post-TVS. Results showed that the amplitude of factors representing the early and late N1b component was substantially larger after tetanic stimulation. These findings indicate that high frequency visual stimulation can enhance the N1b in cognitively high functioning old adults, suggesting that neuroplastic changes in visual pathways can continue into late life. Future studies are needed to determine the extent to which this marker of neuroplasticity is sustained over a longer period of time, and is influenced by age, cognitive status, and neurodegenerative disease.
Assuntos
Envelhecimento/fisiologia , Encéfalo/fisiologia , Potenciais Evocados Visuais/fisiologia , Plasticidade Neuronal/fisiologia , Idoso , Eletroencefalografia , Feminino , Humanos , Masculino , Testes Neuropsicológicos , Estimulação Luminosa/métodos , Análise de Componente Principal , Processamento de Sinais Assistido por ComputadorRESUMO
Infection of the human central nervous system (CNS) by the larvae of Taenia solium, termed neurocysticercosis (NCC), is endemic in most developing countries, where it is a major cause of acquired seizures and other neurological morbidity, including neuropsychiatric symptoms. However, despite its frequent manifestation, some findings, such as cognitive impairment and dementia, remain poorly understood. Less commonly, NCC may affect the ventricular system and subarachnoid spaces and this form is known as extraparenchymal neurocysticercosis. A particular presentation of the subarachnoid form is called racemose cysticercosis, which has a progressive pattern, frequently leads to hydrocephalus and can be life-threatening. Here we review a case of the racemose variety of cysticercosis, complicated by hydrocephalus and reversible dementia, with remission of symptoms after derivation and that remained stable with use of dexchlorpheniramine. We discuss the challenges in diagnosis, imaging findings, treatment and follow-up of this disease.
A infecção do sistema nervoso central (SNC) pela larva da Taenia solium, intitulada neurocisticercose (NCC) é endêmica na maior parte dos países "em desenvolvimento", onde é a principal causa de convulsão adquirida, além de outras morbidades neurológicas, entre elas, sintomas neuropsiquiátricos. No entanto, apesar de manifestações neuropsiquiátricas serem frequentes, alguns achados, tais como comprometimento cognitivo e demência, continuam a ser mal compreendidos. Menos frequentemente, NCC pode afetar o sistema ventricular e espaços subaracnóideos e esta forma é conhecida como NCC extraparenquimatosa. Uma apresentação particular subaracnóidea, chamada cisticercose racemosa, é encontrada mais raramente, evolui de forma progressiva, associada a hidrocefalia e pode levar a morte. Neste artigo revisamos um caso da variedade racemosa de NCC, complicada com hidrocefalia e demência reversível que evoluiu com remissão dos sintomas após derivação ventricular e permaneceu estável com uso de dexclorfeniramina. Discutimos os desafios no diagnóstico, achados de imagem, tratamento e acompanhamento desta forma de doença.
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ABSTRACT. Background: The functional capacity of elderly individuals with Alzheimer disease (AD) progressively declines. Objective: To verify the influence of sociodemographic, clinical, staging, mobility, and postural and cognitive balance data on the impairment of the functional capacity of elderly individuals with AD. Methods: This observational, analytical, cross-sectional study was performed at the Physiotherapy Department of the Universidade Federal do Rio Grande do Norte, Natal, Rio Grande do Norte, Brazil. The study consisted of forty elderly individuals aged ≥60 years old with mild or moderate AD, who could ambulate independently. The instruments used included a questionnaire to assess sociodemographic and anthropometric data; the Mini-Mental Health State Examination (MMSE); the Clinical Dementia Rating (CDR); a clock drawing test (CDT); a verbal fluency test (VFT); the Timed Up and Go Test (TUG); and the Clinical Test of Sensory Organization and Balance (CTSIB). Simple descriptive analyses, Mann-Whitney test, Spearman's correlation test, linear regression modeling, and prediction equation (p<0.05, 95% confidence interval [95%CI]) were performed. Results: Fifteen linear regression models were generated, with the final model chosen for analysis. The variables assumed in that model were CDR, MMSE score, and condition 3 of the CTSIB, which explained 60.1% of the outcome. Conclusions: Impairment of functional capacity in elderly individuals with AD was influenced by disease progression, which was due to cognitive deficits and deficits in postural balance, which are related to the inaccuracy of the somatosensory system in performing sensory integration.
RESUMO. Introdução: A capacidade funcional de idosos com doença de Alzheimer (DA) sofrerá prejuízo progressivo. Objetivo: O presente estudo visou verificar a influências de dados sociodemográficos, clínicos, de estadiamento, mobilidade, equilíbrio postural e cognitivos no prejuízo da capacidade funcional de idosos com DA. Métodos: Trata-se de um estudo observacional, analítico e transversal, realizado no Departamento de Fisioterapia da Universidade Federal do Rio Grande do Norte, em Natal, Rio Grande do Norte, Brasil. O estudo contou com a participação de 40 idosos com idade igual ou superior a 60 anos com DA leve ou moderada, com deambulação independente. Os instrumentos utilizados incluíram um questionário para avaliação de dados sociodemográficos e antropométricos; o Mini-Exame de Estado de Saúde Mental (MEEM); a Avaliação Clínica da Demência (CDR); o Teste do desenho do Relógio (TDR); o Teste de Fluência Verbal (TFV); o Timed Up and Go Test (TUG); e o Clinical Test of Sensory Organization and Balance (CTSIB). Foram realizadas análises descritivas simples, teste de Mann-Whitney, teste de correlação de Spearman, modelo de regressão linear e equação de predição (p<0,05 e intervalo de 95% [IC95%]). Resultados: Foram gerados quinze modelos de regressão linear e foi escolhido o último para a análise. As variáveis assumidas nesse modelo citado foram: CDR, MEEM e condição três do CTSIB, que explicam 60,1% do desfecho. Conclusões: Pode-se concluir que o prejuízo da capacidade funcional de idosos com DA é influenciado pelo avançar da doença, pelos déficits cognitivo e de equilíbrio postural, sendo esse mais relacionado à imprecisão do sistema somatossensorial em realizar a integração sensorial.
Assuntos
Humanos , Pessoas com Deficiência , Equilíbrio Postural , Função Executiva , Doença de AlzheimerRESUMO
BACKGROUND: Aerobic training (AT) is a promising intervention for mild cognitive impairment (MCI). OBJECTIVE: To evaluate the effects of AT on cognition and regional brain glucose metabolism (rBGM) in MCI patients. METHODS: Subjects performed a twice-a-week, moderate intensity, AT program for 24 weeks. Assessment with ADAS-cog, a comprehensive neuropsychological battery, and evaluation of rBGM with positron emission tomography with 18F-fluorodeoxyglucose ([18F]FDG-PET) were performed before and after the intervention. Aerobic capacity was compared using the maximal oxygen consumption VO2max (mL/Kg/min). [18F]FDG-PET data were analyzed on a voxel-by-voxel basis with SPM8 software. RESULTS: Forty subjects were included, with a mean (M) age of 70.3 (5.4) years and an initial Mini-Mental State Exam score of 27.4 (1.7). Comparisons using paired t-tests revealed improvements in the ADAS-cog (M difference: -2.7 (3.7), pâ< â0.001) and VO2max scores (M difference: 1.8 (2.0) mL/kg/min, pâ< â0.001). Brain metabolic analysis revealed a bilateral decrease in the rBGM of the dorsal anterior cingulate cortex, pFWEâ=â0.04. This rBGM decrease was negatively correlated with improvement in a visuospatial function/attentional test (rhoâ=-0.31, pâ=â0.04). Several other brain areas also showed increases or decreases in rBGM. Of note, there was an increase in the retrosplenial cortex, an important node of the default mode network, that was negatively correlated with the metabolic decrease in the dorsal anterior cingulate cortex (râ=-0.51, pâ=â0.001). CONCLUSION: AT improved cognition and changed rBGM in areas related to cognition in subjects with MCI.
Assuntos
Encéfalo/metabolismo , Disfunção Cognitiva/patologia , Disfunção Cognitiva/reabilitação , Terapia por Exercício/métodos , Exercício Físico , Glucose/metabolismo , Idoso , Encéfalo/diagnóstico por imagem , Disfunção Cognitiva/líquido cefalorraquidiano , Feminino , Fluordesoxiglucose F18 , Humanos , Processamento de Imagem Assistida por Computador , Imageamento por Ressonância Magnética , Masculino , Entrevista Psiquiátrica Padronizada , Pessoa de Meia-Idade , Testes Neuropsicológicos , Consumo de Oxigênio , Tomografia por Emissão de PósitronsRESUMO
The spectrum of paraneoplastic neurologic syndromes has increased with the description of encephalitis associated with antibodies against cell surface and synaptic proteins. Subacute cognitive impairment, movement disorders, late onset epilepsy and neuropsychiatric syndromes were recently linked to paraneoplastic encephalitis. Despite that, probably some syndromes and antibodies are yet to be reported. Herein we reported the clinical and neuroimaging pictures of a patient with late onset medial temporal lobe epilepsy, subtle cognitive impairment, psychosis and severe apathy diagnosed with antibody-negative paraneoplastic encephalitis due to colonic adenocarcinoma. The apathy markedly improved after removal of the tumor, without concomitant immunotherapy (steroids, intravenous immunoglobulins, immunosuppressants, plasmapheresis, etc.). Our report highlights the importance of a full clinical and neurologic investigation in cases of atypical neuropsychiatric presentations, particularly in the elderly and with the concomitance of epilepsy and cognitive decline. Even chronic presentations must be considered. Neuroimaging is an important tool to demonstrate structural and functional brain dysfunction in these cases. Colonic adenocarcinoma should be searched for in cases in which a typical tumor related to paraneoplastic neurologic syndromes is not found.
Assuntos
Apatia , Encefalite Límbica/complicações , Síndromes Paraneoplásicas do Sistema Nervoso/complicações , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Fluordesoxiglucose F18 , Humanos , Encefalite Límbica/diagnóstico , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Síndromes Paraneoplásicas do Sistema Nervoso/diagnóstico , Tomografia por Emissão de PósitronsRESUMO
Although not evident clinically, lesions to the prefrontal cortex cause great social and functional impairment to patients. The anterior cingulate cortex is intimately involved with motivational behavior and after injury to this area the onset of an apathetic state can be observed. This paper describes the case of a patient with traumatic brain injury to the prefrontal lobe presenting with a depressive syndrome associated with apathetic symptoms. After appropriate treatment for depression, intense apathy was revealed, an irreversible sequelae of the traumatic brain injury, constituting the main barrier to the patient's return of lifestyle and independence.
Apesar de não se destacarem ao olhar clínico, as lesões do córtex pré-frontal acarretam grande prejuízo funcional e social aos pacientes. O córtex do cíngulo anterior está intimamente envolvido com o comportamento motivacional e o que se observa após um dano a esta região é a instalação de um estado apático. Este trabalho visa relatar o caso de um paciente com lesão traumática do lobo pré-frontal que apresentou uma síndrome depressiva associada a sintomas apáticos. Após tratamento adequado da depressão foi possível perceber intensa apatia, sequela irreversível do traumatismo crânio encefálico, que se constituiu como principal obstáculo ao retorno do estilo de vida e independência do paciente.
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Acquired hepatocerebral degeneration is an underdiagnosed neurologic syndrome characterized by parkinsonism, ataxia or other movement disorders and by neuropsychiatric and cognitive symptoms. It occurs in patients with chronic liver disease, especially those who develop portosystemic shunting and is often unrecognized as a cause of cognitive decline. Recently, its pathogenesis has been associated with manganese accumulation in basal ganglia and some treatments proposed. The aim of this article was to report a case and discuss some discoveries in connection with the disease.
Degeneração hepatocerebral adquirida é uma síndrome neurológica subdiagnosticado caracterizada por parkinsonismo, ataxia ou outros distúrbios do movimento e por sintomas neuropsiquiátricos e cognitivos. Ocorre em pacientes com doença hepática crônica, especialmente aqueles que desenvolvem shunts porto-sistêmicos e frequentemente não é reconhecido como uma causa de declínio cognitivo. Recentemente, sua patogênese tem sido associada ao acúmulo de manganês nos gânglios da base e alguns tratamentos foram propostos. O objetivo do artigo foi relatar um caso e discutir algumas descobertas nesta doença.
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A 35-year-old, previously healthy man presented psychiatric symptoms lasting four years, receiving treatment with neuroleptics. One year later he evolved with gait disequilibrium. After a further six months, cognitive symptoms were characterized with rapid evolution to a profound demented state. MRI showed signal changes in cerebral white matter and very long-chain fatty acids were detected in blood.
Homem de 35 anos, previamente saudável, iniciou há quatro anos sintomas psiquiátricos, recebendo tratamento com neurolépticos. Um ano após evoluiu com alterações do equilíbrio. Há seis meses apresentou distúrbios cognitivos, piorando rapidamente a um estado de profunda demência. RM do encéfalo revelou intensa alteração de sinal na substância branca cerebral e foram detectados ácidos graxos de cadeia muito longa no sangue.
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At times in clinical neurology, the identification of a subtle clinical or radiological sign can lead to prompt diagnosis of a very rare or difficult case. We report on a patient who presented with untreatable headache and unilateral ptosis. Computed tomography (CT) scan of the head did not reveal any structural cause. Magnetic resonance angiogram showed absence of left internal carotid artery, which was eventually confirmed by a catheter angiography. Reviewing the case, it emerged that a feature on the initial CT scan "bone window" would have confirmed the diagnosis, had it been searched for: the underdeveloped carotid canal, which is a consequence and a marker of internal carotid artery agenesis.
Assuntos
Artéria Carótida Interna/anormalidades , Síndrome de Horner/diagnóstico , Adulto , Angiografia Cerebral , Diagnóstico Diferencial , Feminino , Humanos , Imageamento por Ressonância Magnética , Tomografia Computadorizada por Raios XRESUMO
Deep brain stimulation (DBS) has been widely used to control motor symptoms and improve quality of life in patients with Parkinsons disease (PD). Recently, DBS in the subthalamic nucleus (STN) has become the preferred target for patients with mixed motor symptoms. Despite resultant motor and quality of life improvements, the procedure has been associated with cognitive decline, mainly in language skills, and also with psychiatric symptoms. OBJECTIVE: To evaluate the influence of DBS in the STN on cognition, mood and quality of life. METHODS: We studied 20 patients with PD submitted to DBS in the STN from May 2008 to June 2012 with an extensive battery of cognitive tests including memory, language, praxis, executive functions and attention assessments; the Parkinson's Disease Quality of Life Questionnaire (PDQ-39); and the Hospital Anxiety and Depression Scale (HAD), were applied both before and after the surgery. Data was analyzed using SPSS version 17.0 and results compared using the paired Student's t test. RESULTS: A total of 20 patients with pre and post-operative assessments were included. A statistically significant improvement was found in total score and on subscales of mobility, activities of daily living and emotional well-being from the PDQ-39 (P=0.009, 0.025, 0.001 and 0.034, respectively). No significant difference was found on the cognitive battery or mood scale. CONCLUSION: DBS in the SNT improved quality of life in PD with no negative impact on cognitive skills and mood.
Estimulação cerebral profunda tem sido utilizada para controle das alterações motoras e melhorar qualidade de vida dos pacientes com Doença de Parkinson (DP). Mais recentemente, DBS em núcleo subtalâmico (STN) tem sido o alvo preferencialmente escolhido para sintomas mistos. Apesar da melhora motora e da qualidade de vida, o procedimento tem sido associado com declínio cognitivo, principalmente na linguagem e distúrbios psiquiátricos. OBJETIVO: Avaliar a influência do DBS em NST na cognição, humor e qualidade de vida. MÉTODOS: Nós estudamos 20 pacientes submetidos a DBS em NST no período de Maio de 2008 a Junho de 2012, por meio de uma extensa avaliação neuropsicológica incluindo testes de memória, linguagem, praxia, funções executivas, funções atencionais, Parkinsons Disease Quality of Life Questionnaire (PDQ-39) e Escala Hospitalar de depressão e ansiedade (HAD) na fase pré e pós-operatória. Nós analisamos os dados usando o SPSS versão 17.0 e os resultados foram comparados através do teste pareado t-Student. RESULTADOS: Houve melhora estatisticamente significativa no escore total e nas dimensões de mobilidade, atividades de vida diária e bem estar emocional do PDQ-39 (P=0,009, 0,025, 0,001 e 0,034, respectivamente). Diferenças significativas não foram encontradas na bateria cognitiva e nem na escala de humor. CONCLUSÃO: DBS em SNT melhorou a qualidade de vida nos pacientes com DP sem trazer impacto negativo nas funções cognitivas e humor.
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ABSTRACT BACKGROUND AND OBJECTIVES: Neuropathic pain is defined as pain induced by injury or disease involving the somatosensory system. Dysfunctions in anatomic regions responsible for the processing of pain may involve peripheral and central nervous system components. A careful history and clinical evaluation with special attention to neurologic propaedeutics are critical for the syndromic, anatomic and etiologic diagnosis of neuropathic pain. However, diagnosis is not always simple and often depends on additional tests. This chapter aimed at reviewing most commonly used additional tests in the clinical practice to help diagnosing neuropathic pain. CONTENTS: Electroneuromyography is primarily indicated for topographic, etiologic and prognostic diagnosis of peripheral nervous system diseases and for the differential diagnosis between neurogenic, myopathic and neuromuscular junction diseases. It gives real time information on what is going on in the nerve and the muscle, being fundamentally important for differential neuromuscular disease diagnosis. Some imaging methods, such as computerized tomography and magnetic resonance, for their spatial resolution, give details of anatomic structures. Other methods, such as positron emission tomography scan and functional magnetic resonance, in addition to anatomic details, also provide data on metabolic and functional measurements. In addition, imaging techniques such as spectroscopy and diffusion tensor magnetic resonance, allow the study of brain biochemical changes and conectivities with different temporal and spatial resolutions. Other additional tests, such as sensory quantification test and microneurography are seldom used in the clinical practice. CONCLUSION: Additional tests, together with careful history and neurological evaluation focused on neurologic propaedeutics, may provide important data for the diagnosis of neuropathic pain and are often used in the clinical practice.
RESUMO JUSTIFICATIVA E OBJETIVOS: Dor neuropática é definida como a dor causada por lesão ou doença envolvendo o sistema somatossensitivo. Disfunções em regiões anatômicas responsáveis pelo processamento da dor podem envolver componentes do sistema nervoso periférico e central. Uma anamnese cuidadosa e um exame clínico com particular atenção na propedêutica neurológica são fundamentais para o diagnóstico sindrômico, anatômico e etiológico das dores neuropáticas. Entretanto, o diagnóstico nem sempre é simples e frequentemente depende do auxílio de exames complementares. O objetivo deste capítulo foi rever os exames complementares mais usados na prática clínica para o auxílio diagnóstico na dor neuropática. CONTEÚDO: O exame eletroneuromiográfico tem sua principal indicação no diagnóstico topográfico, etiológico e prognóstico das afecções do sistema nervoso periférico e no diagnóstico diferencial entre afecções neurogênicas, miopáticas e da junção neuromuscular. Ele pode fornecer informações em tempo real do que está ocorrendo no nervo e no músculo, sendo de fundamental importância no diagnóstico diferencial das afecções neuromusculares. Existem várias modalidades não invasivas de estudo por imagem que podem auxiliar no diagnóstico de quadros de dores neuropáticas. Alguns métodos por imagem como a tomografia computadorizada, ressonância magnética, pela sua resolução espacial, fornecem detalhamento sobre as estruturas anatômicas. Outros métodos como a tomografia computadorizada por emissão de pósitrons, ressonância magnética funcional fornecem além do detalhamento anatômico, dados sobre mensurações metabólicas e funcionais. Além disso, técnicas de imagem como espectroscopia e tensor de difusão por ressonância magnética, permitem estudar alterações bioquímicas e conectividades cerebrais com diferentes resoluções temporais e espaciais. Outros exames complementares como teste de quantificação sensitiva e microneurografia são pouco utilizados na prática clínica. CONCLUSÃO: Exames complementares, em conjunto com uma anamnese cuidadosa e exame neurológico focado na propedêutica neurológica, podem fornecer dados importantes para o diagnóstico de dor neuropática e são frequentemente utilizados na prática clínica.
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Progressive posterior cortical dysfunction (PPCD) is an insidious syndrome characterized by prominent disorders of higher visual processing. It affects both dorsal (occipito-parietal) and ventral (occipito-temporal) pathways, disturbing visuospatial processing and visual recognition, respectively. We report a case of a 67-year-old woman presenting with progressive impairment of visual functions. Neurologic examination showed agraphia, alexia, hemispatial neglect (left side visual extinction), complete Balint's syndrome and visual agnosia. Magnetic resonance imaging showed circumscribed atrophy involving the bilateral parieto-occipital regions, slightly more predominant to the right. Our aim was to describe a case of this syndrome, to present a video showing the main abnormalities, and to discuss this unusual presentation of dementia. We believe this article can contribute by improving the recognition of PPCD.
Disfunção cortical posterior progressiva (DCPP) é um síndrome insidiosa caracterizada por distúrbios do processamento visual superior. Ela afeta tanto a via dorsal (occipito-parietal) como a ventral (occipito-temporal), alterando o processamento visuoespacial e reconhecimento visual, respectivamente. Nós relatamos o caso de uma mulher de 67 anos com dificuldade progressiva nas funções visuais. O exame neurológico mostrou agraphia, alexia, negligência especial (extinção visual à esquerda), síndrome de Balint complete e agnosia visual. A ressonância magnética mostrou atrofia nas regiões parieto-occipitais, mais evidente à direita. Nosso objetivo foi descrever um caso desta síndrome, apresentar um vídeo mostrando as principais anormalidades e discutir esta apresentação incomum de demência. Nós esperamos que o artigo possa facilitar o reconhecimento da DCPP.