Clinical aspects of dementia with Lewy bodies (original) (raw)
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Diagnosis and management of dementia with Lewy bodies: Third report of the DLB Consortium
Neurology, 2006
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
Journal of Neuropathology and Experimental Neurology, 2008
There is limited information on the validity of the pathologic criteria of the Third Consortium on Dementia with Lewy bodies (CDLB), and none are based on prospectively diagnosed cases. In this study, the core clinical features of dementia with Lewy bodies (DLB) and the suggestive clinical feature of rapid eye movement sleep behavior disorder were assessed using a battery of standardized clinical instruments in 76 patients with the clinical diagnosis of either DLB or Alzheimer disease. At autopsy, 29 patients had high-likelihood, 17 had intermediate-likelihood, and 6 had low-likelihood DLB pathology. The frequency of core clinical features and the accuracy of the clinical diagnosis of probable DLB were significantly greater in high-likelihood than in low-likelihood cases. This is consistent with the concept that the DLB clinical syndrome is directly related to Lewy body pathology and inversely related to Alzheimer pathology. Thus, the Third Consortium on DLB neuropathologic criteria scheme performed reasonably well and are useful for estimating the likelihood of the premortem DLB syndrome based on postmortem findings. In view of differences in the frequency of clinically probable DLB in cases with Braak neurofibrillary tangle stages V (90%) and VI (20%) and diffuse cortical Lewy bodies, a possible modification of the scheme is to consider cases with neurofibrillary tangle stage VI to be lowlikelihood DLB.
Diagnosis and Management of Cognitive and Behavioral Changes in Dementia With Lewy Bodies
Current Treatment Options in Neurology, 2017
This article is part of the Topical Collection on Dementia Keywords Dementia with Lewy bodies I Diagnosis I REM behavior disorder I DaT scan I Cholinesterase inhibitors I Antipsychotics Opinion statement Proper diagnosis of dementia with Lewy bodies (DLB) in clinical practice remains suboptimal as many cases are misdiagnosed, usually as Alzheimer disease (AD) or Parkinson's disease (PD) and, in rare cases, psychosis. Therefore, it is important for patients with dementia to be thoroughly evaluated by a specialist who is familiar with current diagnostic tests and treatment options. New diagnostic criteria from the Dementia with Lewy Bodies Consortium have been developed to increase diagnostic sensitivity for DLB (Diagnosis and management of dementia with Lewy bodies: fourth consensus report of the DLB Consortium; McKeith et al.; Neurology, 89(1): 88-100). REM sleep behavior disorder (RBD) has been studied more thoroughly in correlation with DLB and is now considered a core feature. D2 receptor blocking antipsychotics, which can cause severe antipsychotic sensitivity, are now rarely prescribed for treatment. Therefore, severe antipsychotic sensitivity, which was a suggestive criterion for DLB diagnosis, is now listed as a supportive feature. Reduced DAT uptake in basal ganglia demonstrated by SPECT or PET imaging has high specificity (90%) for distinguishing DLB from AD. Reduced uptake on metaiodobenzylguanidine myocardial scintigraphy correlates with reduced postganglionic sympathetic cardiac innervation in Lewy body diseases, which can increase specificity for discriminating probable DLB from probable AD in milder cases of dementia. However, the latter is more commonly used in Japan and is not used in the USA. The evidence supporting the benefit of other therapeutic modalities is limited in DLB due to lack of extensive studies. There are no FDA-approved medications for the treatment of DLB, although some effective drugs have been used off label to treat various symptoms.
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
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.
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[