title: Rapidly progressive dementia: experience in a tertiary care medical center.
abstract: Diagnosis of rapidly progressive dementia (RPD) poses a complex medical challenge that requires an exhaustive evaluation. Although prion diseases, in particular Creutzfeldt-Jakob disease (CJD), are often suspected, many other nonprion diseases may present as RPD. Our aim was to review the causes of RPD in our center to better understand the underlying conditions. We reviewed clinical, neuroimaging, and cerebrospinal fluid data from patients with RPD admitted to our hospital from 1994 to 2009. Forty-nine patients (mean age at onset 72.4 y) with RPD were admitted to our center during the study period. The mean interval between the onset of symptoms and admission was 4.6 months. The final clinical diagnoses were as follows: nonprion neurodegenerative diseases (36.8%), CJD (30.6%), vascular dementia (8.2%), toxic-metabolic conditions (8.2%), and other disorders (16.2%). Among cases with informed death (n = 19), the average survival time was 8.6 ± 9.5 months. Survival was shorter among patients with prion disease (n = 10) than in those with other diagnoses (n = 9, P = 0.004). In conclusion, nonprion neurodegenerative diseases are the most common cause of RPD in our center. Our results suggest that although CJD is often suspected as a cause of RPD, its frequency depends on the referral differences across specialized centers.
authors: Department of Neurology, Memory Disorders Unit, Hospital de la Santa Creu i Sant Pau, Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Barcelona, Spain.
authors: Isabel
authors: I
authors: Sala
authors: Marta
authors: M
authors: Marquié
authors: María Belén
authors: MB
authors: Sánchez-Saudinós
authors: Raquel
authors: R
authors: Sánchez-Valle
authors: Daniel
authors: D
authors: Alcolea
authors: Beatriz
authors: B
authors: Gómez-Ansón
authors: Teresa
authors: T
authors: Gómez-Isla
authors: Rafael
authors: R
authors: Blesa
authors: Alberto
authors: A
authors: Lleó
mesh_terms: D000368:Aged; D007562:Creutzfeldt-Jakob Syndrome / Q000150:complications / Q000453:epidemiology; D003704:Dementia / Q000209:etiology* / Q000401:mortality* / Q000473:pathology*; D015140:Dementia, Vascular / Q000150:complications / Q000453:epidemiology; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D019636:Neurodegenerative Diseases / Q000150:complications / Q000453:epidemiology; D062606:Tertiary Care Centers