Finally, we summarize current curative and palliative treatment options. We also discuss the diagnostic procedure, the likelihood that certain diseases are related to the speed of disease progression, and the utility of biomarkers. Instead, we describe the most important entities, underlying pathophysiological mechanisms, disease categories and factors that might contribute to rapid cognitive decline in primarily slowly progressive neurodegenerative diseases. We do not provide exhaustive lists of differential diagnoses because they can be readily found elsewhere 2, 9, 11, 12, 13, 14, 15, 16. In this Review, we discuss the definitions of RPD and shed light on its different aetiologies. Moreover, the potential infectivity of some diseases underlying RPD, such as HIV or prion diseases, must be considered as a matter of public health 10. However, the growing recognition of immune-mediated encephalitis 4, rapidly progressive subtypes of classic dementias such as Alzheimer disease (AD) 5 and various other mimics of prion diseases 6, 7 demands a thorough consideration of differential diagnoses, especially potentially reversible conditions 8, 9. As RPD is one of the typical clinical characteristics of Creutzfeldt–Jakob disease (CJD) and has long been part of the diagnostic criteria for this condition 3, prion diseases have been considered to be prototypical RPDs. This rather vague definition encompasses a large group of heterogeneous disorders, including immune-mediated, infectious and metabolic encephalopathies, as well as prion diseases and atypically rapid presentations of other neurodegenerative diseases. The term ‘rapidly progressive dementia’ (RPD) is commonly used to describe a cognitive disorder with fast progression leading to the clinical syndrome of dementia, as defined by the Diagnostic and Statistical Manual of Mental Disorders fourth edition 1, within a relatively brief time period, which is commonly considered to be less than either 1 or 2 years 2. Therapeutic options for many non-neurodegenerative causes of RPD are already available disease-modifying therapies for neurodegenerative RPDs are an important focus of current research and could become a treatment option in the near future. To identify treatable causes of RPD, the technical diagnostic work-up must include MRI and analyses of blood and cerebrospinal fluid, and further diagnostics might be indicated in unclear cases. RPD can occur in rapidly progressive neurodegenerative diseases, such as prion diseases, or in primarily slowly progressive diseases as a consequence of intrinsic factors or concomitant pathologies.īesides neurodegenerative diseases, inflammatory (immune-mediated and infectious), vascular, metabolic and neoplastic CNS diseases are important and frequent causes of RPD. In addition, we discuss treatment options such as high-dose steroid therapy in the context of therapy and diagnosis in clinically ambiguous cases.ĭefinitions of rapidly progressive dementia (RPD) vary according to the aetiological background and relate to the speed of cognitive decline, time from first symptom to dementia syndrome and/or overall survival. We describe an extended diagnostic procedure in the context of important differential diagnoses, discuss the utility of biomarkers and summarize potential treatment options. In this Review, we define the syndrome of RPD and shed light on its different aetiologies and on secondary factors that might contribute to rapid cognitive decline. Knowledge of RPD aetiologies, syndromes and diagnostic work-up protocols will help clinicians to establish an early, accurate diagnosis, thereby reducing morbidity and mortality, especially in immune-mediated and other potentially reversible dementias. Prion disease is considered to be the prototypical RPD, but over the past two decades, epidemiological reports and the identification of various encephalitis-mediating antibodies have led to a growing recognition of other encephalopathies as potential causes of rapid cognitive decline. Some of these conditions are treatable, and some must be diagnosed promptly because of their potential infectivity. Rapidly progressive dementias (RPDs) are a group of heterogeneous disorders that include immune-mediated, infectious and metabolic encephalopathies, as well as prion diseases and atypically rapid presentations of more common neurodegenerative diseases.
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