Abnormal eye movements in parkinsonism: a historical view.

Parkinson's disease (PD), known since ancient times as paralysis agitans, was studied and described by James Parkinson in 1817 in his work "An Essay on the Shaking Palsy". Fifty years later, Charcot and his students delved into the disease, naming it as we know it today, as well as defining the classic disease and its variants. One of these students, Arthur Dutil, addressed patients' abnormal eye movements. Nowadays, it is known that the differential diagnosis of PD is relevant for prognosis, treatment and research, and, despite the advances in the area, it remains largely clinical. The relevance of the eye movement exam has grown along with the history of PD and it has proved to be an excellent tool for the differential diagnosis of parkinsonism. Additionally, it can become a support to identify different types of genetic PD and be useful for improving early recognition of cognitive decline in patients with PD.

Abnormal eye movements in parkinsonism: a historical view / Movimentos oculares anormais em parkinsonismo: uma visão histórica

ABSTRACT Parkinson's disease (PD), known since ancient times as paralysis agitans, was studied and described by James Parkinson in 1817 in his work "An Essay on the Shaking Palsy". Fifty years later, Charcot and his students delved into the disease, naming it as we know it today, as well as defining the classic disease and its variants. One of these students, Arthur Dutil, addressed patients' abnormal eye movements. Nowadays, it is known that the differential diagnosis of PD is relevant for prognosis, treatment and research, and, despite the advances in the area, it remains largely clinical. The relevance of the eye movement exam has grown along with the history of PD and it has proved to be an excellent tool for the differential diagnosis of parkinsonism. Additionally, it can become a support to identify different types of genetic PD and be useful for improving early recognition of cognitive decline in patients with PD.

RESUMO A doença de Parkinson (DP), conhecida desde a antiguidade como paralysis agitans, foi estudada e descrita por James Parkinson em 1817 em seu trabalho An Essay on the Shaking Palsy. Cinquenta anos depois, Charcot e seus alunos aprofundaram os estudos na doença, nomeando-a como a conhecemos atualmente, bem como definindo a doença clássica e suas variantes. Um desses estudantes, Arthur Dutil, investigou os movimentos oculares anormais nos pacientes. Hoje, sabe-se que o diagnóstico diferencial da DP é relevante para o prognóstico, tratamento e pesquisa, e, apesar dos grandes avanços na área, ainda permanece amplamente clínico. A relevância do exame de movimentação ocular cresceu com a história da DP e este se mostrou uma excelente ferramenta para o diagnóstico diferencial do parkinsonismo. Além disso, pode se tornar um auxílio para identificar diferentes formas de DP genética e útil para melhorar o reconhecimento precoce do declínio cognitivo em pacientes com DP.

Striatal dopamine correlates to memory and attention in Parkinson's disease.

Parkinson's disease (PD) is clinically characterized by motor symptoms, however, specific cognitive impairments are common and poorly understood. This study was designed to assess whether cognitive performances are related to dopamine active transporter (DAT) availability in non-demented PD subjects. Fifty-four non-demented PD patients were enrolled. They underwent [99mTc] TRODAT-1 SPECT/CT and a comprehensive neuropsychological battery including attention/executive and memory tests. Multiple linear regression controlling the effect of age, disease duration and education was applied. The significance level was set at P values of < 0.02. After controlling the effect of age, disease duration and education, lower scores in Rey's Auditory-Verbal Learning Test (RAVLT)/immediate recall were significantly related with lower uptake values in the less affected striatum and more affected caudate. Lower scores in RAVLT/short-term recall were also significantly associated with lower uptake values in the more affected caudate and reduced performance in Trail Making Test part B was related with reduced DAT values in the less affected anterior putamen. Our findings suggest that reduced DAT availability in both caudate and putamen is related to reduced performances in some memory and attention/executive tasks. Nigrocaudate dysfunction is related to lower memory performance while dopamine depletion in the anterior putamen is related to poorer attention performance. If the dopaminergic defects can mostly explain all the cognitive symptoms or this phenomenon just co-occur with other anatomical and biochemical changes remains unknown. Further studies in larger patient samples are required to clarify this issue.

Spindle-Shaped Neurons in the Human Posteromedial (Precuneus) Cortex.

The human posteromedial cortex (PMC), which includes the precuneus (PC), represents a multimodal brain area implicated in emotion, conscious awareness, spatial cognition, and social behavior. Here, we describe the presence of Nissl-stained elongated spindle-shaped neurons (suggestive of von Economo neurons, VENs) in the cortical layer V of the anterior and central PC of adult humans. The adapted "single-section" Golgi method for postmortem tissue was used to study these neurons close to pyramidal ones in layer V until merging with layer VI polymorphic cells. From three-dimensional (3D) reconstructed images, we describe the cell body, two main longitudinally oriented ascending and descending dendrites as well as the occurrence of spines from proximal to distal segments. The primary dendritic shafts give rise to thin collateral branches with a radial orientation, and pleomorphic spines were observed with a sparse to moderate density along the dendritic length. Other spindle-shaped cells were observed with straight dendritic shafts and rare branches or with an axon emerging from the soma. We discuss the morphology of these cells and those considered VENs in cortical areas forming integrated brain networks for higher-order activities. The presence of spindle-shaped neurons and the current discussion on the morphology of putative VENs address the need for an in-depth neurochemical and transcriptomic characterization of the PC cytoarchitecture. These findings would include these spindle-shaped cells in the synaptic and information processing by the default mode network and for general intelligence in healthy individuals and in neuropsychiatric disorders involving the PC in the context of the PMC functioning.

Dendritic and Spine Heterogeneity of von Economo Neurons in the Human Cingulate Cortex.

The human cingulate cortex (CC), included in the paralimbic cortex, participates in emotion, visceral responses, attention, cognition, and social behaviors. The CC has spindle-shaped/fusiform cell body neurons in its layer V, the von Economo neurons (VENs). VENs have further developed in primates, and the characterization of human VENs can benefit from the detailed descriptions of the shape of dendrites and spines. Here, we advance this issue and studied VENs in the anterior and midcingulate cortex from four neurologically normal adult subjects. We used the thionin technique and the adapted "single-section" Golgi method for light microscopy. Three-dimensional (3D) reconstructions were carried out for the visualization of Golgi-impregnated VENs' cell body, ascending and descending dendrites, and collateral branches. We also looked for the presence, density, and shape of spines from proximal to distal dendrites. These neurons have a similar aspect for the soma, but features of spiny dendrites evidenced a morphological heterogeneity of CC VENs. Only for the description of this continuum of shapes, we labeled the most common feature as VEN 1, which has main dendritic shafts but few branches and sparse spines. VEN 2 shows an intermediate aspect, whereas VEN 3 displays the most profuse dendritic ramification and more spines with varied shapes from proximal to distal branches. Morphometric data exemplify the dendritic features of these cells. The heterogeneity of the dendritic architecture and spines suggests additional functional implications for the synaptic and information processing in VENs in integrated networks of normal and, possibly, neurological/psychiatric conditions involving the human CC.

Neuropathological findings in entorhinal cortex of subjects aged 50 years or older and their correlation with dementia in a sample from Southern Brazil.

INTRODUCTION: The aims of this study were to survey neurodegenerative changes detected by abnormal protein deposits in the Entorhinal Cortex (EC) of subjects aged 50 years or older and to correlate these findings with suspected dementia, as detected by the IQCODE (Informant Questionnaire on Cognitive Decline in the Elderly). METHODS: Fourteen brains were submitted to the immunohistochemistry technique for different proteins (beta-amyloid, tau, α-synuclein and phospho-TDP-43) and data obtained compared with IQCODE scores. RESULTS: Fifty-seven percent of the individuals exhibited IQCODE results compatible with dementia, being classified into the demented group (DG): 87.5% of patients had neuropathological findings corresponding to Alzheimer's-like brain pathology (ALBP). Of the patients in the non-demented group (NDG), 16.7% met neuropathological criteria for ALBP. All individuals in the DG showed deposits of more than one kind of protein in the EC. The most common association was hyperphosphorylated tau and beta-amyloid protein (87.5%). DISCUSSION: Most individuals with dementia had neuropathological findings of ALBP, as did one individual with no signs of dementia, characterizing a preclinical stage. The results of this study suggest that deposits of a single type of anomalous protein are normal findings in an aging brain, while more than one kind of protein or the combined presence of anomalous protein deposits indicate the presence of dementia.

INTRODUÇÃO: Este trabalho visa avaliar alterações neurodegenerativas detectadas por depósitos proteicos anormais em Córtex Entorrinal (CE) de indivíduos acima de 50 anos e correlacionar os achados com suspeição de demência detectada por meio do IQCODE (Informant Questionnaire on Cognitive Decline in the Elderly). MÉTODOS: Catorze encéfalos foram submetidos à técnica imuno-histoquímica para diferentes proteínas (beta-amiloide, tau, alfa-sinucleína e fosfo-TDP-43) e esses dados foram comparados com os valores obtidos pelo IQCODE. RESULTADOS: 57% dos indivíduos mostraram resultados de IQCODE compatíveis com demência, sendo classificados no grupo com demência (GD): 87,5% desses pacientes tinham achados neuropatológicos correspondentes a patologia cerebral Alzheimer-símile (ALBP). Entre os pacientes do grupo sem demência (GSD), 16,7% apresentaram critérios neuropatológicos para ALBP. Todos os indivíduos do GD tinham depósitos de mais de um tipo de proteína no CE. A associação proteica mais comum foi tau hiperfosforilada e proteína beta-amiloide (87,5%). DISCUSSÃO: A maioria dos indivíduos com demência apresentaram achados neuropatológicos de ALBP e um indivíduo, que não tinha evidências de demência, apresentou achados compatíveis com ALBP, caracterizando um estágio pré-clínico. Este trabalho sugere que depósitos de um único tipo de proteína anômala são achados normais do cérebro em envelhecimento, enquanto mais de um tipo de proteínas ou a presença combinada de depósitos proteicos anômalos indica manifestações de demência.

Distinct Phospho-TDP-43 brain distribution in two cases of FTD, one associated with ALS.

INTRODUCTION: TDP-43 is an intranuclear protein involved in many cellular processes. When altered, it shows a change in pattern of distribution, as well as in functioning, throughout the Central Nervous System structures. Frontotemporal Lobar Degeneration (FTLD) and Amyotrophic Lateral Sclerosis (ALS) are examples of TDP-43 proteinopathy. These disorders form a clinical spectrum, with some patients having a pure cognitive disorder while others also exhibit motor features. METHODS: We studied two donated brains from patients with a diagnosis of Frontotemporal Dementia (FTD), one of which was associated with ALS (ALS-FTD). After fixation and macroscopic examinations, sample analyses were performed. Specific regions were chosen for the application of immunohistochemistry (IHC) with anti-Aß, AT8, anti-α-synuclein and anti-phospho-TDP-43. RESULTS: Both brains presented anti-phospho-TDP-43 positivity, but this was not equally distributed throughout the encephalic zones. In the FTD case, the studied brain presented phosphorylated TDP-43- in the frontal cortex, hippocampus, entorhinal cortex and mesencephalon; in the ALS-FTD case, the abnormal protein was also seen in the pons and medulla oblongata. The brain in the ALS-FTD case presented Aß and AT8 positivity in the hippocampus and entorhinal cortex (Braak I and II). DISCUSSION: The hypothesis supported by scientific literature that these neurodegenerative diseases can have the same etiology with distinct encephalic region involvement is corroborated by the present study.

INTRODUÇÃO: TDP-43 é uma proteína intranuclear envolvida em vários processos celulares. Essa molécula, quando alterada, mostra padrões de distribuição modificados, assim como de funcionamento, ao longo das estruturas do Sistema Nervoso Central. A Degeneração Lobar Frontotemporal (DLFT) e a Esclerose Lateral Amiotrófica (ELA) são dois exemplos de proteinopatia de TDP-43. Esses transtornos formam um espectro clínico, com alguns pacientes apresentando um transtorno cognitivo puro enquanto outros também apresentam disfunções motoras. MÉTODOS: Nós estudamos dois cérebros doados de pacientes com diagnóstico de Demência Frontotemporal (DFT), um dos quais se associava com ELA (ELA-DFT). Após fixação e exame macroscópico, foram realizadas análises de amostras. Regiões específicas foram escolhidas para aplicação de imunohistoquímica (IHQ) com anti-Aß, AT8, anti-α-sinucleina e anti-fosfo-TDP-43. RESULTADOS: Ambos os cérebros foram positivos para anti-fosfo-TDP-43, mas de forma não igualmente distribuida pelas regiões encefálicas. No caso DFT, o cérebro estudado apresentou TDP-43-fosforilada no córtex frontal, hipocampo, córtex entorrinal e mesencéfalo; no caso ELA-DFT, a proteína anormal também foi vista na ponte e no bulbo. O cérebro do caso ELA-DFT foi positivo para Aß e AT8 no hipocampo e no córtex entorrinal (Braak I e II). DISCUSSÃO: O presente estudo corrobora a hipótese atualmente sustentada pela literatura científica de que essas duas doenças neurodegenerativas possuem a mesma etiologia, mas acometem regiões encefálicas distintas.

Distinct Phospho-TDP-43 brain distribution in two cases of FTD, one associated with ALS / Distribuição cerebral distinta de fosfo-tdp-43 em dois casos de dft, um associado com ela

ABSTRACT. INTRODUCTION: TDP-43 is an intranuclear protein involved in many cellular processes. When altered, it shows a change in pattern of distribution, as well as in functioning, throughout the Central Nervous System structures. Frontotemporal Lobar Degeneration (FTLD) and Amyotrophic Lateral Sclerosis (ALS) are examples of TDP-43 proteinopathy. These disorders form a clinical spectrum, with some patients having a pure cognitive disorder while others also exhibit motor features. METHODS: We studied two donated brains from patients with a diagnosis of Frontotemporal Dementia (FTD), one of which was associated with ALS (ALS-FTD). After fixation and macroscopic examinations, sample analyses were performed. Specific regions were chosen for the application of immunohistochemistry (IHC) with anti-Aß, AT8, anti-α-synuclein and anti-phospho-TDP-43. RESULTS: Both brains presented anti-phospho-TDP-43 positivity, but this was not equally distributed throughout the encephalic zones. In the FTD case, the studied brain presented phosphorylated TDP-43- in the frontal cortex, hippocampus, entorhinal cortex and mesencephalon; in the ALS-FTD case, the abnormal protein was also seen in the pons and medulla oblongata. The brain in the ALS-FTD case presented Aß and AT8 positivity in the hippocampus and entorhinal cortex (Braak I and II). DISCUSSION: The hypothesis supported by scientific literature that these neurodegenerative diseases can have the same etiology with distinct encephalic region involvement is corroborated by the present study.

RESUMO. INTRODUÇÃO: TDP-43 é uma proteína intranuclear envolvida em vários processos celulares. Essa molécula, quando alterada, mostra padrões de distribuição modificados, assim como de funcionamento, ao longo das estruturas do Sistema Nervoso Central. A Degeneração Lobar Frontotemporal (DLFT) e a Esclerose Lateral Amiotrófica (ELA) são dois exemplos de proteinopatia de TDP-43. Esses transtornos formam um espectro clínico, com alguns pacientes apresentando um transtorno cognitivo puro enquanto outros também apresentam disfunções motoras. MÉTODOS: Nós estudamos dois cérebros doados de pacientes com diagnóstico de Demência Frontotemporal (DFT), um dos quais se associava com ELA (ELA-DFT). Após fixação e exame macroscópico, foram realizadas análises de amostras. Regiões específicas foram escolhidas para aplicação de imunohistoquímica (IHQ) com anti-Aß, AT8, anti-α-sinucleina e anti-fosfo-TDP-43. RESULTADOS: Ambos os cérebros foram positivos para anti-fosfo-TDP-43, mas de forma não igualmente distribuida pelas regiões encefálicas. No caso DFT, o cérebro estudado apresentou TDP-43-fosforilada no córtex frontal, hipocampo, córtex entorrinal e mesencéfalo; no caso ELA-DFT, a proteína anormal também foi vista na ponte e no bulbo. O cérebro do caso ELA-DFT foi positivo para Aß e AT8 no hipocampo e no córtex entorrinal (Braak I e II). DISCUSSÃO: O presente estudo corrobora a hipótese atualmente sustentada pela literatura científica de que essas duas doenças neurodegenerativas possuem a mesma etiologia, mas acometem regiões encefálicas distintas.

Using body donor demographics to assist the implementation of donation programs in Brazil.

The use of human material in anatomy education depends upon the generosity of body donors. However, little is known regarding the demographics of body donors in Brazil, where voluntary body donation is a relatively rare phenomenon. Hence, the aim of the present study was to elucidate the demographic profile of applicants to the Body Donation Program (BDP) at the Federal University of Health Sciences of Porto Alegre in Brazil, as well as to assess whether the observed characteristics of body donors are unique to that sample, or if they merely reflect the characteristics of the regional population. Information derived from the specific forms filled out by donors between January 2008 and June 2016 at the time of registration were collected. Data from 416 forms were analyzed. Based on this study, the typical applicant in Brazil is typically a white female (67.4%), over 60 years of age (60.3%), unmarried or single (70.6%), affiliated with a religious group (89.1%), of middle class background (40.4%), who has completed high school and/or holds a university degree (93.8%). The motivation of donors was, in most cases, an altruistic gesture, represented by the desire to help society and science. Elucidating these demographic characteristics of potential donors may help identify the target public to which information regarding body donation campaigns could be directed. Anat Sci Educ 10: 475-486. © 2017 American Association of Anatomists.

Neuropathological findings in entorhinal cortex of subjects aged 50 years or older and their correlation with dementia in a sample from Southern Brazil / Achados neuropatológicos no córtex entorrinal de encéfalos de indivíduos acima de 50 anos e sua correlação com demência numa amostra do Sul do Brasil

ABSTRACT. Introduction: The aims of this study were to survey neurodegenerative changes detected by abnormal protein deposits in the Entorhinal Cortex (EC) of subjects aged 50 years or older and to correlate these findings with suspected dementia, as detected by the IQCODE (Informant Questionnaire on Cognitive Decline in the Elderly) . Methods: Fourteen brains were submitted to the immunohistochemistry technique for different proteins (beta-amyloid, tau, -synuclein and phospho-TDP-43) and data obtained compared with IQCODE scores. Results: Fifty-seven percent of the individuals exhibited IQCODE results compatible with dementia, being classified into the demented group (DG): 87.5% of patients had neuropathological findings corresponding to Alzheimer's-like brain pathology (ALBP). Of the patients in the non-demented group (NDG), 16.7% met neuropathological criteria for ALBP. All individuals in the DG showed deposits of more than one kind of protein in the EC. The most common association was hyperphosphorylated tau and beta-amyloid protein (87.5%). Discussion: Most individuals with dementia had neuropathological findings of ALBP, as did one individual with no signs of dementia, characterizing a preclinical stage. The results of this study suggest that deposits of a single type of anomalous protein are normal findings in an aging brain, while more than one kind of protein or the combined presence of anomalous protein deposits indicate the presence of dementia.

RESUMO. Introdução: Este trabalho visa avaliar alterações neurodegenerativas detectadas por depósitos proteicos anormais em Córtex Entorrinal (CE) de indivíduos acima de 50 anos e correlacionar os achados com suspeição de demência detectada por meio do IQCODE (Informant Questionnaire on Cognitive Decline in the Elderly). Métodos: Catorze encéfalos foram submetidos à técnica imuno-histoquímica para diferentes proteínas (beta-amiloide, tau, alfa-sinucleína e fosfo-TDP-43) e esses dados foram comparados com os valores obtidos pelo IQCODE. Resultados: 57% dos indivíduos mostraram resultados de IQCODE compatíveis com demência, sendo classificados no grupo com demência (GD): 87,5% desses pacientes tinham achados neuropatológicos correspondentes a patologia cerebral Alzheimer-símile (ALBP). Entre os pacientes do grupo sem demência (GSD), 16,7% apresentaram critérios neuropatológicos para ALBP. Todos os indivíduos do GD tinham depósitos de mais de um tipo de proteína no CE. A associação proteica mais comum foi tau hiperfosforilada e proteína beta-amiloide (87,5%). Discussão: A maioria dos indivíduos com demência apresentaram achados neuropatológicos de ALBP e um indivíduo, que não tinha evidências de demência, apresentou achados compatíveis com ALBP, caracterizando um estágio pré-clínico. Este trabalho sugere que depósitos de um único tipo de proteína anômala são achados normais do cérebro em envelhecimento, enquanto mais de um tipo de proteínas ou a presença combinada de depósitos proteicos anômalos indica manifestações de demência.

Neuropsychological profile of Parkinson's disease patients selected for deep brain stimulation surgery / Perfil neuropsicológico do paciente com doença de Parkinson selecionado para cirurgia de estimulação cerebral profunda

ABSTRACT Background: Parkinson's disease (PD) shows symptoms involving motor and non-motor complications, including cognitive and behavioral changes, such changes might to contraindicate deep brain stimulation surgery (DBS). Objective: The aim of study was to investigate the neuropsychological profile of patients with PD in a waiting list for DBS. Methods: The neuropsychological evaluation was held in 30 patients of the ISCMPA Movement Disorders Clinic, with surgical indication based on the criteria of the responsible neurologists, in the period of 12 months. Instruments used: MMSE, FAB, MoCA, BDI, Semantic Verbal Fluency, PDQ-39, PDSS; and the UPDRS and Hoehn-Yahr scale. Results: The patients were mostly male (66.7%) with a mean age of 59.37 (SD 10.60) and disease duration 9.33 (SD 4.08). There was cognitive impairment in 56.7% of patients by FAB and 76.7% by MoCA. Conclusion: Even in the earliest stages of the disease, there is the incidence of non-motor symptoms, especially in those subjects who had an early onset of the disease.

RESUMO Embasamento: A doença de Parkinson (DP) apresenta sintomas que envolvem complicações motoras e não-motoras, entre elas alterações cognitivas e comportamentais, tais alterações podem contraindicar a cirurgia de estimulação cerebral profunda (DBS). Objetivo: O objetivo de estudo foi verificar o perfil neuropsicológico dos pacientes com DP candidatos à DBS. Métodos: Realizou-se avaliação neuropsicológica em 30 pacientes do ambulatório de Distúrbios do Movimento da ISCMPA, com indicação cirúrgica segundo critério dos neurologistas responsáveis, no período de 12 meses. Os instrumentos utilizados: MEEM, FAB, MoCA, BDI, FV Semântica, PDQ-39, PDSS; e as escalas UPDRS e Hoehn-Yahr. Resultados: Os pacientes foram, em sua maioria, homens (66,7%), com média de idade de 59,37 (DP 10,6) e tempo de doença 9,33 (DP 4,0). Apresentaram comprometimento cognitivo em 56,7% dos pacientes segundo os escores do FAB e 76,7% segundo o MoCA. Conclusão: Mesmo em fases mais iniciais da doença, percebe-se a incidência de sintomas não motores, principalmente naqueles sujeitos que tiveram um início de doença precoce da doença.

Evaluation of Knee Proprioception and Factors Related to Parkinson's Disease.

Background. Changes in proprioception may contribute to postural instability in individuals with neurological disorders. Objectives. Evaluate proprioception in the lower limbs of patients with Parkinson's disease (PD) and the association between proprioception and cognitive ability, motor symptoms, postural instability, and disease severity. Methods. This is a cross-sectional, controlled study that evaluated proprioception in PD patients and healthy age- and sex-matched individuals. Kinetic postural proprioception of the knee was evaluated using an isokinetic dynamometer (Biodex® Multi-Joint System 4 Pro). Participants were evaluated using the Montreal Cognitive Assessment (MoCA), the Hoehn and Yahr rating scale and postural instability (pull test and stabilometric analysis), and motor function (UPDRS-III) tests. Results. A total of 40 individuals were enrolled in the study: 20 PD patients and 20 healthy controls (CG). The PD patients had higher angular errors on the proprioceptive ratings than the CG participants (p = 0.002). Oscillations of the center of pressure (p = 0.002) were higher in individuals with PD than in the controls. Proprioceptive errors in the PD patients were associated with the presence of tremors as the dominant symptom and more impaired motor performance. Conclusion. These findings show that individuals with PD have proprioceptive deficits, which are related to decreased cognitive ability and impaired motor symptoms.

Neuropsychological profile of Parkinson's disease patients selected for deep brain stimulation surgery.

BACKGROUND: Parkinson's disease (PD) shows symptoms involving motor and non-motor complications, including cognitive and behavioral changes, such changes might to contraindicate deep brain stimulation surgery (DBS). OBJECTIVE: The aim of study was to investigate the neuropsychological profile of patients with PD in a waiting list for DBS. METHODS: The neuropsychological evaluation was held in 30 patients of the ISCMPA Movement Disorders Clinic, with surgical indication based on the criteria of the responsible neurologists, in the period of 12 months. Instruments used: MMSE, FAB, MoCA, BDI, Semantic Verbal Fluency, PDQ-39, PDSS; and the UPDRS and Hoehn-Yahr scale. RESULTS: The patients were mostly male (66.7%) with a mean age of 59.37 (SD 10.60) and disease duration 9.33 (SD 4.08). There was cognitive impairment in 56.7% of patients by FAB and 76.7% by MoCA. CONCLUSION: Even in the earliest stages of the disease, there is the incidence of non-motor symptoms, especially in those subjects who had an early onset of the disease.

EMBASAMENTO: A doença de Parkinson (DP) apresenta sintomas que envolvem complicações motoras e não-motoras, entre elas alterações cognitivas e comportamentais, tais alterações podem contraindicar a cirurgia de estimulação cerebral profunda (DBS). OBJETIVO: O objetivo de estudo foi verificar o perfil neuropsicológico dos pacientes com DP candidatos à DBS. MÉTODOS: Realizou-se avaliação neuropsicológica em 30 pacientes do ambulatório de Distúrbios do Movimento da ISCMPA, com indicação cirúrgica segundo critério dos neurologistas responsáveis, no período de 12 meses. Os instrumentos utilizados: MEEM, FAB, MoCA, BDI, FV Semântica, PDQ-39, PDSS; e as escalas UPDRS e Hoehn-Yahr. RESULTADOS: Os pacientes foram, em sua maioria, homens (66,7%), com média de idade de 59,37 (DP 10,6) e tempo de doença 9,33 (DP 4,0). Apresentaram comprometimento cognitivo em 56,7% dos pacientes segundo os escores do FAB e 76,7% segundo o MoCA. CONCLUSÃO: Mesmo em fases mais iniciais da doença, percebe-se a incidência de sintomas não motores, principalmente naqueles sujeitos que tiveram um início de doença precoce da doença.

Evaluation of quality of life and psychological aspects of Parkinson's disease patients who participate in a support group / Avaliação da qualidade de vida e aspectos psicológicos de pacientes com doença de parkinson participantes de um grupo de apoio

ABSTRACT. Parkinson's disease (PD) is a neurodegenerative disorder that can dramatically impair patient quality of life (QoL). Objective: To analyze the QoL, motor capacity, depression, anxiety and social phobia of individuals who attended a patient support group (PSG) compared to non-participants. Methods: A cross-sectional study was performed. The sample consisted of 20 individuals with PD who attended a PSG and another 20 PD patients who did not attend a support group for PD patients, serving as the control group (nPSG). All patients answered questionnaires on motor capacity (UPDRS), QoL (Parkinson's Disease Questionnaire- PDQ-39), depression (Beck Depression Inventory), anxiety (Beck Anxiety Inventory) and social phobia (Liebowitz Social Anxiety Scale). To determine data distribution, the Shapiro-Wilk test was performed. For comparison of means, Student's t-test was applied. In cases of asymmetry, the Mann-Whitney test was employed. To assess the association between the scales, Pearson's correlation coefficient (symmetric distribution) and Spearman's coefficient (asymmetric distribution) were applied. For the association between qualitative variables, Pearson's Chi-squared test was performed. A significance level of 5% (p≤0.05) was adopted. Results: Individuals in the PSG had a significantly better QoL (p=0.002), and lower depression (p=0.026), anxiety (p<0.001) and social phobia (p=0.01) scores compared to the nPSG. Conclusion: The participation of PD patients in social activities such as support groups is associated with better QoL and fewer symptoms of depression, anxiety and social phobia.

RESUMO. A doença de Parkinson (DP) é uma doença neurodegenerativa que pode prejudicar a qualidade de vida (QV) do paciente. Objetivo: Analisar a qualidade de vida, capacidade motora, depressão, ansiedade e fobia social dos indivíduos que frequentavam um grupo de apoio a pacientes (PSG) em comparação com aqueles que não frequentavam. Métodos: Tratase de um estudo transversal. Amostra composta por 20 indivíduos com Parkinson que participavam de uma PSG e outros 20 pacientes com DP que não participavam de qualquer grupo de apoio para pacientes com DP, denominado de grupo de controle (nPSG). Os participantes responderam questionários sobre capacidade motora (UPDRS), QV (PDQ-39), depressão (Inventário de depressão de Beck), ansiedade (Inventário de ansiedade de Beck) e fobia social (Escala de Ansiedade Social de Liebowitz). Determinando a distribuição dos dados, foi realizado o teste de Shapiro-Wilk. Comparando médias, teste t de Student foi aplicado. Em assimetria, foi realizado o teste de Mann-Whitney. Avaliando a associação entre as escalas, o coeficiente de correlação de Pearson (distribuição simétrica) e o coeficiente de Spearman (distribuição assimétrica) foram aplicados. Para a associação entre as variáveis qualitativas, foi realizado o teste qui-quadrado de Pearson. Adotado o nível de significância de 5% (p≤0,05). Resultados: Os indivíduos do PSG apresentaram uma qualidade de vida significativamente melhor (p=0,002), menos depressão (p=0,026), ansiedade (p<0,001) e fobia social (p=0,01) quando comparado com o nPSG. Conclusão: A participação de pacientes com DP em atividades sociais como os grupos de apoio está associado a melhor qualidade de vida e menor presença de sintomas de depressão, ansiedade e fobia social.

Neurodegenerative changes in the brainstem and olfactory bulb in people older than 50 years old: a descriptive study.

With the increase in life expectancy in Brazil, concerns have grown about the most prevalent diseases in elderly people. Among these diseases are neurodegenerative diseases, such as Alzheimer's and Parkinson's diseases. Protein deposits related to the development of these diseases can pre-date the symptomatic phases by years. The tau protein is particularly interesting: it might be found in the brainstem and olfactory bulb long before it reaches the limbic cortex, at which point symptoms occur. Of the 14 brains collected in this study, the tau protein was found in the brainstems of 10 (71.42%) and in olfactory bulbs of 3 out 11. Of the 7 individuals who had a final diagnosis of Alzheimer's disease (AD), 6 presented tau deposits in some region of the brainstem. Our data support the idea of the presence of tau protein in the brainstem and olfactory bulb in the earliest stages of AD.

Neurodegenerative changes in the brainstem and olfactory bulb in people older than 50 years old: a descriptive study / Alterações neurodegenerativas no tronco cerebral e bulbo olfatório em indivíduos acima de 50 anos: um estudo descritivo

With the increase in life expectancy in Brazil, concerns have grown about the most prevalent diseases in elderly people. Among these diseases are neurodegenerative diseases, such as Alzheimer’s and Parkinson’s diseases. Protein deposits related to the development of these diseases can pre-date the symptomatic phases by years. The tau protein is particularly interesting: it might be found in the brainstem and olfactory bulb long before it reaches the limbic cortex, at which point symptoms occur. Of the 14 brains collected in this study, the tau protein was found in the brainstems of 10 (71.42%) and in olfactory bulbs of 3 out 11. Of the 7 individuals who had a final diagnosis of Alzheimer’s disease (AD), 6 presented tau deposits in some region of the brainstem. Our data support the idea of the presence of tau protein in the brainstem and olfactory bulb in the earliest stages of AD.

Com o aumento da expectativa de vida no Brasil e no mundo, crescem as preocupações com as doenças mais prevalentes entre os idosos, dentre elas as doenças neurodegenerativas (DN) como a doença de Alzheimer (DA) e a doença de Parkinson (DP). Sabe-se que os depósitos proteicos relacionados com o desenvolvimento destas doenças podem preceder a fase sintomática em anos. A proteína tau é de particular interesse, uma vez que parece ser encontrada no tronco encefálico e bulbo olfatório muito antes de atingir o córtex límbico, quando ocorrem os primeiros sintomas. Dos 14 encéfalos coletados neste estudo, a proteína tau foi encontrada, no tronco encefálico, em 10 (71,42%) e no bulbo olfatório em 3 de 11. Dos 7 indivíduos que tiveram diagnóstico final de DA, todos apresentavam depósitos de tau em alguma região do tronco encefálico. Nossos dados estão de acordo com a literatura mais recente, que tem confirmado a presença de proteína tau no tronco encefálico e bulbo olfatório nos estágios mais precoces da DA.

Evaluation of quality of life and psychological aspects of Parkinson's disease patients who participate in a support group.

Parkinson's disease (PD) is a neurodegenerative disorder that can dramatically impair patient quality of life (QoL). OBJECTIVE: To analyze the QoL, motor capacity, depression, anxiety and social phobia of individuals who attended a patient support group (PSG) compared to non-participants. METHODS: A cross-sectional study was performed. The sample consisted of 20 individuals with PD who attended a PSG and another 20 PD patients who did not attend a support group for PD patients, serving as the control group (nPSG). All patients answered questionnaires on motor capacity (UPDRS), QoL (Parkinson's Disease Questionnaire- PDQ-39), depression (Beck Depression Inventory), anxiety (Beck Anxiety Inventory) and social phobia (Liebowitz Social Anxiety Scale). To determine data distribution, the Shapiro-Wilk test was performed. For comparison of means, Student's t-test was applied. In cases of asymmetry, the Mann-Whitney test was employed. To assess the association between the scales, Pearson's correlation coefficient (symmetric distribution) and Spearman's coefficient (asymmetric distribution) were applied. For the association between qualitative variables, Pearson's Chi-squared test was performed. A significance level of 5% (p≤0.05) was adopted. RESULTS: Individuals in the PSG had a significantly better QoL (p=0.002), and lower depression (p=0.026), anxiety (p<0.001) and social phobia (p=0.01) scores compared to the nPSG. CONCLUSION: The participation of PD patients in social activities such as support groups is associated with better QoL and fewer symptoms of depression, anxiety and social phobia.

A doença de Parkinson (DP) é uma doença neurodegenerativa que pode prejudicar a qualidade de vida (QV) do paciente. OBJETIVO: Analisar a qualidade de vida, capacidade motora, depressão, ansiedade e fobia social dos indivíduos que frequentavam um grupo de apoio a pacientes (PSG) em comparação com aqueles que não frequentavam. MÉTODOS: Trata-se de um estudo transversal. Amostra composta por 20 indivíduos com Parkinson que participavam de uma PSG e outros 20 pacientes com DP que não participavam de qualquer grupo de apoio para pacientes com DP, denominado de grupo de controle (nPSG). Os participantes responderam questionários sobre capacidade motora (UPDRS), QV (PDQ-39), depressão (Inventário de depressão de Beck), ansiedade (Inventário de ansiedade de Beck) e fobia social (Escala de Ansiedade Social de Liebowitz). Determinando a distribuição dos dados, foi realizado o teste de Shapiro-Wilk. Comparando médias, teste t de Student foi aplicado. Em assimetria, foi realizado o teste de Mann-Whitney. Avaliando a associação entre as escalas, o coeficiente de correlação de Pearson (distribuição simétrica) e o coeficiente de Spearman (distribuição assimétrica) foram aplicados. Para a associação entre as variáveis qualitativas, foi realizado o teste qui-quadrado de Pearson. Adotado o nível de significância de 5% (p≤0,05). RESULTADOS: Os indivíduos do PSG apresentaram uma qualidade de vida significativamente melhor (p=0,002), menos depressão (p=0,026), ansiedade (p<0,001) e fobia social (p=0,01) quando comparado com o nPSG. CONCLUSÃO: A participação de pacientes com DP em atividades sociais como os grupos de apoio está associado a melhor qualidade de vida e menor presença de sintomas de depressão, ansiedade e fobia social.

Protein intake and the use of levodopa in patients with Parkinson's disease / Ingesta de proteínas y el uso de la levodopa en pacientes con enfermedad de Parkinson

Levodopa is the principal pharmacological treatment for Parkinson's disease (PD); however the protein content in diet may compromise its effectiveness. The aim of this study was to investigate the relationship of protein intake and the use of levodopa in ambulatory patients with PD. Thirty four PD patients were assessed for protein intake, evaluating the relation energy-protein intake by means of a register. An intake of 1762 kcal/day, 70.9 g of protein/day equivalent to 16.6% of the total energy intake as protein at a level of 1.1 g/kg bw/day. Of all patients, 47.1% took their medications with meals. We conclude that the studied patients consume more protein in their diet than those recommended for PD, with half the sample taking the medications with meals, which may affect the treatment.

La levodopa es el principal tratamiento farmacológico para la enfermedad de Parkinson, sin embargo, la proteína de la dieta puede comprometer su eficacia. El objetivo de este estudio fue investigar la ingesta de proteínas y el uso de la levodopa en pacientes con enfermedad de Parkinson tratados en régimen ambulatorio, 34 pacientes fueron evaluados. Evaluación de la ingesta de alimentos por los registros, tomas de 1762 kcal/día, 70.9 g/día, el 16,6% del total de energía y 1.1 g/kg/dia de energía y proteína, respectivamente, y que la ingesta de proteínas durante el día era 70,4% del total de proteínas diario. Todos los pacientes estaban tomando levodopa y 47,1% consumían sus medicamentos con las comidas. Se concluyó que, según las recomendaciones para la enfermedad de Parkinson, los pacientes estudiados consumían una dieta rica en proteínas y con una mayor concentración de este nutriente durante el día. Aproximadamente la mitad de la muestra ingiere los medicamentos con la comida, costumbre que puede afectar el tratamiento farmacológico de la enfermedad.

Cellular components of the human medial amygdaloid nucleus.

The medial nucleus (Me) is a superficial component of the amygdaloid complex. Here we assessed the density and morphology of the neurons and glial cells, the glial fibrillary acidic protein (GFAP) immunoreactivity, and the ultrastructure of the synaptic sites in the human Me. The optical fractionator method was applied. The Me presented an estimated mean neuronal density of 1.53 × 105 neurons/mm³ (greater in the left hemisphere), more glia (72% of all cells) than neurons, and a nonneuronal:neuronal ratio of 2.7. Golgi-impregnated neurons had round or ovoid, fusiform, angular, and polygonal cell bodies (10-30 µm in diameter). The length of the dendrites varied, and pleomorphic spines were found in sparsely spiny or densely spiny cells (1.5-5.2 spines/dendritic µm). The axons in the Me neuropil were fine or coarsely beaded, and fibers showed simple or notably complex collateral terminations. The protoplasmic astrocytes were either isolated or formed small clusters and showed GFAP-immunoreactive cell bodies and multiple branches. Furthermore, we identified both asymmetrical (with various small, clear, round, electron-lucent vesicles and, occasionally, large, dense-core vesicles) and symmetrical (with small, flattened vesicles) axodendritic contacts, also including multisynaptic spines. The astrocytes surround and may compose tripartite or tetrapartite synapses, the latter including the extracellular matrix between the pre- and the postsynaptic elements. Interestingly, the terminal axons exhibited a glomerular-like structure with various asymmetrical contacts. These new morphological data on the cellular population and synaptic complexity of the human Me can contribute to our knowledge of its role in health and pathological conditions.

Evaluation of the expression of C-kit (CD117) in ependymomas and oligodendrogliomas.

C-kit is a proto-oncogene located on the long arm of chromosome 4. Its product, CD117, is a specific immunohistochemical (IHQ) marker that is associated with response to a potent tyrosine kinase inhibitor therapy with STI-571 (Gleevec®) in chronic myelogenous leukemia and GISTs. In our study, we aimed to evaluate the expression of CD117 in glial tumors as this finding may guide therapeutic approaches for these brain tumors. Ependymomas and oligodendrogliomas, in formalin fixed and paraffin embedded blocks were assayed for CD117 immunoreactivity using anti-c-kit (CD117, DAKO). GISTs were used as positive control. We observed immunoreactivity of CD117 protein in 25.5% of tumors in both histological types. In oligodendrogliomas, there was an association between older age at diagnosis and positivity for CD117 (P=0.039). In addition, we observed an association between higher tumor grade (grade III) and positivity for CD117 (P=0.007). No clinical association was observed in ependymomas (P>0.05). This study encourages further investigations, considering that CD117 may be a possible oncogenic factor in some glial tumors. In this case, tumors that express this marker may eventually benefit from a therapy with selective inhibitors of receptor kinases.