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.

Analysis of visual scores of brain magnetic resonance imaging features of dementia with Lewy bodies / 中华老年医学杂志

Objective:To assess the practical value of visual scores of magnetic resonance imaging(MRI)features in the diagnosis and classification of dementia with Lewy bodies(DLB).Methods:In this study, 102 DLB patients were prospectively recruited, with 102 cognitively normal elderly people as the normal control group(NC).All included subjects underwent MRI examinations and neuropsychological assessments.Based on the clinical dementia rating(CDR)scale, DLB patients were divided into a mild(CDR=1.0), a moderate(CDR=2.0)and a severe(CDR=3.0)group.The results of MRI were scored visually and the rating scales included medial temporal lobe atrophy(MTA), global cortical atrophy-frontal subscale(GCA-F), posterior cortical atrophy(PCA), white matter lesions(the Fazekas scale), cerebral microbleeds(CMBs), and the Evans Index(EI).Statistical differences were compared between the DLB and NC groups and between DLB patients with different degrees of cognitive impairment.Results:In terms of neuropsychology, the Mini-Mental State Examination(MMSE) score of the DLB group[16.0(11.0, 21.0)]was statistically significantly lower than that of the NC group[29.0(28.0, 30.0)]( Z=-12.31, P<0.001), the Montreal Cognitive Assessment(MoCA)score of the DLB group[9.5(6.0, 15.0)]was statistically significantly lower than that of the NC group[28.0(27.0, 29.0)]( Z=-12.40, P<0.001), and the Activities of Daily Living(ADL)score of the DLB group[32.0(23.8, 40.0)]was statistically significantly higher than that of the NC group[20.0(20.0, 20.0)]( Z=-11.98, P<0.001).The scores of all MRI visual assessment scales in DLB patients were statistically significantly higher than those in the NC group( P<0.001).There were significant differences in MTA scores between DLB patients with different degrees of cognitive impairment( P0<0.001).The MTA score of the mild group[1.0(1.0, 1.0)]was statistically significantly lower than that of the moderate group[2.0(1.0, 2.0)]( P1<0.001, P2<0.001); The MTA score of the moderate group[2.0(1.0, 2.0)]was statistically significantly lower than that of the severe group[2.0(2.0, 3.0)]( P1=0.003, P2=0.010). Conclusions:This study has for the first time after comprehensively evaluated the value of various visual scores in DLB diagnosis, MTA can be used to help diagnose DLB and distinguish the severity of DLB, providing a new supplemental tool for clinical diagnosis.

Role of positron emission tomography in etiological evaluation of patients with dementia / 国际脑血管病杂志

Dementia is a syndrome mainly characterized by acquired cognitive impairment, which is mainly manifested by the decrease of cognitive functions such as understanding, orientation, and visuospatial ability. Due to different intervention methods for different types of dementia, differential diagnosis is extremely important. Positron emission tomography (PET) can reflect the changes of brain function from multiple angles through different tracers, providing imaging basis for the differential diagnosis of dementia. This article reviews the characteristics of PET in patients with different types of dementia in order to provide ideas for the differential diagnosis of patients with different types of dementia.

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.

Correlates of neuropsychiatric and motor tests with language assessment in patients with Lewy body dementia

Abstract Background Lewy body dementia (LBD) impairs performance in daily activities and affects motor, language and visuospatial tasks. Objective We aimed to correlate neuropsychiatric and motor assessments with language and visual organization tests in LBD. Methods Twenty-two patients with dementia with Lewy bodies and ten patients with Parkinson's disease dementia participated on a cross-sectional study that assessed cognition, functionality, caregiver burden, verbal fluency, the primer-level dictation section of the Boston Diagnostic Aphasia Examination (PLD-BDAE), the Hooper Visual Organization Test, the Neuropsychiatric Inventory and the Movement Disorder Society - Unified Parkinson's Disease Rating Scale. Results Language and visuospatial test results followed motor impairment and general cognitive performance. Whereas visual organization did not predict performance in the PLD-BDAE, visuospatial abilities and verbal fluency were concurrently associated, suggesting that linguistic impairment in LBD may be attributed to neuropsychological components of cognition and language. Only visual organization was associated with behaviour, suggesting that neuropsychiatric symptoms associate with differential impairment of visual organization in comparison with language in LBD. Schooling did not affect visual organization or language test performance, while the length of dementia was negatively associated with visual organization and verbal fluency. Discussion Though visual organization tests follow behaviour and motor performance in LBD, there is differential impairment regarding language skills.

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.

Investigation of the frequency and location of cerebral microbleeds in dementia with Lewy bodies versus in Alzheimer's disease / 中华老年医学杂志

Objective To investigate the frequency and location of cerebral microbleeds(CMB) in dementia with Lewy bodies(DLB)versus in Alzheimer's disease(AD).Methods This retrospective study included three groups of probable AD patients (n =156),dementia with Lewy bodies (n =67) and normal controls(n=172).Frequencies and location of CMBs in the three groups were calculated and recorded.The foci of MRI signal for CMB were confirmed by two radiologists at moments of unknowingness about diagnosis.The correlations of cerebral small vessel disease and cerebral amyloid angiopathy with the development of cognitive decline in AD were analyzed.Results The incidence rate of CMBs was higher in patients with groups of DLB(22.4 ％,15/67) and AD(19.8 ％,31/156) than in normal controls (8.2 ％,14/172) (P =0.002 and 0.002),while there was no significant difference in incidence rate of CMBs between DLB and AD groups(P＞0.05).The MRI signal intensity of CMBs was the highest in the occipital lobe of the DLB group,and was higher in the deep temporal lobe or temporal lobe in the AD group.Conclusions The frequency of CMB is higher in patients with DLB or AD than in normal controls and there is no significant difference in frequency of CMB between DLB and AD groups,which suggests that the pathophysiological mechanisms of CMB may be similar between AD and DLB.

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.

A comparative analysis of clinical manifestations of Alzheimer's disease patients and Lewy body dementia patients / 中华老年医学杂志

Objective To compare cognitive ,behavioral ,and psychological symptoms observed in Alzheimer's disease (AD ) and dementia with Lewy body (DLB ) in order to provide helpful information for differential diagnosis. Methods We selected 215 patients with AD (AD group)and 66 patients with DLB(DLB group)to retrospectively analyzed their general information ,family history , and disease history.The Mini-Mental State Examination(MMSE scale)was used to evaluate cognitive abilities and Neuropsychiatric Inventory ( NPI scale ) was used for analysis of behavioral and psychological symptoms of dementia in both groups. Results The prevalence of hypertension was higher in the AD group(52.6%,n＝113)than in the DLB group(40%,n＝24)(P＜0.05).The DLB group showed better general cognitive scores ,disorientation scores ,immediate recall scores ,and delayed recall scores than the AD group. Fluctuating cognitive deficiencies and Parkinson's symptoms were more prevalent in the DLB group than in the AD group with 2 patients (0.9%) showing fluctuating cognitive deficiencies in the AD group ,and 19(28.8%)in the DLB group(χ2＝8.580 ,P＝0.003) ;with 26 patients(12.1%)showing Parkinson's symptoms in the AD group and 25(37.9%)in the DLB group(χ2＝ 22.602 ,P＝ 0.000).As to behavioral and psychological symptoms ,the DLB group had higher scores in total NPI ,hallucinations and nighttime abnormal behaviors with 32 patients (14.9%)showing hallucinations and 64 (29.8%)showing nighttime abnormal behaviors in the AD group ;30(46.2%)showing hallucinations and 30 (46.2%)showing nighttime abnormal behaviors in the DLB group (all P ＜ 0.05 ) . Conclusions Hypertension is more prevalent in AD patients. Fluctuating cognitive deficiencies ,Parkinson's symptoms ,hallucinations ,and nighttime abnormal behaviors are prominent manifestations in DLB patients.

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.

Clinical analysis of dementia with lewy body / 中国神经精神疾病杂志

Objective To investigate the clinical features,diagnosis,treatment and prognosis of Dementia with Lewy Body (DLB) in order to improve doctors' understanding and experience.Methods Thirteen cases of clinicall diagnosed DLB were analyzed.The clinical manifestations,neurological examination,laboratory tests,treatment and prognosis were analyzed.Results All of 13 patients had fluctuating dementia.Among them,11 were diagnosed with probable DLB,and 2 were diagnosed with possible DLB.There were 8 cases (8/13) with volatility cognitive impairment,8 cases (8/13) with Parkinson's syndrome,8 casess (8/13) with visual hallucinations,13 cases (13/13) with memory loss,4 patients with limb tremor (4/13),5 cases with delirium (5/13),3 cases with increased sleep (3/ 13),1 case with silence (1/13),9 cases with anxiety and depression (9/13),one case with dizziness (1/13),6 cases with abnormal dysplasia (6/13),and one case with abnormal sensitive to diazepam (1/13).The cognitive function fluctuated,the condition deteriorated and two patients died during the follow-up from 6 months and 5 years.Conclusions Lewy body dementia is an irreversible and progressive neurodegenerative disease.Comprehensive understanding of its clinical features is helpful for the early diagnosis and treatment,which may in turn improve the prognosis.

Comparison of mental behavior and cognition between dementia with lewy body and Parkinson′s disease de-mentia / 实用医学杂志

Objective To investigate the characteristics of cognitive function and mental behavior symp-toms in patients with Parkinson′s disease dementia(PDD)and dementia with lewy body(DLB).Methods Forty-eight patients with DLB and 38 with PDD were collected in Tianjin Huanhu Hospital and Tianjin Jianhua Hospital. All patients were assessed on cognitive function and mental behavior symptoms using Montreal Cognitive Assess-ment Scale,Mini-Mental State Examination,Hamilton Depression Scale,Hamilton Anxiety Scale and The Neuro-psychiatric Inventory Questionnaire.The Unified Parkinson Disease Rating Scale-Part 3(UPDRSⅢ)and modified Hoehn Yahr staging were used to assess the severity of motor symptoms in patients. Results Scores of UPDRSⅢ,posture and gait disorder were higher in DLB group than those in PDD group but the score of tremor was lower in DLB group than that in PDD group. The proportions of non tremor onset and bilateral onset were also lower in DLB group than those in PDD group,and the differences were statistically significant(P<0.05).Cognitive func-tion impairment was more serious in DLB group than that in PDD group in all cognitive fields including execution, attention,visual space,orientation and the difference was statistically significant(P < 0.05). Hallucinations (81.3%)and irritability/emotional instability(62.5%)were more common in DLB group but apathy(76.9%),de-pression(73.7%)and anxiety(73.7%)were more common in PDD group. Conclusions Cognitive impairment is more progressive in DLB patients than that in PDD patients.Hallucinations and irritability/emotional instability are more common in DLB group but apathy,depression and anxiety are more common in PDD group.

Robin Williams' suicide: a case study / O suicídio de Robin Williams: estudo de caso

Abstract Introduction: The world renowned comedian and four-time Oscar nominated actor Robin Williams died on August 11, 2014. From the outset, the news indicated that his death was believed to be a suicide and this was later confirmed to be true by the autopsy reports. Williams had been suffering from severe depression, which is believed to be the leading contributor to his suicide. In this case study, I will highlight the event of the actor's suicide and the main risk factors along with depression leading to his tragic death. As of the end of 2015, no other case study seemed to have addressed or explored the links between the cause (or causes) and events leading to Robin Williams' suicide. Case description: Robin Williams was suffering from relationship problems, financial problems, drug addiction, and major depression. All of these factors led to his suicide. Comments: The chances of committing suicide drastically increase in the presence of any of the key risk factors. Unfortunately, the actor Robin Williams was dealing with four of the major risk factors all together, which put him at a high risk of committing suicide and eventually led to his tragic death.

Resumo Introdução: O ator Robin Williams, comediante reconhecido internacionalmente e quatro vezes indicado ao Oscar, faleceu no dia 11 de agosto de 2014. Desde o início, os noticiários indicavam que sua morte provavelmente resultara de suicídio, e isso foi confirmado posteriormente nos relatórios da autópsia. Williams vinha sofrendo de depressão severa, a qual, acredita-se, foi o fator que mais contribuiu para seu suicídio. Neste estudo de caso, vou discorrer sobre o suicídio do ator e sobre os principais fatores de risco, além da depressão, que levaram à sua morte trágica. Do final de 2015 para cá, nenhum outro estudo de caso parece ter abordado ou explorado as associações entre a causa (ou as causas) e os eventos que levaram ao suicídio do ator Robin Williams. Descrição do caso: Robin Williams vinha sofrendo com problemas de relacionamento, problemas financeiros, abuso de drogas e depressão severa. Todos esses fatores levaram ao seu suicídio. Comentários: As chances de cometer suicídio aumentam drasticamente na presença de qualquer fator de risco chaves. Infelizmente, o ator Robin Williams estava exposto a quatro fatores de risco chaves simultaneamente, colocando-o sob um alto risco de cometer suicídio, o que eventualmente culminou com sua trágica morte.

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.

Perfil clínico y neuropsicológico: enfermedad de Parkinson/enfermedad por cuerpos de Lewy / Neuropsychological profile: Parkinson’s Disease/Lewy Body Dementia

La demencia por cuerpos de Lewy y la enfermedad de Parkinson son dos síndromes comunes con signos y síntomas similares que hacen difícil establecer un diagnóstico exacto, especialmente en las etapas intermedias y tardías de cada cuadro clínico en particular. La enfermedad de Parkinson es una entidad lenta y progresiva que se caracteriza por temblor de reposo, rigidez, bradiscinesias e inestabilidad postural. En ella, los síntomas neurocognitivos y conductuales como la depresión, la disfunción ejecutiva y atencional, la baja fluidez verbal y las fallas de memoria inmediata pueden aparecer desde las etapas iniciales de la enfermedad e ir empeorando y progresando, cuando aparece un cuadro demencial secundario a la enfermedad. Por su parte, la enfermedad por cuerpos de Lewy se considera un cuadro neurodegenerativo que se ha asociado a la presencia de cuerpos de Lewy a nivel cortical y subcortical y se caracteriza por signos extrapiramidales, fluctuaciones cognitivas y alucinaciones visuales. En ella los síntomas neurocognitivos y conductuales son "fluctuantes" en lo relacionado con el nivel de consciencia, las funciones ejecutivas y atencionales, la memoria episódica y la presencia/ausencia de alucinaciones visuales. Las diferencias sutiles de ambas enfermedades exigen una revisión exhaustiva en la evolución de los síntomas. La evaluación neuropsicológica como herramienta diagnóstica se limita en tanto que no reemplaza, los estudios de imagen y otros hallazgos neuropatológicos para el diagnóstico definitivo, pero permite objetivar el perfil neuropsicológico más propio de cada enfermedad, en particular para un diagnóstico más preciso.

Lewy body dementia and Parkinson´s disease are two frequent syndromes that share similar signs and symptoms, especially during intermediate and chronic phases of each particular clinical picture, making an accurate diagnosis very difficult to establish. Parkinson’s disease is a slow and progressive disorder characterized by tremor at rest, stiffness, bradykinesia and postural imbalance. Many neurocognitive and behavioral symptoms such as depression, executive and attentional dysfunction, lower verbal fluency and immediate memory failures can appear in early stages of the disease, increasing and progressing as a secondary dementia develops. Lewy body dementia is considered a neurodegenerative disorder associated to Lewy bodies in both cortical and subcortical regions. It is characterized by the presence of extrapyramidal features, cognitive fluctuation and visual hallucinations. Neurocognitive and behavioral symptoms fluctuate concerning level of consciousness, executive and attentional function, episodic memory and the presence/absence of visual hallucinations. Subtle differences in both disorders demand an exhaustive review of symptom´s evolution. Neuropsychological evaluation as a diagnostic tool is limited. It cannot replace neuroimaging studies and other neuropathological findings for a definite diagnosis, but determines each neuropsychological profile in particular for a more accurate diagnosis.

A study of sleep architecture and cognitive functions in dementia with Lewy bodies / 中国医师杂志

Objective To investigate the sleep architecture in dementia with Lewy bodies (DLB),and study the sleep architecture and cognitive functions in DLB.Methods We described polysomnography (PSG) findings in 34 consecutive subjects diagnosed with DLB.All the patients underwent Mini-Mental State Examination (MMSE),Montreal Cognitive Assessment (MoCA),Clinical Dementia Rating (CDR) to quantify cognitive functions.Results (1)Sleep architecture analysis:DLB group compared to normal control group,the sleep period time (SPT) was reduced (P ＜ 0.05),total sleep time (TST) and sleep efficiency (SE) were decreased,total wake time (TWT) and wake after sleep onset (WASO) were increased,1 non-rapid eye movement (NREM) sleep (TS1),2NREM sleep (TS2),total NREM sleep (TNREMS),and REM sleep (TREMS) time were significantly decreased (P ＜0.01).(2)The DLB patients were divided into groups based on MMSE,MoCA,qnd CDR scores,the sleep architecture of each group was no significant difference (P ＞ 0.05).Conclusions Patients with DLB exists sleep architecture disorder.The cognitive functions and sleep architecture changes in patients with DLB have no obvious correlation.It is different from other degenerative dementia.

Qualidade de vida de quem cuida de portadores de demência com corpos de Lewy / Quality of life of caregivers of patients with Lewy body dementia

INTRODUÇÃO: Cuidar de portadores de demência pode provocar impacto na qualidade de vida dos familiares. OBJETIVO: Verificar a qualidade de vida de familiares que cuidam de portadores de demência com corpos de Lewy (DCL). MÉTODO: Estudo transversal, realizado com 90 familiares de portadores de DCL atendidos em hospital universitário de Goiânia/GO (Brasil), que responderam ao instrumento de avaliação de qualidade de vida WHOQOL-BREF. RESULTADOS: Os entrevistados eram 53,3% homens, na maioria casados e com ensino médio de escolaridade, com idade média de 47,4 ± 13,8 anos e média de tempo como cuidador de 13,9 ± 9,3 meses. O domínio Meio Ambiente obteve maior média (26,92 ± 5,88) e o Psicológico (19,66 ± 4,32) e Relação social (9,84 ± 2,18), as menores. A média dos domínios foi influenciada pelo sexo e pelo tempo que o familiar era cuidador. CONCLUSÃO: Atenção especial deve ser dada às alterações na saúde do cuidador, para que ele não se torne um "paciente oculto" e incapaz de lidar com as demandas do portador de DCL. O conhecimento das especificidades e compreensão dos sintomas da patologia auxiliam na adequação dos recursos pessoais para enfrentar as alterações comportamentais, apontadas como o fator mais impactante na vida do cuidador.

INTRODUCTION: Taking Taking care of people with dementia may have an impact on the quality of life for family members. OBJECTIVE: To verify the quality of life for family members who take care of people with dementia with Lewy bodies (DLB). METHOD: A cross-sectional study with 90 relatives of patients with DLB treated in a University hospital in Goiânia/GO (Brazil), who responded to the instrument of evaluation for the quality of life WHOQOL-BREF. RESULTS: From the people who were interviewed, 53.3% were male, most married and high school graduated, average age of 47.4 ± 13.8 years and average time as a care taker 13.9 ± 9.3 months. The Environment Domain got the highest average (26.92 ± 5.88), the Psychological (19,66 ± 4,32) and the Social Relationship (9.84 ± 2.18) the lowest. The average for the domains was influenced by genus and by the time that the family member was a care taker. CONCLUSION: Special attention should be given to changes in the health of caretakers, so they do not become a "hidden patient", and unable to deal with the demands of patients with DLB. The knowledge and understanding of the specific symptoms of the pathology helps to accommodate the human resources to face the behavioral changes, identified as the most impacting factor in the lives of caretakers.

Transmission of Synucleinopathies in the Enteric Nervous System of A53T Alpha-Synuclein Transgenic Mice

Parkinson's disease (PD) and dementia with Lewy bodies (DLB) are characterized by abnormal deposition of alpha-synuclein aggregates in many regions of the central and peripheral nervous systems. Accumulating evidence suggests that the alpha-synuclein pathology initiates in a few discrete regions and spreads to larger areas in the nervous system. Recent pathological studies of PD patients have raised the possibility that the enteric nervous system is one of the initial sites of alpha-synuclein aggregation and propagation. Here, we evaluated the induction and propagation of alpha-synuclein aggregates in the enteric nervous system of the A53T alpha-synuclein transgenic mice after injection of human brain tissue extracts into the gastric walls of the mice. Western analysis of the brain extracts showed that the DLB extract contained detergent-stable alpha-synuclein aggregates, but the normal brain extract did not. Injection of the DLB extract resulted in an increased deposition of alpha-synuclein in the myenteric neurons, in which alpha-synuclein formed punctate aggregates over time up to 4 months. In these mice, inflammatory responses were increased transiently at early time points. None of these changes were observed in the A53T mice injected with saline or the normal brain extract, nor were these found in the wild type mice injected with the DLB extract. These results demonstrate that pathological alpha-synuclein aggregates present in the brain of DLB patient can induce the aggregation of endogenous alpha-synuclein in the myenteric neurons in A53T mice, suggesting the transmission of synucleinopathy lesions in the enteric nervous system.

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.