Years of severe and isolated amnesia can precede the development of dementia in early-onset Alzheimer's disease.
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.
Stokholm
Jette
J
Memory Disorders Research Unit, Neuroscience Centre, Copenhagen University Hospital, Rigshospitalet, Denmark. jette.neu@rh.dk
Jakobsen
Oda
O
Czarna
Justyna M
JM
Mortensen
Henrik V
HV
Waldemar
Gunhild
G
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
D016229:Amyloid beta-Peptides; D010446:Peptide Fragments; C075222:amyloid beta-protein (1-42); D016875:tau Proteins