title: Years of severe and isolated amnesia can precede the development of dementia in early-onset Alzheimer's disease.
abstract: A young patient with a severe and isolated memory disorder, meeting the criteria for MCI, was followed for a period of 8 years. His overall functional level remained stable with a CDR-score at 0.5 until the last year when it dropped to 1.0. Neuropsychological testing showed severe memory deficits but otherwise normal cognitive functions. Only minimal progression was measured; however, the last testing showed impaired abstraction and executive functioning as well as discrete problems generating names of objects and people. Neuroimaging, with MRI and SPECT, was consistent with a progressive degenerative disorder, and cerebrospinal fluid levels of beta-amyloid 1-42, tau protein, and phosphorylated tau protein were characteristic of Alzheimer's disease (AD). We argue that this is a case of prodromal AD, which illustrates an extreme version of the normal course with respect to slow progression of the disease and severity of amnesia early in the course.
authors.affiliation: Memory Disorders Research Unit, Neuroscience Centre, Copenhagen University Hospital, Rigshospitalet, Denmark. jette.neu@rh.dk
authors.forename: Jette
authors.initials: J
authors.lastname: Stokholm
authors.forename: Oda
authors.initials: O
authors.lastname: Jakobsen
authors.forename: Justyna M
authors.initials: JM
authors.lastname: Czarna
authors.forename: Henrik V
authors.initials: HV
authors.lastname: Mortensen
authors.forename: Gunhild
authors.initials: G
authors.lastname: Waldemar
mesh_terms: D000544:Alzheimer Disease / Q000134:cerebrospinal fluid / Q000150:complications*; D000647:Amnesia / Q000134:cerebrospinal fluid / Q000150:complications*; D016229:Amyloid beta-Peptides / Q000134:cerebrospinal fluid; D003704:Dementia / Q000134:cerebrospinal fluid / Q000209:etiology*; D018450:Disease Progression; D006801:Humans; D007802:Language; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D008568:Memory / Q000502:physiology; D008875:Middle Aged; D009483:Neuropsychological Tests / Q000706:statistics & numerical data; D010446:Peptide Fragments / Q000134:cerebrospinal fluid; D011340:Problem Solving / Q000502:physiology; D011597:Psychomotor Performance / Q000502:physiology; D015899:Tomography, Emission-Computed, Single-Photon; D016875:tau Proteins / Q000134:cerebrospinal fluid
chemical_list: D016229:Amyloid beta-Peptides; D010446:Peptide Fragments; C075222:amyloid beta-protein (1-42); D016875:tau Proteins