Early-onset dementia in adults
- Jared R Brosch, MD, MSc
Jared R Brosch, MD, MSc
- Assistant Professor of Neurology
- Indiana University
- Martin R Farlow, MD
Martin R Farlow, MD
- Professor of Neurology
- Indiana University School of Medicine
Diagnosis and treatment of progressive cognitive impairment in the younger adult requires a different approach than that of the older adult. In particular, the differential diagnosis is much broader and often requires a more extensive evaluation that includes consideration of both common and rare disorders. Nevertheless, the most common causes of early-onset dementia are the same in younger and older adults: Alzheimer disease, vascular dementia, and frontotemporal dementia.
The epidemiology, etiology, evaluation, and diagnosis of early-onset dementia will be reviewed here. A more general approach to adults with cognitive impairment or dementia and disease-specific diagnosis and management are presented elsewhere. (See "Evaluation of cognitive impairment and dementia" and "Treatment of dementia".)
Dementia — Several definitions for dementia exist, but a commonly used framework is one provided by the Diagnostic and Statistical Manual (DSM). According the DSM-5, dementia is defined as significant acquired cognitive impairment in one or more cognitive domains (eg, learning and memory, language, executive function, complex attention, perceptual-motor function, social cognition) that represents a significant decline from previous baseline and interferes with independence in daily activities (table 1) . (See "Evaluation of cognitive impairment and dementia", section on 'Definition of dementia'.)
While this definition recognizes multiple different cognitive domains, a decline in memory is one of the earliest and most prominent features of Alzheimer disease dementia and most other forms of dementia. A prominent exception is frontotemporal dementia, in which memory may be relatively preserved in relation to other cognitive domains such as language function or social cognition, at least initially. For most forms of dementia, however, memory and language dysfunction are generally always present.
Early-onset dementia — Although terminology varies, we consider early-onset dementia (EOD) to refer to cases of dementia occurring in adults ranging from 18 to 65 years of age. Some authors further subdivide EOD, using young-onset dementia to refer to cases with an age of onset between 18 and 45 years. This distinction is somewhat arbitrary, however, and most of the causative diseases do not follow such strict age cut-offs. A common approach to differential diagnosis, including consideration of rare diseases, should be followed regardless of the age of the younger adult with new cognitive change.
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- Early-onset dementia
- CAUSES OF EARLY-ONSET DEMENTIA
- Neurodegenerative dementias
- Vascular diseases
- Infectious diseases
- Inflammatory and autoimmune diseases
- Neurometabolic disorders
- DIFFERENTIAL DIAGNOSIS
- INITIAL EVALUATION
- Physical and neurologic examination
- Cognitive testing
- Routine laboratories
- Structural neuroimaging
- ADDITIONAL TESTING
- General approach
- Lumbar puncture and other laboratories
- Advanced neuroimaging
- Brain and other tissue biopsies
- Genetic testing
- SUMMARY AND RECOMMENDATIONS