title: Rapidly progressive young-onset dementias: neuropsychiatric aspects.
abstract: Rapidly progressive dementia (RPD) is roughly defined as neurocognitive decline resulting in dementia or death within 2 years. Although RPDs affect all age groups, many occur in patients with young-onset dementia. Although prion disease (eg, Creutzfeldt-Jakob disease) is often thought to be the prototypic rapidly progressive young-onset dementia, the differential diagnosis is broad and some etiologies may be treatable. Hence, an appropriate workup to determine the etiology of RPD is crucial to planning the appropriate management. This article reviews the differential diagnosis, diagnostic workup, and management considerations for this unique patient population.
authors.affiliation: Department of Neurology, Case Western Reserve University School of Medicine & University Hospitals, 3619 Park East Drive, Suite 211, Beachwood, OH 44122, USA; Department of Psychiatry, Case Western Reserve University School of Medicine & University Hospitals, 3619 Park East Drive, Suite 211, Beachwood, OH 44122, USA.
authors.forename: Rajeet
authors.initials: R
authors.lastname: Shrestha
authors.affiliation: Department of Neurology, Case Western Reserve University School of Medicine & University Hospitals, 3619 Park East Drive, Suite 211, Beachwood, OH 44122, USA.
authors.forename: Timothy
authors.initials: T
authors.lastname: Wuerz
authors.affiliation: Department of Neurology, Case Western Reserve University School of Medicine & University Hospitals, 3619 Park East Drive, Suite 211, Beachwood, OH 44122, USA; Department of Psychiatry, Case Western Reserve University School of Medicine & University Hospitals, 3619 Park East Drive, Suite 211, Beachwood, OH 44122, USA; Department of Pathology, Case Western Reserve University School of Medicine & University Hospitals, 3619 Park East Drive, Suite 211, Beachwood, OH 44122, USA. Electronic address: bsa35@case.edu.
authors.forename: Brian S
authors.initials: BS
authors.lastname: Appleby
mesh_terms: D017668:Age of Onset; D007562:Creutzfeldt-Jakob Syndrome / Q000150:complications / Q000523:psychology; D003704:Dementia / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000523:psychology / Q000628:therapy; D003937:Diagnosis, Differential; D019468:Disease Management; D018450:Disease Progression; D006801:Humans; D008875:Middle Aged; D059906:Neuroimaging / Q000379:methods*; D011569:Psychiatric Status Rating Scales
keywords: Creutzfeldt–Jakob disease; Early onset dementia; Neuropsychiatric symptoms; Neuropsychiatry; Presenile dementia; Prion disease; Rapidly progressive dementia; Young onset dementia