Hallucinations and signs of parkinsonism help distinguish patients with dementia and cortical Lewy bodies from patients with Alzheimer's disease at presentation: a clinicopathological study (original) (raw)
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Early and Presenting Symptoms of Dementia with Lewy Bodies
Dementia and Geriatric Cognitive Disorders, 2011
Background/Aims: To explore the presenting and early symptoms of dementia with Lewy bodies (DLB). Method: Patients with mild dementia fulfilling diagnostic criteria for DLB (n = 61) and Alzheimer's disease (AD) (n = 109) were recruited from outpatient dementia clinics in western Norway. At diagnosis, caregivers were asked which symptom had been the presenting symptom of dementia. Results: Caregivers reported that memory impairment was the most common presenting symptom in DLB (57%), followed by visual hallucinations (44%), depression (34%), problem solving difficulties (33%), gait problems (28%), and tremor/stiffness (25%). In contrast, 99% of AD carers reported impaired memory as a presenting symptom, whereas visual hallucinations were a presenting symptom in 3% of the AD cases. Conclusion: DLB should be suspected in predementia cases with visual hallucinations.
Behavioural and Psychological symptoms in a case of Dementia with Lewy Bodies
2018
Behavioural and psychological symptoms of dementia (BPSD) are common in dementia. These symptoms differ with the type of dementia. Dementia with lewy bodies(DLB) is a subtype of dementia characterised by visual hallucinations and sleep disorders. There are many criteria for diagnosing DLB including the behavioural and psychological symptoms. But clinical application of such criteria remains limited. Untreated BPSD symptoms can cause significant distress to patient and caregiver. This case report highlights the importance of proper application of the latest criteria for DLB and also the importance of early and prudent management of BPSD symptoms in DLB.
A prospective study of dementia with Lewy bodies
Age and Ageing, 1998
little is known about the longitudinal course of dementia with Lewy bodies (DLB) and how this differs from Alzheimer's disease (AD). Method: standardized baseline and annual assessments of cognitive and non-cognitive symptoms are reported in a cohort of 72 patients with DLB or AD. AD was diagnosed using the NLNCDS ADRDA criteria and DLB was diagnosed with the criteria of McKeith et al. Cognitive assessment was undertaken using the MMSE schedule and operationalized definitions were used to diagnose non-cognitive symptoms. Results: 42 patients with DLB and 30 patients with AD were assessed. Of the 19 on whom post mortem examinations have been performed, 18 (95%) have had the clinical diagnosis confirmed. DLB patients were significantly more likely to experience visual hallucinations, disturbances of consciousness and parkinsonism at both baseline and at annual assessments. Of DLB patients exposed to neuroleptics, 33% developed sensitivity reactions. The magnitude and pattern of cognitive decline was similar in both groups. Conclusion: the importance of the core features highlighted in the newly proposed consensus DLB criteria is supported. These features appear to be stable over time.
Psychiatric symptoms in dementia with Lewy Bodies: Diagnostic and management issues
2011
Dementia with lewy bodies is the second most common type of dementia, but may often be misdiagnosed in clinical situations. It commonly presents with visual hallucinations and fluctuating cognition on a background of parkinsonian features and progressive cognitive decline in an elderly patient. A variety of psychiatric symptoms may occur in the course of the illness and often, there is an extreme sensitivity to neuroleptic drugs. In view of several complex and challenging issues, we discuss a case of dementia with lewy bodies from a diagnostic and management perspective.
Advances in Psychiatric Treatment, 1998
Lewy body formation is central to the pathological phenotype of a spectrum of disorders. The most familiar of these is the extrapyramidal syndrome of idiopathic Lewy-body Parkinson's disease (PD). Studies of dementia in the elderly suggest that another manifestation of Lewy body pathology is equally or more common than Parkinson's disease. This syndrome of Dementia with Lewy bodies (DLB) has been given a number of diagnostic labels and is characterised by dementia, relatively mild parkinsonism, visual hallucinations, and fluctuations in conscious level. Although many of these features can arise in Parkinson's disease, the patients with DLB tend to have early neuropsychiatric features which predominate the clinical picture, and the diagnosis of the syndrome in practice is more concerned with the differential diagnosis of Alzheimer's disease (AD). Distinction from AD has clinical importance because of potentially differing therapeutic implications. Diagnostic guidelines for the clinical diagnosis and pathological evaluation of DLB are reviewed. Research into the disorder has centered around characterising the clinical, neuropsychological, pathological, neurochemical and genetic relationships with Alzheimer's disease on the one hand, and Parkinson's disease on the other. Many cases of DLB have prominent pathological features of AD and there are some shared genetic risk factors. Differences from the pathology of PD are predominantly quantitative rather than qualitative and evidence is discussed which suggests that DLB represents a clinicopathological syndrome within the spectrum of Lewy body disorders. The possibility that the syndrome represents a chance association of PD and AD is not supported by published studies.
Dementia with Lewy bodies: findings from an international multicentre study
International Journal of Geriatric Psychiatry, 2000
Objectives[ To describe the baseline demographic\ neuropsychiatric and neurological data of a large selected clinical sample of patients with dementia with Lewy Bodies "DLB# from an international multicentre trial with rivastigmine[ To examine the usefulness of the Consensus Criteria for the diagnosis of DLB in di}erent countries[ Methods[ Seventeen centres from Spain\ the UK and Italy recruited patients diagnosed clinically as probable DLB according to recent Consensus Criteria "McKeith et al[\ 0885#[ A standard clinical protocol including inclu! sion:exclusion criteria\ collection of demographic and medical data\ cognitive "Mini Mental State Examination] MMSE#\ motor "Uni_ed Parkinson|s Disease Rating Scale] UPDRS# and neuropsychiatric "Neuropsychiatric Inven! tory] NPI# examinations\ was applied after obtaining informed consent[ Data were summarised and compared across countries with uni! and multivariate analyses[ Results[ One hundred and twenty patients were recruited] 45[6) males\ mean "SD# age 62[8 "5[3# years\ range 46Ð 76 years[ Sixty percent ful_lled all three core diagnostic features of DLB\ and 39) only two "{parkinsonism| 81[3)\ {cognitive~uctuations| 78[0)\ {visual hallucinations| 66[2)#[ {Systematised delusions| "35)# and {repeated falls| "31)# were the most frequent supportive diagnostic features[ There were no di}erences across countries in demo! graphic\ diagnostic or clinical features[ Patients showed a wide range of psychopathology which was weakly correlated with cognitive impairment[ Some mild extrapyramidal signs "EPS# were observed in most patients[ Conclusions[ The Consensus Criteria for DLB can be consistently applied across many di}erent sites for multicentre studies[ {Parkinsonism| and {cognitive~uctuations| as core features and {systematised delusions| and {repeated falls| as supportive features are the most frequent diagnostic clues[ Neuropsychiatric disturbances\ in particular apathy\ delusions\ hallucinations and anxiety\ and mild symmetric EPS are frequent in DLB and are only related weakly to cognitive impairment[
Movement Disorders, 2010
Visual Hallucinations (VH) are among the core features of Dementia with Lewy Bodies (DLB), but are also very frequent in demented patients with Parkinson's Disease (PDD). The purpose of this study was to investigate the pattern of gray matter and cognitive impairment underlying VH in DLB and PDD. We applied voxel-based morphometry and behavioral assessment to 12 clinically diagnosed DLB patients and 15 PDD patients. Subjects with VH showed greater gray matter loss than non-hallucinators, specifically in the right inferior frontal gyrus (BA 45) in the DLB patients and in the left orbitofrontal lobe (BA 10) in the PDD patients. Comparing the two subgroups with VH, DLB patients had greater decrease of the bilateral premotor area (BA 6) than PDD patients. Furthermore, decreased volume in associative visual areas, namely left precuneus and inferior frontal lobe, correlated with VH in the DLB but not in PDD patients. VH were related to impaired verbal fluency, inhibitory control of attention and visuoperception in the DLB group and to visual memory in the PDD group. In conclusion, DLB and PDD patients with VH had more frontal gray matter atrophy than non-hallucinators, the impairment being greater in the DLB group. The patterns of structural and functional correlations were different in both pathologies.
Clinical Subtypes of Dementia with Lewy Bodies Based on the Initial Clinical Presentation
Journal of Alzheimer's Disease, 2018
Background: Dementia with Lewy bodies (DLB) is a heterogeneous disease in which clinical presentation, symptoms, and evolution widely varies between patients. Objective: To investigate the existence of clinical subtypes in DLB based on the initial clinical presentation. Methods: 81 patients with a clinical diagnosis of probable DLB were consecutively included. All patients underwent a neurological evaluation including a structured questionnaire about neuropsychiatric symptoms and sleep, an assessment of motor impairment (Unified Parkinson Disease Rating Scale subscale III), and a formal neuropsychological evaluation. Onset of core symptoms (hallucinations, parkinsonism, and fluctuations) and dementia were systematically reviewed from medical records. We applied a K-means clustering method based on the initial clinical presentation. Results: Cluster analysis yielded three different groups. Patients in cluster I (cognitive-predominant, n = 46) presented more frequently with cognitive symptoms (95.7%, n = 44, p < 0.001), and showed a longer duration from onset to DLB diagnosis (p < 0.001) than the other clusters. Patients in cluster II (neuropsychiatric-predominant, n = 22) were older at disease onset (78.1 ± 5 versus 73.6 ± 6.1 and 73.6 ± 4.2 in clusters I and III, respectively, both p < 0.01), presented more frequently with psychotic symptoms (77.3%, n = 17), and had a shorter duration until the onset of hallucinations (p < 0.001). Patients in cluster III (parkinsonism-predominant, n = 13) showed a shorter time from onset to presence of parkinsonism (p < 0.001) and dementia (0.008). Conclusions: Three subtypes of clinical DLB can be defined when considering the differential initial presentations. The proposed subtypes have distinct clinical profiles and progression patterns.
Diagnosis and management of dementia with Lewy bodies
The dementia with Lewy bodies (DLB) Consortium has revised criteria for the clinical and pathologic diagnosis of DLB incorporating new information about the core clinical features and suggesting improved methods to assess them. REM sleep behavior disorder, severe neuroleptic sensitivity, and reduced striatal dopamine transporter activity on functional neuroimaging are given greater diagnostic weighting as features suggestive of a DLB diagnosis. The 1-year rule distinguishing between DLB and Parkinson disease with dementia may be difficult to apply in clinical settings and in such cases the term most appropriate to each individual patient should be used. Generic terms such as Lewy body (LB) disease are often helpful. The authors propose a new scheme for the pathologic assessment of LBs and Lewy neurites (LN) using alpha-synuclein immunohistochemistry and semiquantitative grading of lesion density, with the pattern of regional involvement being more important than total LB count. The new criteria take into account both Lewy-related and Alzheimer disease (AD)-type pathology to allocate a probability that these are associated with the clinical DLB syndrome. Finally, the authors suggest patient management guidelines including the need for accurate diagnosis, a target symptom approach, and use of appropriate outcome measures. There is limited evidence about specific interventions but available data suggest only a partial response of motor symptoms to levodopa: severe sensitivity to typical and atypical antipsychotics in ϳ50%, and improvements in attention, visual hallucinations, and sleep disorders with cholinesterase inhibitors. NEUROLOGY 2005;65:1863-1872 Clinical diagnostic criteria for DLB. Since the publication of Consensus criteria for clinical and pathologic diagnosis of dementia with Lewy bodies (DLB), 1,2 new information indicates that clinical criteria for probable DLB have acceptable specificity, but suboptimal sensitivity. Reasons identified in-clude difficulties in recognition of the core feature fluctuation 5,6 and a low rate of all core features (fluctuation, visual hallucinations, parkinsonism) in the presence of neocortical, neurofibrillary tangle (NFT) pathology. 7-9 The criteria have therefore been modified (table 1) to incorporate additional items indicative of LB pathology. Distinction is made between clinical features or investigations that are suggestive of DLB, i.e., have been demonstrated to be significantly more frequent than in