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.

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.

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.

Psychosis in Dementia

Psychosis in patients with dementia contributes substantially to patient morbidity and caregiver distress. The concept of psychosis of Alzheimer's disease (AD) and other types of dementia is developed with respect to prevalence, incidence, clinical characteristics, clinical course, and potential response to treatment. This article provides an overview of concept of psychosis in dementia. Published prevalence estimates of psychosis in patients with AD range from 10 to 73% within clinical populations. There is a continuing persistence of psychotic symptomatology among people with AD;most patients with psychosis continue to fulfill criteria for psychosis of dementia over at least 3 months, and over a half may have psychotic symptoms persist over a year. Among people with AD who have no psychotic symptoms there appears to be an annualized incidence of psychosis of about 20% in outpatients, and a much higher rate in nursing home patients. Frontal hypometabolism and greater frontal neuropsycological deficits occur in AD patients with psychosis in comparison to those without. There is some evidence that psychotic symptoms improve modestly with antipsychotic medication treatment, although optimal treatment guidelines have been elusive. The characteristics of psychosis in Parkinson's disease, Lewy body dementia, frontotemporal dementia and vascular dementia were also reviewed. Conclusively, further research to support the validity of a syndrome of psychosis in various types of dementia, as well as AD is needed.

Decisões clínicas 3 "Ele não anda mais" / Clinical decisions 3 "He can no longer walk"

A 79-year-old male with cognitive impairment and parkinsonism is described. The use of the clinical method is highlighted as a sure way of leading to the eventual disgnosis of Dementia with Lewy Bodies.

Clinical Features of Other Dementias

Dementias can be calssified into cortical, subcortical, cortical-subcortical and multifocal ones based on the major pathological distribution within the brain. The literatures of recent knowledge about clinical features of other dementias than Alzheimer's and vascular ones, which were most frequently experienced by many clinicians were reviewed. That is, cortical dementias such as Pick's disease, frontal lobe type dementia and non-Alzheimer's type lobar atrophy including fronto-temporal dementia, progressive dysphasia, fronto-temporal dementia with motor neuron disease, and alcohol-related dementia were reviewed. Subcortical dementias such as dementias accompanying Parkinson's disease, Huntington's disease and progressive supranuclear palsy, and cortical-subcortical dementias such as Lewy body dementiaq and cortical-basal degeneration were also reviewed. As multifocal dementias, prion dementias including KUru, Creutzfeldt-Jakob disease, fatal familial insomnia and Gerstmann-Strussler-Sheinker syndrone, and AIDS dementia were also reviewed.