. 2016 Feb 5;113(5):61–69. doi: 10.3238/arztebl.2016.0061

Table 1. Dementia with Lewy bodies.

Areas	Findings/recommendations
Neuropathology	Aggregates of α -synuclein in neuronal somata (Lewy bodies) and processes (Lewy neurites) in typical distribution (neocortex and brain stem)
Clinical syndrome	Dementia: cognitive impairments (attention, executive functions, visuospatial skills) with relevance to activities of daily living At least one of the following key characteristics: Parkinsonian syndrome, onset after or at most 12 months before onset of dementia Fluctuations in alertness and attention Repeated visual hallucinations
MRI results	Normal medial temporal lobes, in contrast to Alzheimer’s dementia Rule out symptomatic causes, for example, vascular encephalopathy, normal pressure hydrocephalus, aqueductal stenosis, encephalitis, tumor
Basic diagnostic evaluation	Neuropsychological tests, early visuospatial impairment (clock-drawing test, figure copying test), impaired speech (naming, verbal fluency), executive dysfunction, recommended Montreal Cognitive Assessment (MoCA), Frontal Assessment Battery (FAB); memory impairments may occur at a later stage
Extended diagnostic evaluation	¹²³I-FP-CIT-SPECT/¹⁸F-Dopa-PET: striatal dopaminergic denervation ⁹⁹ Tc-HMPAO-SPECT/ 18 FDG-PET: occipital hypometabolism ¹²³ I-MIBG-SPECT: cardiac noradrenergic denervation may occur Sleep laboratory: where applicable, rapid eye movement (REM) sleep behavior disorder
Symptomatic treatment	Cholinesterase inhibitors, for example, donepezil (1 × 5–10 mg) or rivastigmine (2–3 × 6–12 mg), for dementia (evidence level 1+, recommendation grade A) Memantine (1 × 5–20 mg) for behavioral symptoms (evidence level 1+, recommendation grade A) Levodopa (3–4 × 100–200 mg) for akinetic-rigid symptoms (evidence level 2+, recommendation grade B) Atypical neuroleptics, e.g., clozapine (6.25–100 mg/day, 2/3 of this at nighttime) or quetiapine (off-label, 25–100 mg at nighttime), for psychosis (evidence level 4, recommendation grade 0)

Evidence level 1 = randomized controlled study (studies); 2 = case-control study (studies); 3 = non-analytic case study (studies); 4 = expert opinion; recommendation grade A = strong recommendation [should]; B = recommendation; 0 = open recommendation [can]

MRI, magnetic resonance imaging; SPECT, single photon emission computed tomography; PET, positron emission tomography