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.

An Evaluation of Community Pharmacists' Knowledge and Experience regarding Dementia with Lewy Bodies: / 医薬品情報学

Objective: Owing to inconspicuous memory impairment during early disease stage, patients with dementia with Lewy bodies (DLB) are often diagnosed with mental disorders according to depressive symptoms and visual hallucinations. Severe sensitivity to antipsychotic agents, a DLB characteristic, increases mortality. Herein, we reviewed current challenges and approaches for early DLB detection and appropriate drug use by evaluating pharmacists' ability to recognition of DLB and their level of involvement in medication consultation with dementia patients.Designs: This is a cross-sectional study in Japan.Methods: We provided an anonymous self-administered survey questionnaire to 372 community pharmacists. Descriptive statistics,chi-square test (attributes, recognition, and experiences with medication consultation), and content analysis (free description of drug hypersensitivity) were used for data analysis.Results: The recognition rates for questions on DLB symptoms were as follows: visual hallucinations, 76%; delusion, 63%; other symptoms, including those categorized as core clinical features, such as fluctuating cognition, and REM sleep behavior disorder,＜40%. The rate of other symptoms was similar to that of false recognition of Alzheimer's disease symptoms. The recognition rate of certain DLB symptoms varied depending on pharmacists' experience in medication consultation with dementia patients and drug-induced evaluation during delirium/cognitive decline over the previous month. Approximately 65% of the participants did not respond to open questions on symptoms suggestive of drug hypersensitivity, whereas 55% of those who responded referred to allergic symptoms such as rashes.Conclusion: Owing to their lack of recognition of DLB symptoms, the current contribution of pharmacists to early DLB detection and proper drug use is limited. Thus, it is important to provide patients' observation points and method of questioning during interviews so that pharmacists can easily recognize DLB symptoms. It is critical to clarify that DLB drug hypersensitivity is attributed to mechanisms different from that of drug allergy.

Diagnosis and Clinical Progress in a Case of Dementia with Lewy Bodies / 생물치료정신의학

Dementia with Lewy bodies(DLB) is the second most common neurodegenerative disease. However, DLB might not be adequately diagnosed due to its variety of clinical symptoms. The authors present 65-year-old Mrs. A. who showed Parkinson's movement, cognitive decline, psychological symptoms, and autonomic dysfunction. According to the clinical features and biological markers in the recently revised DLB criteria, Mrs. A. was diagnosed with probable DLB. Differential diagnoses of delirium, Parkinson's dementia, and Alzheimer's dementia were discussed. Psychopharmacological treatments of antidepressants or anxiolytics caused intolerable side effects and showed little efficacy to Mrs. A. She experienced two episodes of hyponatremia during her one-year treatment. Recovery from neurological symptoms due to the first hyponatremia was time-consuming, and in the second, it was associated with changes in the level of consciousness despite relatively mild hyponatremia. A fall that occurred in the latter part of treatment triggered remarkable deterioration of DLB symptoms and daily life function. Prevention of falls is important for maintaining the quality of life of patients with DLB.

Demencia Por Cuerpos De Lewy, Un Reto Diagnóstico / Dementia With Lewy Bodies, A Diagnostic Challenge

Resumen La Demencia por cuerpos de Lewy es una enfermedad neurodegenerativa de etiología desconocida, corresponde a la segunda causa de demencia a partir de la sexta década de vida; su diagnóstico es un reto, debido a que ciertos de los signos y síntomas que presenta son típicos de la Enfermedad de Parkinson y la Enfermedad de Alzheimer. El siguiente reporte de caso es de los primeros en documentar un paciente con Demencia por cuerpos de Lewy en el Ecuador. Se expone un caso con Demencia por cuerpos de Lewy con el fin de plasmar la dificultad diagnóstica que genera esta patología y describir las características principales que la diferencian de otros síndromes demenciales, destacadas en los criterios recientemente actualizados por el Consorcio de Demencia por Cuerpos de Lewy. Un meticuloso examen neurológico y valoración neuropsicológica fueron ejes en el estudio y pronóstico del paciente que presentamos. La Demencia por cuerpos de Lewy requiere un diagnóstico minucioso, debido al desafío que origina su reconocimiento precoz; los criterios descritos aceleraron su reconocimiento gracias a la actualización de las recomendaciones sobre el diagnóstico clínico de Demencia por cuerpos de Lewy.

Abstract Dementia with Lewy bodies is a neurodegenerative disease of unknown etiology, it is the second cause of dementia of the sixth decade of life; Its diagnosis is a challenge, because certain signs and symptoms that it presents are typical of Parkinson's Disease and Alzheimer's Disease. The following case report is one of the few documented patients with Dementia with Lewy bodies in Ecuador. We report this in order to state the diagnostic difficulty that this pathology generates and describe the main characteristics that differentiate it from other dementia syndromes, highlighted in the recently updated criteria by the Consortium of Dementia with Lewy bodies. A meticulous neurological examination and neuropsychological assessment were essential in the study and prognosis of the patient. Dementia with Lewy bodies requires a thorough diagnosis, due to the challenge that originates its early recognition; the criteria described accelerated their recognition due the update of the recommendations on the clinical diagnosis of Dementia with Lewy bodies.

Neural structural imaging in the assessment of cognitive impairment / 医学研究生学报

As our country steps into the aging society gradually, the number of cognitive impairments and the prevalence rate are increasing yearly. The family and society bear a heavy burden. It is more important to explore the more direct and Objective morphological changes of cognitive impairment through neural structural imaging , which is better for early diagnosis, intervention and delay or even prevent its progress. Here we present a review of this topic focusing on neural structural imaging in the assessment of cognitive impairment.

Relationships between Rapid Eye Movement Sleep Behavior Disorder and Neurodegenerative Diseases: Clinical Assessments, Biomarkers, and Treatment / 中华医学杂志(英文版)

<p><b>Objective</b>Rapid eye movement sleep behavior disorder (RBD) is characterized by dream enactment and loss of muscle atonia during rapid eye movement sleep. RBD is closely related to α-synucleinopathies including Parkinson's disease, dementia with Lewy bodies, and multiple system atrophy. Many studies have investigated the markers of imaging and neurophysiological, genetic, cognitive, autonomic function of RBD and their predictive value for neurodegenerative diseases. This report reviewed the progress of these studies and discussed their limitations and future research directions.</p><p><b>Data Sources</b>Using the combined keywords: "RBD", "neurodegenerative disease", "Parkinson disease", and "magnetic resonance imaging", the PubMed/MEDLINE literature search was conducted up to January 1, 2018.</p><p><b>Study Selection</b>A total of 150 published articles were initially identified citations. Of the 150 articles, 92 articles were selected after further detailed review. This study referred to all the important English literature in full.</p><p><b>Results</b>Single-nucleotide polymorphisms in SCARB2 (rs6812193) and MAPT (rs12185268) were significantly associated with RBD. The olfactory loss, autonomic dysfunction, marked electroencephalogram slowing during both wakefulness and rapid eye movement sleep, and cognitive impairments were potential predictive markers for RBD conversion to neurodegenerative diseases. Traditional structural imaging studies reported relatively inconsistent results, whereas reduced functional connectivity between the left putamen and substantia nigra and dopamine transporter uptake demonstrated by functional imaging techniques were relatively consistent findings.</p><p><b>Conclusions</b>More longitudinal studies should be conducted to evaluate the predictive value of biomarkers of RBD. Moreover, because the glucose and dopamine metabolisms are not specific for assessing cognitive cognition, the molecular metabolism directly related to cognition should be investigated. There is a need for more treatment trials to determine the effectiveness of interventions of RBD on preventing the conversion to neurodegenerative diseases.</p>

Clinical Features and Pharmacological Treatment of Dementia with Lewy Bodies

Dementia with Lewy bodies (DLB) is the second most common causes of dementia. It can exhibit a variety of clinical symptoms including cognitive decline, cognitive fluctuation, visual hallucinations, parkinsonism, REM sleep behavior disorder, hypersensitivity to neuroleptics and autonomic dysfunctions. Despite more well-known criteria for DLB, there are often misdiagnosis and inappropriate treatment. It gives a lot of clinical burden to the clinician as well as to patients and families. When reducing the misdiagnosis, the burden of all will be reduced. The special concern and solicitation are needed in order not to miss the diagnosis when the cardinal features of DLB may not be volunteered by patients and the caregivers. To control the symptoms, clinicians must find and reduce drugs that can have the negative effects on DLB symptoms. There is limited evidence about specific interventions but available data suggest cholinesterase inhibitors improve the cognitive and behavioral symptoms and menmantine slightly improves the global impression.

Trial of the Dementia Differentiation Questionnaire-41 items (DDQ41) / 日本プライマリ・ケア連合学会誌

<b>Introduction</b> : To detect major symptoms of dementia, especially symptoms of non-Alzheimer-type dementia, we tried to develop an informant-based questionnaire, the Dementia differentiation questionnaire-41 items (DDQ41).<br><b>Methods</b> : The DDQ41 consisted of 11 questions on symptoms of early dementia (Q-Dementia11), 8 on Alzheimer's disease dementia(Q-ADD8), 9 on dementia with Lewy bodies (Q-DLB9), 8 on vascular dementia (Q-VD8), 5 on frontal lobe signs (Q-Frontal5), and additional 2 questions on urinary incontinence and speech disturbance. Caregivers of the 575 outpatients, who included only 1 diagnosis of dementia disease, checked the DDQ41.<br><b>Results</b> : Mean score of Q-Dementia11 in the MCI group was significantly lower than that in the other dementia groups. Mean score of Q-ADD8 in the ADD group was not significantly different from that in the other dementia groups. Mean score of Q-DLB9 in the DLB group was significantly higher than that in the other dementia groups. Area under the ROC curve of Q-DLB9 was 85.6%, and sensitivity and specificity were 82.6% and 77.7%(cut-off : 3 items/4 items), respectively, for DLB.<br><b>Conclusion</b> : We developed the DDQ41, an informant-based questionnaire sheet, for detecting symptoms of dementia. It may be useful in detecting frontal lobe signs and symptoms of non-Alzheimer-type dementia, especially those of DLB.

Diagnosis and Management of Dementia with Lewy Bodies

Dementia with Lewy bodies (DLB) is considered the second most common cause of neurodegenerative dementia. It is characterized with clinical and pathological feature that distinguish it from Alzheimer's disease (AD). These characteristics include fluctuating cognition, visual hallucination, and spontaneous feature of parkinsonism. DLB is commonly accompanied by neuropsychiatric symptoms and recent literature suggests that behavioral and psychological symptoms of dementia (BPDS) are frequently associated with DLB. So, psychiatrist caring for older adults may need a greater awareness of the clinical presentations of DLB in order to make a timely diagnosis. Also geriatric psychiatrist need to increase knowledge in order to raise awareness about the risks and benefits of medications in patients with DLB and to provide practical information and support for caregivers. This article reviews the clinical syndrome of DLB and its management.

Dementia with Lewy Bodies versus Alzheimer's Disease and Parkinson's Disease Dementia: A Comparison of Cognitive Profiles

BACKGROUND AND PURPOSE: It is particularly difficult to differentiate dementia with Lewy bodies (DLB) from the related dementias of Alzheimer's disease (AD) and Parkinson's disease dementia (PDD). Few studies have been designed to comparatively analyze detailed neuropsychological assessments of DLB patients and patients with AD and PDD. METHODS: Three groups of patients participated in this study: 10 with DLB, 76 with AD, and 17 with PDD, who had been diagnosed as probable DLB, AD, and PDD, respectively, according to the clinical criteria of the consortium on DLB, National Institute of Neurological and Communicative Diseases and Stroke/Alzheimer's Disease and Related Disorder Association, and the clinical diagnostic criteria for PDD. All patients were evaluated by careful neurological examination with detailed neuropsychological testing. RESULTS: Significant differences among the three groups were found for attention, memory, and executive function, which included tasks of backward digit span, three-word recall, verbal delayed recall, and the Stroop test. Post hoc analysis revealed that the deficiencies of attention on the digit span task were greater in the DLB group than in the AD and PDD groups. The scores for episodic verbal memory tasks were significantly lower in the DLB and AD groups than in the PDD group. The performance in frontal executive function, as indicated by the Stroop test, was significantly worse in the DLB and PDD groups than in the AD group. CONCLUSIONS: The results of the present study show that the pattern of cognitive dysfunction, in terms of attention, episodic memory, and executive functions, differ between patients with DLB and patients with AD and PDD.

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.

Comparison of cerebral glucose metabolism between Alzheimer's disease and dementia with lewy bodies / 中国综合临床

Objective To investigate the feature of cerebral glucose metabolism of Alzheimer's disease (AD)and dementia with lewy bodies (DLB).Methods 28 patients with AD and 25 patients with DLB underwent positron emission tomography (PET)with 18 F-fluorodeoxyglucose (18F-FDG)showing glucose metabolism.The region of interest (ROI)was selected from frontal cortex,temporal cortex,parietal cortex,occipital cortex,cerebellum cortex and corpora striata.The 18 F-FDG metabolism ratios between various cerebral regions and cerebellum cortex were compared as an indicator of regional cerebral glucose metabolic patterns.Results The FDG metabolism ratio of parietal cortex and temporal cortex decreased similarly in AD.The FDG metabolism ratio of frontal cortex,parietal cortex,temporal cortex,occipital cortex,dorsal caudate putamen and caudate nucleus in AD was [(0.861 ± 0.173),(0.625 ± 0.149),(0.598 ± 0.185 ),(0.914 ± 0.214),( 1.030 ± 0.084)and ( 0.997 ± 0.102 )].The FDG metabolism ratio of frontal cortex,parietal cortex,occipital cortex,temporal cortex and corpus striatum decreased similarly in DLB.The FDG metabolism ratio of frontal cortex,parietal cortex,temporal cortex,occipital cortex,dorsal caudate putamen and candate nucleus in DLB was [ (0.538 ±0.147),(0.615 ±0.138),( 0.587 ±0.142),(0.415 ±0.107 ),(0.685 ± 0.094)and (0.547 ± 0.103 )].The FDG metabolism ratio of frontal cortex,occipital cortex and corpus striatum decreased more significantly in DLB than in AD (P＜0.01 ).Conclusion There are discrepancies in cerebral glucose metabolism between AD and DLB.These features may be useful in differential diagnosis of these two kinds of dementia.

Imaging Improves Diagnosis of Dementia with Lewy Bodies

Dementia with Lewy bodies (DLB) is the second most common cause of degenerative dementia after Alzheimer's disease (AD), and is clinically characterized by the progressive cognitive decline with fluctuations in cognition and alertness, recurrent visual hallucinations and Parkinsonism. Once these characteristic symptoms of DLB emerge, discriminating it from AD is relatively easy. However, in the early disease stages, the clinical symptoms of various types of dementias largely overlap and it is difficult to distinguish DLB from other neurodegenerative dementias based on clinical manifestations alone. To increase the accuracy of antemortem diagnosis of DLB, the latest diagnostic criteria incorporate findings from 123I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy, or from neuroimaging such as computed tomography (CT), magnetic resonance imaging (MRI), single photon emission computed tomography (SPECT), and positron emission tomography (PET). In the present guidelines, decreased dopamine transporter uptake revealed by SPECT or PET receives the greatest importance among various neuroimaging findings and is listed as one of the suggestive features. Supportive features that commonly present but are not proven to have diagnostic specificity include relatively-preserved medial-temporal-lobe structures, occipital hypoperfusion, and abnormal MIBG myocardial scintigraphy. In this paper, we review the major findings on various neuroimaging modalities and discuss the clinical usefulness of them for the diagnosis of DLB. Although there is not enough evidence to reach the conclusion, considering the accessibility in clinical practice, in our personal views, we recommend the use of brain-perfusion SPECT and MIBG myocardial scintigraphy to improve the diagnosis of DLB.

Imaging Improves Diagnosis of Dementia with Lewy Bodies

Dementia with Lewy bodies (DLB) is the second most common cause of degenerative dementia after Alzheimer's disease (AD), and is clinically characterized by the progressive cognitive decline with fluctuations in cognition and alertness, recurrent visual hallucinations and Parkinsonism. Once these characteristic symptoms of DLB emerge, discriminating it from AD is relatively easy. However, in the early disease stages, the clinical symptoms of various types of dementias largely overlap and it is difficult to distinguish DLB from other neurodegenerative dementias based on clinical manifestations alone. To increase the accuracy of antemortem diagnosis of DLB, the latest diagnostic criteria incorporate findings from 123I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy, or from neuroimaging such as computed tomography (CT), magnetic resonance imaging (MRI), single photon emission computed tomography (SPECT), and positron emission tomography (PET). In the present guidelines, decreased dopamine transporter uptake revealed by SPECT or PET receives the greatest importance among various neuroimaging findings and is listed as one of the suggestive features. Supportive features that commonly present but are not proven to have diagnostic specificity include relatively-preserved medial-temporal-lobe structures, occipital hypoperfusion, and abnormal MIBG myocardial scintigraphy. In this paper, we review the major findings on various neuroimaging modalities and discuss the clinical usefulness of them for the diagnosis of DLB. Although there is not enough evidence to reach the conclusion, considering the accessibility in clinical practice, in our personal views, we recommend the use of brain-perfusion SPECT and MIBG myocardial scintigraphy to improve the diagnosis of DLB.

Comparison of Cerebral Glucose Metabolism between Dementia with Lewy Bodies and Parkinson's Disease Dementia / 中国康复理论与实践

@# Objective To investigate the feature of cerebral glucose metabolism of dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PDD). Methods Twenty-five DLB patients and Thirty-one PDD patients underwent positron emission tomography(PET) with 18F-fluorodeoxyglucose (18F-FDG) showing glucose metabolism. The region of interest (ROI) was selected from frontal cortex, temporal cortex, parietal cortex, occipital cortex, cerebellum cortex and corpora striata. 18F-FDG metabolism ratios between various cerebral regions and cerebellum cortex were compared as an indicator of regional cerebral glucose metabolic patterns.Results FDG metabolism ratio of frontal cortex, occipital cortex, parietal cortex, temporal cortex and corpus striatum decreased similarly in DLB and PPD (P>0.05). FDG metabolism ratio of occipital cortex decreased more significantly in DLB than in PDD (P<0.01). The decrease of FDG metabolism in corpus striatum of DLB was symmetric. For patients with PDD, FDG metabolism in corpus striatum contralateral to onset side decreased more significantly than that of corpus striatum ipsilateral to onset side (P<0.05). Conclusion There are similarities and discrepancies in cerebral glucose metabolism between DLB and PDD. These features may be useful in differential diagnosis between these two kinds of Lewy body disease.

Dementia With Lewy Bodies Diagnosed by Cognitive Fluctuation After Anticholinergic Medication

Dementia with Lewy bodies (DLB) usually presents with progressive cognitive decline and parkinsonism. We report a 68-year-old man who showed parkinsonism including resting tremor and mild cognitive decline. After anticholinergic medication, he showed recurrent visual hallucination. Neuropsychological tests revealed frontal and memory impairments. PET showed hypometabolism in the primary visual and visual association cortices. It is necessary to consider the possibility of DLB as well as anticholinergic side effect when visual hallucination occurs during the treatment of parkinsonism.

FDG PET Imaging For Dementia / 핵의학분자영상

Dementia is a major burden for many countries including South Korea, where life expectancy is continuously growing and the proportion of aged people is rapidly growing. Neurodegenerative disorders, such as, Alzheimer disease, dementia with Lewy bodies, frontotemporal dementia, Parkinson disease, progressive supranuclear palsy, corticobasal degeneration, Huntington disease, can cause dementia, and cerebrovascular disease also can cause dementia. Depression or hypothyroidism also can cause cognitive deficits, but they are reversible by management of underlying cause unlike the forementioned dementias. Therefore these are called pseudodementia. We are entering an era of dementia care that will be based upon the identification of potentially modifiable risk factors and early disease markers, and the application of new drugs postpone progression of dementias or target specific proteins that cause dementia. Efficient pharmacologic treatment of dementia needs not only to distinguish underlying causes of dementia but also to be installed as soon as possible. Therefore, differential diagnosis and early diagnosis of dementia are utmost importance. F-18 FDG PET is useful for clarifying dementing diseases and is also useful for early detection of the diseases. Purpose of this article is to review the current value of FDG PET for dementing diseases including differential diagnosis of dementia and prediction of evolving dementia.

Dementia with Lewy Bodies

Here the author reviews the clinical and pathologic characteristics of dementia with Lewy bodies (DLB). DLB took many years to crystallize into a recognizable clinico-pathologic entity. Based on sensitive immunostaining technique, DLB is now considered the second most commonest cause of neurodegenerative dementia in the elderly. It is part of the range of clinical presentations that share a neuritic pathology based on abnormal aggregation of the synaptic protein alpha-synuclein. Lewy body pathology is found from the brainstem to the cortex and, in many cases, associated with concurrent Alzheimer' disease pathology. A recent international consortium on DLB has resulted in revised criteria for the clinical and pathological diagnosis of DLB incorporating new information about the clinical features and improved methods for their assessment. Neuropathologic diagnosis now assigns a weight to both alpha-synuclein and Alzheimer tangle pathology. Accurate identification of patients is important because they have specific symptoms, impairments, and functional disabilities that differ from those of other dementing illness including Alzheimer's disease.

PET studies in Alzheimer Disease and Other Degenerative Dementias / 대한핵의학회잡지

Neurodegenerative disorders cause a variety of dementia including Alzheimer disease, frontotemporal dementia, dementia with Lewy bodies, corticobasal degeneration, progressive supranuclear palsy, and Huntington's disease. PET scan is useful for early detection and differential diagnosis of these dementing disorders. Also, it provides valuable information about clinico-anatomical correlation, allowing better understanding of function of brain. Here we discuss recent achievements PET studies regarding these dementing disorders. Future progress in PET technology, new tracers, and image analysis will play an important role in further clarifying the disease pathophysiology and brain functions.

Clinical Features, Drug Responsiveness and Neuroimaging Findings in Dementia with Lewy Bodies

BACKGROUND: Dementia with Lewy bodies (DLB) is the second common degenerative dementia and has several characteristics including fluctuating cognition, visual hallucination and parkinsonism. We investigated clinical manifestations, responsiveness to drugs and neuroimaging findings in DLB patients. METHODS: Ten probable DLB patients were included in this study. For responsiveness to drugs, we measured Unified Parkinson's Disease Rating Scale (UPDRS) of rest tremor, rigidity and bradykinesia (finger tapping and leg agility), and Korean version of mini-mental state examination (K-MMSE) before and after administration of levodopa or cholinesterase inhibitors. Brain MRI, cerebral perfusion SPECT (Tc-99m HMPAO) and dopamine transporter SPECT (123I-IPT) were also performed. RESULTS: All patients were men and mean age of onset was 66.9 years (range from 58 to 80). All had fluctuating cognition and parkinsonism, and 7 had visual hallucination. Mean increment of K-MMSE after administration of cholinesterase inhibitors was 2.1 points. Levodopa reduced UPDRS scores of tremor, rigidity and bradykinesia by 0.4, 0.6 and 1.0 points, respectively. Cerebral perfusion SPECT using Tc-99m HMPAO showed hypoperfusion in occipital area as well as fronto-temporoparietal areas. Dopamine transporter SPECT using 123I-IPT revealed reduced uptake comparable to Parkinson's disease in the striatum. CONCLUSIONS: DLB should be first considered as one of possible diagnoses in patients showing dementia in the early stage of parkinsonism. Responsiveness of parkinsonism to levodopa seems favorable. Cholinesterase inhibitors are thought promising in enhancing cognition in short term periods. It is suggested that occipital hypoperfusion be a characteristic finding in DLB and be helpful in differentiating DLB from other degenerative dementias.