Affiliations: [a] Memory Unit, Neurology Department, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain
| [b] Institut d’Investigacions Biomediques Sant Pau — Universitat Autònoma de Barcelona, Barcelona, Spain
| [c] Nuclear Medicine Department, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain
| [d] Centro de Investigación Biomédica en Red en Enfermedades Neurodegenerativas, CIBERNED, Instituto de Salud Carlos III, Madrid, Spain
Correspondence:
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Correspondence to: Alberto Lleó, MD, PhD, Memory Unit Director, Neurology Department, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. Tel.: +34 93 556 59 86; Fax: +34 93 556 56 02; E-mail: alleo@santpau.es.
Abstract: 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 clinical subtypes of DLB can be defined when considering the differential initial presentations. The proposed subtypes have distinct clinical profiles and progression patterns.
