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.
Keywords: Antipsychotics; Cholinesterase inhibitors; DaT scan; Dementia with Lewy bodies; Diagnosis; REM behavior disorder.
Sensitivity and Specificity of Dopamine Transporter Imaging With 123I-FP-CIT SPECT in Dementia With Lewy Bodies: A Phase III, Multicentre StudyI McKeith et al. Lancet Neurol 6 (4), 305-13. PMID 17362834. - Clinical TrialA revision of the International Consensus Criteria for DLB has recommended that low DAT uptake in the basal ganglia, as shown by SPECT or PET imaging, be a suggestive fea …
Dementia With Lewy BodiesD Weisman et al. Semin Neurol 27 (1), 42-7. PMID 17226740. - ReviewSynucleinopathies, with and without dementia, encompass a wide range of diseases including Parkinson's disease, multiple system atrophy, rapid eye movement (REM) sleep be …
[Revised 2017 Clinical Diagnostic Criteria for Dementia With Lewy Bodies: Inclusion of REM Sleep Behavior Disorder and Indicative Biomarkers]H Fujishiro. Brain Nerve 70 (8), 869-877. PMID 30082497. - ReviewAlthough dementia with Lewy bodies (DLB) is the second most common neurodegenerative disorder after Alzheimer disease, its diagnostic sensitivity in clinical practice is …
Diagnosis and Management of Dementia With Lewy Bodies: Third Report of the DLB ConsortiumIG McKeith et al. Neurology 65 (12), 1863-72. PMID 16237129. - ReviewThe dementia with Lewy bodies (DLB) Consortium has revised criteria for the clinical and pathologic diagnosis of DLB incorporating new information about the core clinical …
Early Detection of Dementia With Lewy Bodies in Patients With Amnestic Mild Cognitive Impairment Using 123I-MIBG Cardiac ScintigraphyH Fujishiro et al. J Neurol Sci 315 (1-2), 115-9. PMID 22129938. - Case ReportsIncreasing clinical attention has been focused on cardiac sympathetic denervation for the differential diagnosis of dementia with Lewy bodies (DLB) from Alzheimer's disea …
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Clinical Drug Development for Dementia With Lewy Bodies: Past and PresentG Lee et al. Expert Opin Investig Drugs 28 (11), 951-965. PMID 31614096. - ReviewIntroduction: Dementia with Lewy bodies (DLB) is an under-researched area despite being the second most common type of degenerative dementia after Alzheimer's dise …