Assistência de enfermagem em pacientes com Demência do Corpo de Lewy / Nursing assistance in patients with Lewy's Body Dementia

Objetivo: Investigar sobre a assistência de Enfermagem a pacientes com Demência do Corpo de Lewy. Método: Revisão integrativa da literatura, pela busca nas bases de dados, entre os anos de 2009 a 2021, utilizando os descritores: Doença por corpos de Lewy, Doença de Alzheimer, Doença de Parkinson, Assistência de Enfermagem. Resultado: A Demência do Corpo de Lewy é uma doença de difícil diagnóstico, por causa das semelhanças com as Doenças de Alzheimer e Parkinson, seu tratamento é baseado nessas patologias, não seguindo protocolos específicos da doença. A enfermagem tem por função principalmente orientar a família e oferecer uma assistência integral tanto para o paciente, quanto para o cuidador. Conclusão: É necessária, a realização de mais estudos, para entender como assistir um paciente diagnosticado com esta patologia adequadamente, dando suporte para um cuidado de enfermagem mais científico e integral, estabelecendo rotinas, promoveno assim qualidade de vida ao paciente e sua família.(AU)

Objective: To investigate Nursing care for patients with Lewy Body Dementia. Method: Integrative literature review, using the Scielo database, between 2009 and 2021, using the descriptors: Lewy body disease, Alzheimer's disease, Parkinson's disease, Nursing care. Result: Lewy Body Dementia is a disease that is difficult to diagnose, because of the similarities with Alzheimer's and Parkinson's Diseases, its treatment is based on these pathologies, not following disease-specific protocols. Nursing's main function is to guide the family and offer comprehensive care for both the patient and the caregiver. Conclusion: Further studies are needed to understand how to properly care for a patient diagnosed with this pathology, supporting a more scientific and comprehensive nursing care, establishing routines, thus promoting quality of life for patients and their families.(AU)

Objetivo: Investigar el cuidado de Enfermería a pacientes con Demencia con Cuerpos de Lewy. Método: Revisión integrativa de la literatura, utilizando la base de datos Scielo, entre 2009 y 2021, utilizando los descriptores: Enfermedad de cuerpos de Lewy, Enfermedad de Alzheimer, Enfermedad de Parkinson, Cuidados de enfermería. Resultado: La Demencia con Cuerpos de Lewy es una enfermedad de difícil diagnóstico, debido a las similitudes con el Alzheimer y el Parkinson, su tratamiento se basa en estas patologías, no siguiendo protocolos específicos de la enfermedad. La función principal de enfermería es orientar a la familia y ofrecer una atención integral tanto al paciente como al cuidador. Conclusión: Se necesitan más estudios para comprender cómo cuidar adecuadamente a un paciente diagnosticado con esta patología, apoyando un cuidado de enfermería más científico e integral, estableciendo rutinas, promoviendo así la calidad de vida de los pacientes y sus familias.(AU)

The clinical value of 123I-metaiodobenzylguanidine myocardial imaging in the diagnosis of dementia with Lewy bodies / 生物医学工程学杂志

Metaiodobenzylguanidine (MIBG) is an analog of norepinephrine that accumulates in sympathetic nerve endings soon after intravenous administration. The degree of accumulation reflects the uptake, storage and release of transmitters by noradrenergic neurons. Myocardial imaging with 123I labeled MIBG ( 123I-MIBG) can be used to estimate the extent of local myocardial sympathetic nerve damage, which has been widely used in the diagnosis and treatment of various heart diseases. In recent years, numerous studies have been carried out on the application of 123I-MIBG in the diagnosis of degenerative diseases of the nervous system (such as Parkinson's disease and dementia of Lewy body), and have made some achievements. The purpose of this review is to summarize the current clinical application of 123I-MIBG myocardial imaging in the diagnosis of dementia with Lewy bodies, the problems in imaging technology and the possible research directions in the future, so as to provide valuable reference information for clinicians to reasonably and accurately apply this technology in the early diagnosis and discrimination of dementia.

Cambios en la marcha asociados con demencia por cuerpos de Lewy: una revisión narrativa de la literatura / Gait changes associated with Lewy body dementia: a narrative review of the literature

RESUMEN INTRODUCCIÓN: La demencia por cuerpos de Lewy (DCL) es una enfermedad neurodegenerativa con alta prevalencia y a menudo subdiagnosticada. En las demencias pueden presentarse alteraciones en la marcha que potencialmente permitan identificar su subtipo y dar una orientación clínica, diagnóstica y terapéutica temprana. Esta revisión narrativa de la literatura busca revisar los cambios de la marcha que se han descrito asociados con DCL. MATERIALES Y MÉTODOS: Se realizó una revisión de la literatura sobre la relación de las alteraciones de la marcha con la DCL. Se seleccionaron los siguientes parámetros de búsqueda mediante el buscador Scopus: ((falls and dementia and gait and (evaluation or analysis))). Los datos se ordenaron según relevancia y se obtuvieron 267 resultados. Igualmente, se hizo una búsqueda en PubMed, para a la que se introdujeron los términos (gait and lewy-body-disease), y no se utilizaron otros filtros; se obtuvieron 139 resultados. Se hizo una selección no sistemática de los artículos para llevar a cabo una revisión narrativa acerca de los cambios en la marcha asociados con DCL. RESULTADOS: Las alteraciones en la marcha pueden tener un valor predictor importante en la DCL. Los pacientes con demencias no debido a EA o causas vasculares muestran un deterioro de la funcionalidad física más rápido comparado con pacientes con EA y sin problemas cognitivos. La priorización incorrecta de las tareas, evidenciada en la EP, también es observable en los pacientes con DCL, y se asocia con el paradigma de doble tarea en el paciente con trastorno neurocognitivo mayor. El congelamiento de la marcha, también conocido como bloqueo de la marcha, se ha asociado con mayor progresión de la alteración cognitiva. Los pacientes con DCL también presentan un mayor compromiso en el tiempo de balanceo y la variabilidad de duración de la zancada, como también peor desempeño en ritmo y variabilidad de la marcha, e inestabilidad de la marcha, con posturas inadecuadas. CONCLUSIONES: Existe una relación entre la DCL y las caídas en el adulto mayor. En este grupo de edad, los cambios en la marcha y en las pruebas de desempeño podrían tener una utilidad clínica como factores asociados a con DCL, así como con las caídas. Al parecer, existe una variación característica entre los parámetros de la marcha y los subtipos de demencias que puede tener un valor como marcador diagnóstico. Se requieren más estudios con respecto a este tema puesto que hay escasa evidencia disponible hasta el momento, lo cual impide definir con mayor precisión las alteraciones más sensibles de cada dominio de la marcha que permitan diferenciar el envejecimiento normal del patológico.

ABSTRACT INTRODUCTION: Lewy body dementia is a highly prevalent neurodegenerative disease and often goes unnoticed due to little knowledge about it. In dementias there may be gait alterations that potentially allow the identification of its subtype and provide early clinical, diagnostic, and therapeutic guidance. This narrative review of the literature aims to review gait changes that have been described as associated with Lewy body dementia. MATERIALS AND METHODS: A literature review was carried out on the relationship of gait disturbances and LBD. The following search parameters were selected using the Scopus search engine: ((falls and dementia and gait and (evaluation or analysis))). The data were ordered according to relevance, obtaining 267 results. Likewise, a search was made in PubMed, using the terms (gait and lewy-body-disease), and no other filters were used, obtaining 139 results. A non-systematic selection of literature was made to carry out a narrative review about the changes in gait associated with LBD. RESULTS: We found that gait disturbances may have an important predictive value in LBD. Patients with dementias not due to AD or vascular causes have a faster deterioration of physical function compared to patients with AD and without cognitive problems. The incorrect prioritization of tasks evidenced in PD is also observable in patients with LBD and is associated with the "dual-task" paradigm in patients with major neurocognitive disorder. Freezing of gait, also known as motor block or "freezing of gait" has been associated with a greater progression of cognitive impairment. Patients with LBD also show greater compromise in swing time, stride duration variability, poorer performance in gait pace and variability, and gait instability with inappropriate postures. CONCLUSIONS: We observe that there is a relationship between LBD and falls in the elderly. Changes in gait and performance tests could have clinical utility as factors associated with LBD as well as falls in the elderly. There appears to be a characteristic variation between gait parameters and dementia subtypes that may have value as a diagnostic marker. More studies are required on this subject since there is little evidence available to date, which makes it impossible to define with greater precision the most sensitive alterations in each domain of gait that make it possible to differentiate normal from pathological aging.

Anosognosia in dementia with Lewy bodies: a systematic review / Anosognosia na demência com corpos de Lewy: uma revisão sistemática

ABSTRACT Background: Anosognosia, i.e. lack of awareness of one's own symptoms, is a very common finding in patients with dementia and is related to neuropsychiatric symptoms and worse prognosis. Although dementia with Lewy bodies (DLB) is the second most common form of degenerative dementia, literature on anosognosia in this disease is scarce. Objectives: This paper aimed to review the current evidence on anosognosia in patients with DLB, including its prevalence in comparison with other neurological conditions, its severity and anatomical correlations. Methods: Database searches were performed in PubMed, Web of Knowledge and PsycINFO for articles assessing anosognosia in DLB. A total of 243 studies were retrieved, but only six were included in the review. Results: Potential risk of selection, comparison or outcome biases were detected in relation to all the studies selected. Most of the studies used self-report memory questionnaires to assess cognitive complaints and compared their results to scores from informant-based instruments or to participants' cognitive performance in neuropsychological tasks. Subjects with DLB had worse awareness regarding memory than healthy older controls, but the results concerning differences in anosognosia between DLB and Alzheimer's disease (AD) patients were inconsistent across studies. Presence of AD pathology and neuroimaging biomarkers appeared to increase the prevalence of anosognosia in individuals with DLB. Conclusion: Anosognosia is a common manifestation of DLB, but it is not clear how its prevalence and severity compare with AD. Co-existence of AD pathology seems to play a role in memory deficit awareness in DLB.

RESUMO Introdução: Anosognosia, i.e. a perda da consciência dos próprios sintomas, é um achado muito comum em pacientes com demência e está relacionada a sintomas neuropsiquiátricos e a pior prognóstico. Embora a doença por Corpos de Lewy (DCL) seja a segunda demência degenerativa mais comum, há pouca evidência sobre anosognosia nessa doença. Objetivos: Este artigo teve como objetivo revisar a evidência disponível sobre anosognosia em pacientes com DCL, incluindo sua prevalência em comparação a outras condições neurológicas, gravidade e correlações anatômicas. Métodos: Foram feitas buscas nos bancos de dados PubMed, Web of Knowledge e PsycINFO por artigos que avaliassem anosognosia na DCL. Um total de 243 estudos foi encontrado, mas apenas 6 foram incluídos nesta revisão. Resultados: Potenciais riscos de viés de seleção, comparação ou resultado foram encontrados em todos os estudos selecionados. A maior parte dos estudos utilizou questionários de memória preenchidos pelo próprio paciente e os comparou a resultados de instrumentos preenchidos por informantes ou à performance cognitiva em tarefas neuropsicológicas. Indivíduos com DCL têm pior consciencia de memória do que idosos saudáveis, mas os resultados tocantes à diferença de anosognosia entre DCL e doença de Alzheimer (DA) são inconsistentes entre estudos. A presença de achados patológicos e de neuroimagem de DA parece aumentar a prevalência de anosognosia entre pacientes com DCL. Conclusão: Anosognosia é uma manifestação comum da DCL, mas não é possível afirmar como sua prevalência e gravidade se comparam à DA. A coexistência de achados patológicos de DA parece influenciar a consciência de déficits de memória na DCL.

The Role of I-131 MIBG cardiac scintigraphy in diagnosing dementia with lewy bodies : A case report

@#The objective of this case report is to highlight the role of Iodine-131 metaiodobenzylguanidine (MIBG) cardiac scintigraphy in discriminating Dementia with Lewy Bodies (DLB) from other neurodegenerative diseases such as Alzheimer’s Disease. This patient is a known case of Parkinson’s disease and has been treated as such since 2011. However, the patient also concurrently deals with visual hallucinations and because of this, the patient’s attending neurologist wanted to rule in the diagnosis of DLB rather than AD. Hence, an I-131 MIBG cardiac scan was requested in order to support the diagnosis of DLB. The use of I-131 MIBG cardiac scintigraphy as a diagnostic tool for diagnosing Lewy Body Dementia is not prevalent and to our knowledge, this was the first time in the country that this procedure was done (December 9, 2019).

Ekbom Syndrome associated with Lewy Body Dementia: A case report / Síndrome de ekbom associada à demência com corpos de lewy: um relato de caso

ABSTRACT Ekbom Syndrome, also known as parasitosis delusion or psychogenic parasitosis, is a rare condition in which patients present with a fixed belief of being infested by parasites, vermin or small insects, along with tactile hallucinations (such as pruritus or sensations of the parasites crawling over or under the skin). The syndrome may occur idiopathically or be associated with other medical conditions and drug use. This case report describes the occurrence of Ekbom syndrome in a patient diagnosed with Lewy Body Dementia (LBD), a neurodegenerative disease that commonly presents with sensory perception and thought disorders and other neuropsychiatric symptoms. Although visual hallucination is considered a core diagnostic criterion, other modalities of psychiatric symptoms can also occur posing a further challenge for correct diagnosis. Proper recognition allows early diagnosis and adequate treatment, preventing hazardous antipsychotic use in these patients.

RESUMO A síndrome de Ekbom, também conhecida como delírio parasitário ou parasitose psicogênica, é uma condição rara na qual os pacientes apresentam crença fixa de estarem infestados por parasitas, vermes ou insetos, acompanhada de alucinações táteis (como prurido ou sensação dos parasitas andando sobre ou sob a pele). A síndrome pode ocorrer de forma idiopática ou associada a outras condições médicas ou uso de drogas. Este relato de caso descreve a ocorrência da síndrome de Ekbom em um paciente diagnosticado com Demência com corpos de Lewy (DCL), uma doença degenerativa que comumente se apresenta com desordens de sensopercepção e pensamento, e outros sintomas neuropsiquiátricos. A alucinação visual é considerada um dos critérios diagnósticos nucleares, entretanto outras modalidades de sintomas psiquiátricos podem ocorrer criando desafios adicionais ao diagnóstico correto. O reconhecimento apropriado permite o diagnóstico precoce e tratamento adequado, prevenindo o uso arriscado de antipsicóticos nesses pacientes.

Atenção psicossocial em um caso de Demência potencialmente reversível / Psychosocial Care in a potentially reversible case of Dementia / Atención psicosocial en un caso de Demencia potencialmente reversible

As síndromes demenciais têm múltiplas etiologias e, entre as causas de demências potencialmente reversíveis, está a depressão. Apresenta-se aqui um estudo de caso de paciente idoso diagnosticado com Demência por Corpos de Lewy e que, após tratamento multiprofissional, teve melhora da sua condição cognitiva e emocional, fazendo-se rever o diagnóstico inicial. Considerou-se que os sintomas demenciais iniciais estavam associados aos sintomas depressivos e o cuidado baseado na atenção psicossocial foi fundamental para as mudanças observadas.

Dementia syndromes have multiple etiologies and among the causes of potentially reversible dementias is depression. We present here a case study of an elderly patient diagnosed with Dementia by Lewy Bodies and who, after multiprofessional treatment, had an improvement in his cognitive and emotional condition, and the initial diagnosis was reviewed. Initial dementia symptoms were considered to be associated with depressive symptoms and care based on psychosocial care was central to the observed changes.

Los síndromes demenciales tienen múltiples etiologías y, entre las causas de demencias potencialmente reversibles, está la depresión. Se presenta aquí un estudio de caso de paciente mayor diagnosticado con Demencia por Cuerpos de Lewy y que, después del tratamiento multiprofesional, tuvo mejoría de su condición cognitiva y emocional, haciéndose revisar el diagnóstico inicial. Se consideró que los síntomas demenciales iniciales estaban asociados a los síntomas depresivos y el cuidado basado en la atención psicosocial fue fundamental para los cambios observados.

Characteristics of facial expression recognition ability in patients with Lewy body disease

BACKGROUND@#The facial expression of medical staff has been known to greatly affect the psychological state of patients, making them feel uneasy or conversely, cheering them up. By clarifying the characteristics of facial expression recognition ability in patients with Lewy body disease, the aim of this study is to examine points to facilitate smooth communication between caregivers and patients with the disease whose cognitive function has deteriorated.@*METHODS@#During the period from March 2016 to July 2017, we examined the characteristics of recognition of the six facial expressions of "happiness," "sadness," "fear," "anger," "surprise," and "disgust" for 107 people aged 60 years or more, both outpatient and inpatient, who hospital specialists had diagnosed with Lewy body diseases of Parkinson's disease, Parkinson's disease with dementia, and dementia with Lewy bodies. Based on facial expression recognition test results, we classified them by cluster analysis and clarified features of each type.@*RESULTS@#In patients with Lewy body disease, happiness was kept unaffected by aging, age of onset, duration of the disease, cognitive function, and apathy; however, recognizing the facial expression of fear was difficult. In addition, due to aging, cognitive decline, and apathy, the facial expression recognition ability for sadness and anger decreased. In particular, cognitive decline reduced recognition of all of the facial expressions except for happiness. The test accuracy rates were classified into three types using the cluster analysis: "stable type," "mixed type," and "reduced type". In the "reduced type", the overall facial recognition ability declined except happiness, and in the mixed type, recognition ability of anger particularly declined.@*CONCLUSION@#There were several facial expressions that the Lewy body disease patients were unable to accurately identify. Caregivers are recommended to make an effort to compensate for such situations with language or body contact, etc., as a way to convey correct feeling to the patients of each type.

Actualización en enfermedad con cuerpos de Lewy / Update in Lewy body disease

La enfermedad con cuerpos de Lewy incluye 2 entidades que podrían ser consideradas variantes clínicas de una misma patología: la demencia con cuerpos de Lewy y la demencia en enfermedad de Parkinson. Con la finalidad de describir correctamente lo que sucede en la evolución de la enfermedad se divide el cuadro en etapa prodrómica y de demencia propiamente dicha. La primera está clínicamente representada por aquel período en el cual, si bien el paciente exhibe algunos signos y síntomas propios de la enfermedad, no reúne criterios de demencia. A pesar de ser difícil de definir y por carecerse todavía de contundentes datos clínicos y biomarcadores, se caracteriza principalmente por deterioro leve selectivo en función atencional ­ visuoespacial, trastorno del sueño REM y disautonomía‒. La segunda etapa está claramente caracterizada en los criterios de consenso del año 2005. Recientemente hemos publicado la validación de un instrumento llamado ALBA Screening Instrument, que permite diagnosticar con alta sensibilidad y especificidad la enfermedad aun en etapas tempranas y diferenciarla de otras patologías semejantes. La tomografía por emisión de positrones (PET) para transportador de dopamina es el procedimiento de referencia (gold standard) del diagnóstico. El tratamiento sintomático con anticolinesterásicos y neurolépticos atípicos favorece una buena evolución de la enfermedad y es fundamental tener en cuenta evitar medicamentos que pueden dañar gravemente a los pacientes como los anticolinérgicos y antipsicóticos típicos. Los avances en el diagnóstico y la difusión del impacto de esta enfermedad en la población contribuirán a generar mayores esfuerzos de investigación para hallar un tratamiento eficaz, preventivo o curativo o de ambas características. (AU)

Lewy body disease includes 2 entities that could be considered clinical variants of the same pathology: Dementia with Lewy bodies and Parkinson's disease Dementia. Two stages of the disease are described in this review, a prodromal stage and one of explicit dementia. The first one is clinically represented by that period in which, the patient exhibits some typical features of the disease, but not dementia criteria. Despite being difficult to define the prodromal stage and that strong clinical data and biomarkers are still lacking, there is evidence to characterize it mainly by mild selective impairment in attention and visuo-spatial function, REM sleep disorder and dysautonomia. The second stage is clearly characterized in the known consensus criteria of 2005. We have recently published the validation of an instrument called ALBA Screening Instrument which showed a high sensitivity and specificity for diagnosis of the disease even in the early stages. It´s useful to differentiate the disease from other similar pathologies. Positron Emission Tomography for dopamine transporter is the gold standard of diagnosis in life. Symptomatic treatment with anticholinesterases and atypical neuroleptics help patients in their evolution of the disease. Anticholinergics and typical antipsychotics are agents to avoid in the treatmen of the disease because can severely damage patients. Future advances in the diagnosis and dissemination of the knowledge of the disease will contribute to generate greater research efforts to find an effective preventive and / or curative treatment. (AU)

Improvement of Visuo-spatial Function Assessed by Raven’s Colored Progressive Matrices in Dementia with Lewy Bodies by Donepezil Treatment

OBJECTIVE: Donepezil is used to improve cognitive impairment of dementia with Lewy bodies (DLB). Visuo-spatial dysfunction is a well-known symptom of DLB. Non-verbal Raven’s Colored Progressive Matrices (RCPM) were used to assess both visual perception and reasoning ability in DLB subjects treated with donepezil. METHODS: Twenty-one DLB patients (mean age, 78.7±4.5 years) were enrolled. RCPM assessment was performed at the time of starting donepezil and within one year after starting donepezil. RESULTS: There were significant improvements of RCPM in the total scores between one year donepezil treatment (p=0.013), in both Set A score (p=0.002) and Set AB score (p=0.015), but trend in the Set B score (p=0.083). CONCLUSION: Donepezil is useful for improving visuo-spatial impairment in DLB, but not for problem-solving impairment.

¹²³I-Meta-iodobenzylguanidine Sympathetic Imaging: Standardization and Application to Neurological Diseases / 전남의대학술지

¹²³I-meta-iodobenzylguanidine (MIBG) has become widely applied in Japan since its introduction to clinical cardiology and neurology practice in the 1990s. Neurological studies found decreased cardiac uptake of ¹²³I-MIBG in Lewy-body diseases including Parkinson's disease and dementia with Lewy bodies. Thus, cardiac MIBG uptake is now considered a biomarker of Lewy body diseases. Although scintigraphic images of ¹²³I-MIBG can be visually interpreted, an average count ratio of heart-to-mediastinum (H/M) has commonly served as a semi-quantitative marker of sympathetic activity. Since H/M ratios significantly vary according to acquisition and processing conditions, quality control should be appropriate, and quantitation should be standardized. The threshold H/M ratio for differentiating Lewy-body disease is 2.0-2.1, and was based on standardized H/M ratios to comparable values of medium-energy collimators. Parkinson's disease can be separated from various types of parkinsonian syndromes using cardiac ¹²³I-MIBG, whereas activity is decreased on images of Lewy-body diseases using both ¹²³I-ioflupane for the striatum and ¹²³I-MIBG. Despite being a simple index, the H/M ratio of ¹²³I-MIBG uptake is reproducible and can serve as an effective tool to support a diagnosis of Lewy-body diseases in neurological practice.

¹²³I-Meta-iodobenzylguanidine Sympathetic Imaging: Standardization and Application to Neurological Diseases / 전남의대학술지

¹²³I-meta-iodobenzylguanidine (MIBG) has become widely applied in Japan since its introduction to clinical cardiology and neurology practice in the 1990s. Neurological studies found decreased cardiac uptake of ¹²³I-MIBG in Lewy-body diseases including Parkinson's disease and dementia with Lewy bodies. Thus, cardiac MIBG uptake is now considered a biomarker of Lewy body diseases. Although scintigraphic images of ¹²³I-MIBG can be visually interpreted, an average count ratio of heart-to-mediastinum (H/M) has commonly served as a semi-quantitative marker of sympathetic activity. Since H/M ratios significantly vary according to acquisition and processing conditions, quality control should be appropriate, and quantitation should be standardized. The threshold H/M ratio for differentiating Lewy-body disease is 2.0-2.1, and was based on standardized H/M ratios to comparable values of medium-energy collimators. Parkinson's disease can be separated from various types of parkinsonian syndromes using cardiac ¹²³I-MIBG, whereas activity is decreased on images of Lewy-body diseases using both ¹²³I-ioflupane for the striatum and ¹²³I-MIBG. Despite being a simple index, the H/M ratio of ¹²³I-MIBG uptake is reproducible and can serve as an effective tool to support a diagnosis of Lewy-body diseases in neurological practice.

Papillary Meningioma Presenting as Rapidly Progressive Dementia and Parkinsonism

There are a variety of different causes of parkinsonism including PD, secondary parkinsonism, and the parkinsonism plus syndromes. Secondary parkinsonism is caused by structural, toxic, metabolic, or infectious mechanisms. Among structural causes, intracranial neoplasms are a rare cause of secondary parkinsonism. Moreover, there are almost never case reports with intracranial space-occupying lesions resulting in parkinsonism associated with rapid cognitive impairment. Therefore, we report herein a 37-year-old woman diagnosed with papillary meningioma who presented with parkinsonism associated with rapidly progressive cognitive impairment mimicking diffuse Lewy body disease.

Características clínicas y epidemiológicas de los pacientes con enfermedad de Parkinson atípica / Clinical and epidemiological characteristics of patients with atypical Parkinson's disease

Objetivo: Describir las características clínicas y epidemiológicas de los pacientes con enfermedad de Parkinson atípico. Material y métodos: El estudio fue descriptivo y retrospectivo. Se incluyó todos los pacientes con diagnóstico de enfermedad de Parkinson atípico que recibieron atención médica en el Hospital Nacional EsSalud Edgardo Rebagliati Martins de Lima, de cuyas historias clínicas se extrajo la información. Resultados. Se incluyó a 41 pacientes con diagnóstico de Parkinson atípico de los cuales la mayor frecuencia fueron pacientes con la parálisis supranuclear progresiva (PSP) seguidos de atrofia multisistémica (AMS), degeneración ganglionar corticobasal (DGCB) y demencia por cuerpos de Lewy (DCL). Hubo predominio de sexo masculino en pacientes con PSP y de sexo femenino en pacientes con AMS. Todos los pacientes con PSP presentaron un cuadro progresivo, edad de inicio mayor o igual a 40 años y parálisis supranuclear de la mirada vertical; todos los pacientes con AMS presentaron rigidez y la mayoría inestabilidad postural y ataxia de la marcha como características más frecuentes; todos los pacientes con DGCB presentaron un curso crónico progresivo y asimetría al inicio; finalmente, todos los pacientes con DCL presentaron un deterioro cognitivo progresivo de suficiente magnitud para interferir con las funciones normales sociales u ocupacionales y fluctuaciones en la función cognitiva con variaciones pronunciadas en la atención y alerta. Conclusión: Las características clínicas de las entidades catalogadas como parkinsonismo atípico son variadas y a veces con diferencias muy sutiles en cada uno de ellos; por tanto, establecer el diagnóstico correcto a veces resulta difícil.

Purpose: Describe the clinical and epidemiological characteristics in patients with atypical parkinsonism. Methods. The present study was descriptive and retrospective. It included all patients with atypical parkinsonian syndrome: progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), multiple system atrophy (MSA) and dementia with Lewy bodies (DLB) that were attended in the Edgardo Rebagliati Martins Hospital, Lima. All data was collected from the clinical records. Results: There were 41 patients with atypical parkinsonism; the most frequent diagnosis was PSP followed by AMS, DGCB and finally DCL. Most PSP patients were males; otherwise, most AMS patients were females. All PSP patients had a progressive disease, age of onset ¡Ý 40 year-old and supranuclear palsy of vertical gaze. All AMS patients had rigidity, postural instability and ataxic gait as frequent characteristics. All DGCB patients had a chronic progressive course and asymmetry at onset. Finally patients with DCL had a progressive cognitive deterioration that interfere with normal social or occupational functions. Conclusion: The clinical characteristics of patients with an atypical parkinsonism syndrome were diverse and sometimes with few differences. So, to establish a correct diagnosis is difficult in some cases.

Aspectos sintomáticos neuropsiquiátricos asociados a la enfermedad de Parkinson con demencia / Sumptomatic and Neuropsychiatric Aspects Associated with Parkinson's Disease with Dementia

Como es sabido, la enfermedad de Parkinson suele presentar una evolución crónica, prolongada, e insidiosa. Para la cual aún no se disponen de terapéuticas efectivas que curen a dicha enfermedad, si no se posee un arsenal de fármacos (agonistas dopaminérgicos) capaces de mitigar los síntomas, mejorar y prolongar la calidad de vida y enlentecer el propio desarrollo de la enfermedad, que lleva hacia un deterioro profundo de la motricidad, funcionalidad y de las funciones neurocognitivas. También es sabida la asociación de la enfermedad de Parkinson con la demencia por la misma enfermedad. Pero a lo largo de ella, con o sin demencia, se suelen presentar en una proporción importante, una serie de síntomas asociados neuropsiquiátricos (NP), y neuroconductuales (NC) que hay que diferenciar si son producto de la propia enfermedad de Parkinson, o son desencadenados por el tratamiento. Algunos de estos síntomas asociados, los más importantes en cuanto a su frecuencia y gravedad sobre todo asociados a demencia son, depresión, excitación psicomotriz, ideación patológica, delirios, alucinaciones visuales, síndrome confusional (delirium), trastornos del sueño, ansiedad, apatía. Estos síntomas agravan la evolución de la propia enfermedad de Parkinson y la demencia asociada haciendo que su pronóstico se torne más desfavorable, y más deteriorante, afectando también a los cuidadores y alterando la calidad de vida del paciente y su entorno. En este trabajo me propongo dar una noción básica de dichos trastornos para su rápido reconocimiento y tratamiento, teniendo en cuenta la posible polifarmacia en estos pacientes, con las implicancias de las interacciones farmacológicas. La rápida resolución de estos síntomas asociados a la EP, con o sin demencia redundará en un menor deterioro funcional del paciente, mejorando su pronóstico y la calidad de vida del propio paciente y sus cuidadores.

Throughout the progression of Parkinson's disease, whether or not with dementia, a series of associated neuropsychiatric and neurobehavioral symptoms may appear, such as depression, anxiety, psychomotor agitation, delirium, visual hallucinations, confusional syndrome (delirium), sleep disorders, apathy, which aggravate the patients' prognosis and accelerate their overall deterioration and the quality of life of them and the people around them. The prompt identification and management of these associated neuropsychiatric and neurobehavioral symptoms, considered in conjunction with the possible pharmacological interactions among polymedicated patients, shall result in the patients' better quality of life and a more favorable prognosis.

As diversas faces da síndrome demencial: como diagnosticar clinicamente?: [revisão] / The several faces of dementia syndrome: how to diagnose clinically?: [review]

Objetivos: a presente revisão descreve aspectos clínicos relacionados às diferentes síndromes demenciais, objetivando tornar comum, no meio médico, o conhecimento das nuanças que permeiam o diagnóstico diferencial dessas afecções. Fonte de Dados: foi realizada uma revisão da literatura através das bases de dados Medline, Ovid e Scopus até outubro de 2009, assim como de livros-texto. Artigos com enfoque na fisiopatogenia e na patologia não foram priorizados, tendo em vista os objetivos deste estudo. Síntese dos Dados: este artigo revisa aspectos dealgumas síndromes demenciais reversíveis e irreversíveis, como Doença de Alzheimer e Demência Vascular, enfocando principalmente as características clínicas e diagnósticas que as tornam entidades distintas. Conclusões: a literatura sugere que o processo diagnóstico das síndromes demenciais assenta-se fundamentalmente na clínica, priorizando uma avaliação rigorosa do estado mental. Entretanto, a avaliação por neuroimagem e exames laboratoriais tem participação considerável em determinar a causa subjacente ao quadro demencial, revelando peculiaridades que podem nortear o diagnóstico diferencial. Diagnosticar diferentes etiologias é importante para o prognóstico e conduta terapêutica específica.

Aims: This review describes the clinical aspects related to different dementia syndromes, aiming to become common in the medical , the knowledge of the nuances that permeate the differential diagnosis of these disorders. Data Source: A review of literature published up to October 2009 was conducted on Medline, Ovid, and Scopus databases, as well as on textbooks. Articles that focused on the pathogenesis and pathology were not prioritized, given the objectives of this study. Summary of the Findings: This article reviews some aspects of reversible and irreversible dementia syndromes, such as Alzheimer?s disease and Vascular Disease, focusing on the clinic and diagnostic features that make them distinct entities. Conclusions: The literature suggests that the diagnostic process of dementia syndromes is based mainly on clinical practice, prioritizing a rigorous assessment of mental status. However, neuroimaging evaluation and laboratory tests have considerable input in determining the underlying cause of dementia, revealing peculiarities that can guide the differential diagnosis. The diagnosis of different etiologies is important to the prognosis and the specific therapeutic approach.

Demencia de los Cuerpos de Lewy: presentación de un caso / Dementia with Lewy Bodies: a case report

Se presenta un caso de dos años de evolución con síntomas principales de incontinencia urinaria, alucinaciones visuales llamativas, pérdida de la conciencia transitoria, dificultad para reconocer el lugar, rigidez muscular con inestabilidad en la marcha y fluctuaciones del rendimiento cognitivo en el transcurso del día. Se concluye que coincide con los criterios.

A case of two years of evolution that fit with the diagnoses criteria of Dementia with Lewy bodies (DLB) is presented being the main symptoms urinary incontinence, showy visual allucinations, loss of transitory consciousness, difficulty to recognize place, muscular rigidity with instability to walk and fluctuations of the mental performance as day went by.

Diagnóstico diferencial das síndromes parkinsonianas / Differential diagnosis of Parkinsonian syndromes

As síndromes parkinsoninas, em especial a Doença de Parkinson são bastante presentes na prática clínica do neurologista, geriatra e clínico geral. Suas manifestações clínicas são bastante conhecidas; entretanto uns grandes números de situações enfermidade podem cursar com quadros semelhantes e, muitas vezes de difícil diagnóstico. Messes artigo, são abordadas as principais causas de síndromes parkinsonianas, com orientações no que tange ao seu diagnóstido, aspectos clínicos e prognóstico.