Dementia with Lewy (DLB) bodies is the most common type of degenerative dementia after Alzheimer Disease (AD). Although an accurate diagnosis of DLB is important for adequate prognosis and therapy, its differentiation from other dementias and, especially, from AD may be extremely challenging for clinicians, as highlighted by the high variability in reported sensitivity (0.22 -0.83) and specificity (0.79 -1.00) rates for a diagnosis of probable DLB applying current clinical criteria. Various kinds of imaging procedures, including conventional MRI and brain perfusion SPECT, have been proposed for improving diagnostic accuracy, especially for most controversial cases. Among such techiques, those using radioactive tracers measuring the striatal binding at pre-synaptic dopamine transporter sites or myocardial uptake in post-ganglionic sympathetic fibers have emerged as the most useful for diagnostic purposes.Keywords: Alzheimer disease, Dementia with Lewy bodies, DAT scan, MIBG myocardial scintigraphy.
DEMENTIA WITH LEWY BODIES: CLINICAL PHENOMENOLOGY AND DIAGNOSTIC CONTRO-VERSIESDementia with Lewy bodies (DLB) has been reported to be the second most common form of degenerative dementia, after Alzheimer Disease (AD). The importance of identifying this entity lies essentially in its pharmacologic management, with a potential good response to cholinesterase inhibitors [1], but increased sensitivity to adverse effects of neuroleptic drugs [2,3].Much attention has been focused on the identification of reliable criteria that may help the clinician to discriminate DLB from other dementias, and especially from AD [4]. In addition to cognitive decline, the core clinical features of DLB, according to the Consortium on DLB criteria [4,5], are visual hallucinations (VH), which typically are recurrent, well formed and detailed, fluctuating cognition with pronounced variations in attention and alertness, and spontaneous (i.e., not drug-induced) features of parkinsonism, with an overrepresentation of the "postural instability-gait difficulty" phenotype [6]. The motor manifestations of parkinsonism may also include limb rigidity, bradykinesia, and a symmetrical postural tremor, while unilateral rest tremor is relatively uncommon. Neuropsychologically, compared to AD, patients with DLB tend to show a different pattern of cognitive impairment, with more preserved memory [7], but worse performances on attentional and executive tasks [8,9] and on tests of visuospatial/constructional abilities [10,11]. However, despite emphasis placed on these distinctive characteristics, while neuropathologic series have demonstrated high *Address correspondence to this author at the Department of Neurology Ospedale Niguarda Ca' Granda, Milano, Italy; Tel: +39 02 6444.2137; Fax: +390264442819; E-mail: ptiraboschi@yahoo.com accuracy for the clinical diagnosis of AD [12], the accuracy for the clinical diagnosis of DLB using the criteria originally suggested by the Consortium on DLB [4] has been in general less satisfactory [13][14][15][16][17],...