Perfil epidemiológico dos casos de hanseníase em um estado do nordeste brasileiro de 2018 a 2022 / Epidemiological profile of the cases of leprosy in a state in the northeast of brazil from 2018 to 2022 / Perfil epidemiológico de los casos de hanseniasis en un estado del noreste de brasil de 2018 a 2022

A hanseníase é uma doença crônica, infectocontagiosa, de notificação compulsória e investigação obrigatória no Brasil, de evolução lenta, causada pelo bacilo Mycobacterium leprae que acomete predominantemente pele e nervos periféricos causando incapacidades funcionais permanentes de diversos graus, além de ser uma patologia ainda cercada por estigma e discriminação social. O objetivo do artigo consiste em avaliar o perfil epidemiológico da hanseníase no Estado do Rio Grande do Norte entre 2018 a 2022, estado que ocupa, atualmente, o 3° lugar a nível nacional de registro geral de casos de hanseníase. Trata-se de um estudo epidemiológico, descritivo, retrospectivo e de abordagem quantitativa, com base em dados obtidos no Departamento de Informática do Sistema Único de Saúde (DATASUS) e analisados com auxílio do software livre Jamovi 2.3.21 solid. O perfil epidemiológico da hanseníase no período de 2018 a 2022, no Estado do Rio Grande do Norte, apresentou predomínio pelo sexo feminino, acometendo adultos entre 50 a 69 anos de idade, com registro ignorado ou em branco no grau de escolaridade, predominando a forma multibacilar do tipo Virchowiana, causando grau 0 de incapacidade e a grande parte dos casos evoluindo para a cura. Portanto, faz-se relevante conhecer melhor o perfil epidemiológico e a realidade de cada município, visto que é uma patologia fortemente relacionada a condições econômicas, sociais e ambientais desfavoráveis passíveis de intervenção.

Leprosy is a chronic, contagious infectious disease, compulsorily notifiable and compulsory research in Brazil, of slow evolution, caused by the bacillus Mycobacterium leprae that predominantly affects skin and peripheral nerves causing permanent functional impairments of various degrees, besides being a pathology still surrounded by stigma and social discrimination. The objective of the article is to evaluate the epidemiological profile of leprosy in the State of Rio Grande do Norte between 2018 and 2022, which currently occupies the 3rd place at the national level of general registration of cases of leprosy. This is an epidemiological, descriptive, retrospective and quantitative approach study, based on data obtained in the Department of Information Technology of the Unified Health System (DATASUS) and analyzed with the help of free software Jamovi 2.3.21 solid. The epidemiological profile of leprosy in the period from 2018 to 2022, in the state of Rio Grande do Norte, showed predominance by the female sex, affecting adults between 50 and 69 years of age, with ignored or blank registration in the level of schooling, predominating the multibacillary form of the Virchowiana type, causing grade 0 disability and the great majority of cases evolving to cure. Therefore, it is important to know better the epidemiological profile and reality of each municipality, since it is a pathology strongly related to unfavorable economic, social and environmental conditions that can intervene.

La Hanseniasis es una enfermedad crónica, una enfermedad contagiosa, una notificación obligatoria e investigación obligatoria en Brasil, de evolución lenta, causada por el bacilo Mycobacterium leprae, que ataca predominantemente la piel y los nervios periféricos, causando discapacidades funcionales permanentes de diversos grados, además de ser una enfermedad aún rodeada de estigma y discriminación social. El propósito del artículo es evaluar el perfil epidemiológico de la lepra en el estado de Río Grande del Norte entre 2018 y 2022, un estado que actualmente ocupa el tercer lugar en el registro general nacional de casos de lepra. Se trata de un estudio de enfoque epidemiológico, descriptivo, retrospectivo y cuantitativo basado en datos obtenidos del Departamento de Datos del Sistema de Salud Pública (DATASUS) y analizado con la ayuda del software sólido Jamovi 2.3.21 libre. El perfil epidemiológico de la lepra en el período 2018-2022, en el estado de Rio Grande do Norte, mostró predominio del sexo femenino, atacando a adultos de 50 a 69 años, con un registro desconocido o en blanco en el grado de escolaridad, predominando en la forma multibacible del tipo Virchowiana, causando un grado de discapacidad y el gran número de casos. evolucionando hacia la curación. Por lo tanto, es importante conocer mejor el perfil epidemiológico y la realidad de cada municipio, ya que se trata de una enfermedad que está fuertemente relacionada con condiciones económicas, sociales y ambientales desfavorables que pueden ser intervenidas.

The controversial Third Reich history of Hans Creutzfeldt: was he a supporter or just another adept of the "hand washing policy"?

Creutzfeldt-Jakob disease (CJD) is a transmissible spongiform encephalopathy whose initial description is associated with two German authors, Alfons Maria Jakob and Hans Gerhard Creutzfeldt. As polemic as the issue about the Creutzfeldt's merit in the first description of the disease, is his history during the Third Reich. Some evidence pointed to the idea that he was essentially against the Nazi ideology, though some did not. He was an official member of the SS, but his own wife was convicted by a Nazi court. Some authors have argued that Creutzfeldt helped save many patients during Aktion T4 operation, but, in fact, he could have done more. Even during the post-war period, he sent a letter to authorities reporting the name of a Nazi physician who worked as a medical reviewer at the euthanasia court, but he did not proceed any further when his letter initially failed to start an investigation.

The controversial Third Reich history of Hans Creutzfeldt: was he a supporter or just another adept of the "hand washing policy"? / A controversa história do terceiro reich de Hans Creutzfeldt: seria ele um apoiador ou apenas outro adepto da "política de lavagem das mãos"?

ABSTRACT Creutzfeldt-Jakob disease (CJD) is a transmissible spongiform encephalopathy whose initial description is associated with two German authors, Alfons Maria Jakob and Hans Gerhard Creutzfeldt. As polemic as the issue about the Creutzfeldt's merit in the first description of the disease, is his history during the Third Reich. Some evidence pointed to the idea that he was essentially against the Nazi ideology, though some did not. He was an official member of the SS, but his own wife was convicted by a Nazi court. Some authors have argued that Creutzfeldt helped save many patients during Aktion T4 operation, but, in fact, he could have done more. Even during the post-war period, he sent a letter to authorities reporting the name of a Nazi physician who worked as a medical reviewer at the euthanasia court, but he did not proceed any further when his letter initially failed to start an investigation.

RESUMO A doença de Creutzfeldt-Jakob (DCJ) é uma encefalopatia espongiforme transmissível, cuja descrição original está associada ao nome de dois autores alemães, Alfons Maria Jakob e Hans Gerhard Creuztfeldt. Tão polêmica quanto a questão sobre o real mérito de Creutzfeldt na primeira descrição da doença, é sua história de vida durante o Terceiro Reich. Algumas evidências apontavam para a ideia de que ele era essencialmente contra a ideologia nazista, mas outras não. Ele foi um membro oficial da SS, mas sua própria esposa foi condenada por um tribunal nazista. Alguns autores têm argumentado que Creutzfeldt ajudou a salvar muitos pacientes durante a operação Aktion T4, mas, na verdade, ele poderia ter feito muito mais. Mesmo durante o período pós-guerra, ele enviou uma carta às autoridades revelando o nome de um médico nazista que havia se empregado como revisor médico na corte sobre eutanásia, mas ele não insistiu mais quando sua carta inicialmente não desencadeou uma investigação.

Profile of caregivers of Parkinson's disease patients and burden measured by Zarit Scale Analysis of potential burden-generating factors and their correlation with disease severity.

Parkinson's disease (PD) promotes burden among patients and caregivers. OBJECTIVE: To analyze whether disease severity (UPDRS and Karnofsky index), total disease duration, patient cognitive status (MMSE), presence of other diseases, patient age, socioeconomic conditions (ABEP2015), living together with patient, total time caregiving, weekly hours of care and presence of assistance from other caregivers are correlated with, and influence statistically, the degree of caregiver burden measured by the Zarit Burden Interview (ZBI). METHODS: After ethics Committee approval, patients and respective caregivers were recruited. Following evaluation with the proper scales, all data were submitted to Pearson's correlation method and multivariate linear regression analysis (ANOVA). RESULTS: A total of 21 patients and respective caregivers were evaluated. 72% (N=15) of caregivers reported burden. One third of caregivers reported a moderate or severe level of burden. A cause-effect relationship could not be established by the statistical method adopted, but disease severity measured by the UPDRS was the sole variable showing statistically significant moderate positive Pearson's correlation with ZBI (r=0.48, for p<0.05). On ANOVA, however, no independent variable had a statistically significant impact on ZBI scores. CONCLUSION: Despite our conflicting results, optimization of the available treatment, with better control of PD severity, can be considered an important element to effectively achieve the goal of reducing burden among caregivers.

A doença de Parkinson (PD) promove sobrecarga entre pacientes e cuidadores. OBJETIVO: Analisar se a severidade da doença (UPDRS e Karnofsky index), o tempo total de doença, as condições cognitivas do paciente (MEEM), a presença de outras doenças, a idade do paciente, as condições socioeconómicas (ABEP2015), o fato de se ambos vivem juntos, o tempo total que o cuidador exerce esta função, as horas semanais de cuidados e a presença de assistência de outros cuidadores poderiam ser correlacionados e influenciar estatisticamente o grau de sobrecarga dos cuidadores, medido pela entrevista de Zarit (ZBI). MÉTODOS: Após a aprovação do comitê de ética, o recrutamento de pacientes e respectivos cuidadores foram feitos e após a avaliação com as escalas adequadas, todos os dados foram submetidos ao método de correlação de Pearson e análise de regressão linear multivariada (ANOVA). RESULTADOS: Avaliou-se um total de 21 pacientes e respectivos cuidadores. 72% (N=15) de cuidadores reportaram ter sobrecarga. Um terço deles relatou nível moderado ou severo de sobrecarga. Não foi possível estabelecer relação causa-efeito pelo método estatístico adotado, porém a severidade da doença, medida pelo UPDRS, foi a única variável que alcançou correlação de Pearson positiva moderada com significância estatística com a ZBI (r=0,48, com p<0,05), mas na ANOVA, nenhuma variável independente teve um impacto estatisticamente significante nas pontuações de ZBI. CONCLUSÃO: Apesar dos nossos resultados conflitantes, a otimização do tratamento disponível, com melhor controle da severidade PD, poderia ser considerada como um elemento importante e que poderia efetivamente atingir o objetivo de reduzir a carga entre os cuidadores.

Profile of caregivers of Parkinson's disease patients and burden measured by Zarit Scale Analysis of potential burden-generating factors and their correlation with disease severity / Perfil de cuidadores de pacientes com doença de Parkinson e sobrecarga medida pela Escala Zarit - análise de potenciais fatores geradores de sobrecarga e sua correlação com a gravidade da doença

ABSTRACT: Parkinson's disease (PD) promotes burden among patients and caregivers. Objective: To analyze whether disease severity (UPDRS and Karnofsky index), total disease duration, patient cognitive status (MMSE), presence of other diseases, patient age, socioeconomic conditions (ABEP2015), living together with patient, total time caregiving, weekly hours of care and presence of assistance from other caregivers are correlated with, and influence statistically, the degree of caregiver burden measured by the Zarit Burden Interview (ZBI). Methods: After ethics Committee approval, patients and respective caregivers were recruited. Following evaluation with the proper scales, all data were submitted to Pearson's correlation method and multivariate linear regression analysis (ANOVA). Results: A total of 21 patients and respective caregivers were evaluated. 72% (N=15) of caregivers reported burden. One third of caregivers reported a moderate or severe level of burden. A cause-effect relationship could not be established by the statistical method adopted, but disease severity measured by the UPDRS was the sole variable showing statistically significant moderate positive Pearson's correlation with ZBI (r=0.48, for p<0.05). On ANOVA, however, no independent variable had a statistically significant impact on ZBI scores. Conclusion: Despite our conflicting results, optimization of the available treatment, with better control of PD severity, can be considered an important element to effectively achieve the goal of reducing burden among caregivers.

RESUMO: A doença de Parkinson (PD) promove sobrecarga entre pacientes e cuidadores. Objetivo: Analisar se a severidade da doença (UPDRS e Karnofsky index), o tempo total de doença, as condições cognitivas do paciente (MEEM), a presença de outras doenças, a idade do paciente, as condições socioeconómicas (ABEP2015), o fato de se ambos vivem juntos, o tempo total que o cuidador exerce esta função, as horas semanais de cuidados e a presença de assistência de outros cuidadores poderiam ser correlacionados e influenciar estatisticamente o grau de sobrecarga dos cuidadores, medido pela entrevista de Zarit (ZBI). Métodos: Após a aprovação do comitê de ética, o recrutamento de pacientes e respectivos cuidadores foram feitos e após a avaliação com as escalas adequadas, todos os dados foram submetidos ao método de correlação de Pearson e análise de regressão linear multivariada (ANOVA). Resultados: Avaliou-se um total de 21 pacientes e respectivos cuidadores. 72% (N=15) de cuidadores reportaram ter sobrecarga. Um terço deles relatou nível moderado ou severo de sobrecarga. Não foi possível estabelecer relação causa-efeito pelo método estatístico adotado, porém a severidade da doença, medida pelo UPDRS, foi a única variável que alcançou correlação de Pearson positiva moderada com significância estatística com a ZBI (r=0,48, com p<0,05), mas na ANOVA, nenhuma variável independente teve um impacto estatisticamente significante nas pontuações de ZBI. Conclusão: Apesar dos nossos resultados conflitantes, a otimização do tratamento disponível, com melhor controle da severidade PD, poderia ser considerada como um elemento importante e que poderia efetivamente atingir o objetivo de reduzir a carga entre os cuidadores.

Sexual dysfunction in obese women is more affected by psychological domains than that of non-obese.

PURPOSE: To compare differences in the occurrence and changed domains of sexual dysfunction in obese and non-obese Brazilian women. METHODS: Female Sexual Function Index, based on six domains, to investigate 31 sexual dysfunction incidence for obese compared to 32 non-obese women, was used. Statistical analysis using ANOVA and MANOVA were performed to compare total scores of Female Sexual Function Index among groups and to identify the differences among domains, Student t -test was used. Statistical significant level was established for all tests for p<0.05. RESULTS: No difference in female sexual dysfunction frequency between obese (25.8%) and non-obese women (22.5%) was found. However, an important distinction in which aspects of sexual life were affected was found. While the obese group was impaired in three domains of sexual life (desire, orgasm, and arousal), in the control group five aspects were dysfunctional (desire, orgasm, arousal, pain and lubrication). Future research exploring psychological outcomes in obese females, such as body image and measures of positive and negative effect, might better characterize the female sexual dysfunction in this group. CONCLUSIONS: Obesity does not appear to be an independent factor for allow quality of female sexual life. However, disturbance associated to obesity indicates a low frequency of disorder in physical domains, suggesting that psychological factors seem to be mainly involved in the sexual dysfunction in obese women.

Sexual dysfunction in obese women is more affected by psychological domains than that of non-obese / Disfunção sexual em mulheres obesas é mais afetada por domínios psicológicos do que nas não obesas

Abstract PURPOSE: To compare differences in the occurrence and changed domains of sexual dysfunction in obese and non-obese Brazilian women. METHODS: Female Sexual Function Index, based on six domains, to investigate 31 sexual dysfunction incidence for obese compared to 32 non-obese women, was used. Statistical analysis using ANOVA and MANOVA were performed to compare total scores of Female Sexual Function Index among groups and to identify the differences among domains, Student t -test was used. Statistical significant level was established for all tests for p<0.05. RESULTS: No difference in female sexual dysfunction frequency between obese (25.8%) and non-obese women (22.5%) was found. However, an important distinction in which aspects of sexual life were affected was found. While the obese group was impaired in three domains of sexual life (desire, orgasm, and arousal), in the control group five aspects were dysfunctional (desire, orgasm, arousal, pain and lubrication). Future research exploring psychological outcomes in obese females, such as body image and measures of positive and negative effect, might better characterize the female sexual dysfunction in this group. CONCLUSIONS: Obesity does not appear to be an independent factor for allow quality of female sexual life. However, disturbance associated to obesity indicates a low frequency of disorder in physical domains, suggesting that psychological factors seem to be mainly involved in the sexual dysfunction in obese women.

Resumo OBJETIVO: Comparar as diferenças na incidência de disfunção sexual nos seis diferentes domínios de mulheres brasileiras obesas e não obesas. MÉTODOS: Foi usado o Female Sexual Function Index , que discrimina seis domínios de disfunção, para investigar a incidência de disfunção sexual em 31 mulheres obesas e 32 mulheres não obesas. Foi realizada análise estatística utilizando ANOVA e MANOVA para comparar os escores totais doFemale Sexual Function Index entre os grupos, bem como empregado o teste t para identificar as diferenças relacionadas aos domínios. O nível de significância estatística estabelecido para todos os testes foi de p<0,05. RESULTADOS: Não foi encontrada diferença significante nas diferentes incidências de disfunção sexual feminina entre o grupo de pacientes obesas (25,8%) e o grupo de não obesas (22,5%). Contudo, foi evidenciada uma importante distinção em quais aspectos da vida sexual foram afetados nos dois grupos. Enquanto as mulheres obesas foram impactadas em apenas três domínios subjetivos do Female Sexual Function Index (desejo, orgasmo e excitação), o grupo controle apresentou disfunção em cinco aspectos (desejo, orgasmo, excitação, dor e lubrificação). Pesquisas futuras explorando aspectos psicológicos em mulheres obesas, como a avaliação da autoimagem corporal e seus aspectos negativos ou positivos sobre as pacientes, deverão auxiliar na melhor caracterização da disfunção sexual feminina neste grupo. CONCLUSÕES: A obesidade não parece constituir um fator de risco independente para uma baixa qualidade de vida sexual feminina. Contudo, as disfunções associadas à obesidade foram menos evidenciadas em domínios fisiológicos, sugerindo que aspectos psicológicos parecem estar primariamente envolvidos na etiologia da disfunção sexual de mulheres obesas.

Marchiafava-Bignami disease: a rare entity with a poor outcome.

Marchiafava-Bignami disease is a rare affliction characterized by primary degeneration of the corpus callosum associated with chronic consumption of ethanol. The disease may occasionally occur in patients who are not alcoholics but are chronically malnourished. A complex deficiency of group B vitamins is the main etiopathogenic hypothesis, and many patients improve after the administration of these compounds. However, a good response is not always observed. The definitive diagnosis of Marchiafava-Bignami disease can be problematic and is based on features of neuroimaging studies, especially magnetic resonance imaging. Its treatment is still controversial and shows variable results. Because nutritional factors are implicated, as in Wernicke's encephalopathy, some authors claim that replacement of B vitamins is beneficial. The present article is a case report of a severe acute form of Marchiafava-Bignami disease in an alcohol-dependent male patient who improved after the administration of parenteral B vitamins. As a consequence of his neurological and immunologic conditions, he developed multiple pulmonary infections and had a protracted course in the intensive care unit. He eventually died of sepsis associated with an uncommon fungus, Rhodotorula mucilaginosa. The present article reports the clinical and neuroimaging data from this patient and contains a review of Marchiafava-Bignami disease and Rhodotorula infections in the intensive care unit.

Doença de Marchiafava-Bignami: uma rara entidade com prognóstico sombrio / Marchiafava-Bignami disease: a rare entity with a poor outcome

A doença de Marchiafava-Bignami é uma entidade rara, caracterizada por uma degeneração primária do corpo caloso, associada com o consumo crônico do etanol. A doença pode, ocasionalmente, ocorrer em pacientes não etilistas cronicamente desnutridos. Uma deficiência de vitaminas do complexo B é considerada como a hipótese etiopatogênica principal, uma vez que muitos pacientes obtiveram uma melhora após a administração desses compostos. Algumas vezes, entretanto, tal resposta terapêutica não foi observada. O diagnóstico definitivo da doença de Marchiafava-Bignami pode ser problemático e depende das características de estudos de neuroimagem, especialmente a ressonância magnética. Seu tratamento, dessa forma, é ainda controverso, com resultados variáveis. Como estão implicados fatores nutricionais, analogamente à encefalopatia de Wernicke, alguns autores recomendam a reposição de vitaminas do complexo B, particularmente da B1. O presente artigo relata a forma aguda da doença de Marchiafava-Bignami em um paciente masculino dependente do álcool, que apresentou discreta melhora após a administração parenteral das vitaminas do complexo B. Como consequência de suas más condições neurológicas e imunológicas, ele desenvolveu infecções pulmonares múltiplas e permaneceu, por longo tempo, na unidade de terapia intensiva. Seu óbito ocorreu por sepse causada por um fungo raro, o Rhodotorula mucilaginosa. O artigo é um relato clínico da evolução desse paciente, com a apresentação de seus dados de neuroimagem, acompanhada por uma revisão sobre doença de Marchiafava-Bignami e sobre as infecções por Rhodotorula dentro da perspectiva da unidade de cuidado intensivo.

Marchiafava-Bignami disease is a rare affliction characterized by primary degeneration of the corpus callosum associated with chronic consumption of ethanol. The disease may occasionally occur in patients who are not alcoholics but are chronically malnourished. A complex deficiency of group B vitamins is the main etiopathogenic hypothesis, and many patients improve after the administration of these compounds. However, a good response is not always observed. The definitive diagnosis of Marchiafava-Bignami disease can be problematic and is based on features of neuroimaging studies, especially magnetic resonance imaging. Its treatment is still controversial and shows variable results. Because nutritional factors are implicated, as in Wernicke's encephalopathy, some authors claim that replacement of B vitamins is beneficial. The present article is a case report of a severe acute form of Marchiafava-Bignami disease in an alcohol-dependent male patient who improved after the administration of parenteral B vitamins. As a consequence of his neurological and immunologic conditions, he developed multiple pulmonary infections and had a protracted course in the intensive care unit. He eventually died of sepsis associated with an uncommon fungus, Rhodotorula mucilaginosa. The present article reports the clinical and neuroimaging data from this patient and contains a review of Marchiafava-Bignami disease and Rhodotorula infections in the intensive care unit.

Neurological disorders associated with glutamic acid decarboxylase antibodies: a Brazilian series.

Neurological disorders associated with glutamic acid decarboxylase (GAD) antibodies are rare pleomorphic diseases of uncertain cause, of which stiff-person syndrome (SPS) is the best-known. Here, we described nine consecutive cases of neurological disorders associated with anti-GAD, including nine patients with SPS and three cases with cerebellar ataxia. Additionally, four had hypothyroidism, three epilepsy, two diabetes mellitus and two axial myoclonus.

Neurological disorders associated with glutamic acid decarboxylase antibodies: a Brazilian series / Distúrbios neurológicos associadas a anticorpos anti-GAD (descarboxilase do ácido glutâmico): uma série de casos brasileira

Neurological disorders associated with glutamic acid decarboxylase (GAD) antibodies are rare pleomorphic diseases of uncertain cause, of which stiff-person syndrome (SPS) is the best-known. Here, we described nine consecutive cases of neurological disorders associated with anti-GAD, including nine patients with SPS and three cases with cerebellar ataxia. Additionally, four had hypothyroidism, three epilepsy, two diabetes mellitus and two axial myoclonus.

Distúrbios neurológicos associados com anticorpos anti-GAD são doenças pleomórficas, raras, de causa incerta, das quais a rigidez muscular espasmódica (SPR) é a mais conhecida. Neste estudo, descrevemos nove casos consecutivos de distúrbios neurológicos associados com a presença de anticorpos anti-GAD, incluindo nove pacientes com SPR e três casos com ataxia cerebelar. Adicionalmente, foram encontrados quatro casos com hipotireoidismo, três com epilepsia, dois com diabetes mellitus e dois casos com mioclonia axial.

Evolução incomum de provável histoplasmose de sistema nervoso central: de leptomeningite para granuloma da fossa posterior: Relato de caso com imagens por ressonância magnética / Uncommon evolution of probable central nervous system histoplasmosis: from leptomeningitis to posterior fossa granuloma: A case report with magnetic resonance images

Um caso de jovem paciente imunocompetente com provável histoplasmose do sistema nervoso central é aqui reportado, com ênfase nos aspectos evoluktivos muito peculiares de neuroimagem por ressonncia magnética que foram encontrados. Inicialmente foi observado espessamento leptomeníngeo, que, posteriormente, acabou organizando-se em granuloma de fossa posterior. O diagnóstico da infecção fúngica só foi obtido através do estudo histopatológico e o tratamento empregado baseou-se no uso do fluconazol em terapia de longo prazo, com boa resposta inicial.

We report a case of a young immunocompetent patient with probable central nervous system histoplasmosis with evolutive peculiar findings seen on magnetic resonance imaging. Leptomeningeal thickening was initially observed which subsequently became a posterior fossa granuloma. The diagnosis of fungal infection was only reached by histopathological study and the treatment was based on long term therapy with fluconazole with good initial response.

Progressive supranuclear palsy in a sample of brazilian population: clinical features of 16 patients

Progressive supranuclear palsy (PSP) is an uncommon disorder characterized by marked postural instability, vertical gaze abnormalities and axial rigidity. The purpose of this study is to report the clinical features of 16 consecutive subjects seen over a 10-year period at a Movement Disorders Clinic. These subjects fulfilled criteria for probable PSP namely those of the National Institute of Neurologic Disorders and Stroke (NINDS) and the Society for PSP (SPSP). This patient-group represented 2.1 percent of all degenerative parkinsonians observed and the mean age of onset of the disease was 64.7 years (sd = ± 7.2). Postural instability with falls was the most frequent initial feature presented in PSP patients (62.5 percent). The hallmark of the disease, the supranuclear vertical gaze palsy, appeared after 2.3 years of disease onset, and only 12.5 percent had such manifestation at the first evaluation. Transient tremor was observed with a relatively high frequency in this group (44 percent), but only 19 percent had rest tremor. Chronic dacryocystitis, probably related to a paucity of blinking, was observed in two patients as an inaugural manifestation. In the first evaluation, only 19 percent of the 16 patients were diagnosed as probable PSP. The mean interval prior to the final diagnosis was 2.4 years

Progressive supranuclear palsy in a sample of Brazilian population: clinical features of 16 patients.

Progressive supranuclear palsy (PSP) is an uncommon disorder characterized by marked postural instability, vertical gaze abnormalities and axial rigidity. The purpose of this study is to report the clinical features of 16 consecutive subjects seen over a 10-year period at a Movement Disorders Clinic. These subjects fulfilled criteria for probable PSP namely those of the National Institute of Neurologic Disorders and Stroke (NINDS) and the Society for PSP (SPSP). This patient-group represented 2.1% of all degenerative parkinsonians observed and the mean age of onset of the disease was 64.7 years (sd = +/- 7.2). Postural instability with falls was the most frequent initial feature presented in PSP patients (62.5%). The hallmark of the disease, the supranuclear vertical gaze palsy, appeared after 2.3 years of disease onset, and only 12.5% had such manifestation at the first evaluation. Transient tremor was observed with a relatively high frequency in this group (44%), but only 19% had rest tremor. Chronic dacryocystitis, probably related to a paucity of blinking, was observed in two patients as an inaugural manifestation. In the first evaluation, only 19% of the 16 patients were diagnosed as probable PSP. The mean interval prior to the final diagnosis was 2.4 years.

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

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