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.

We wish to thank the 1991 Pharmacy Foundation, The Health Insurance Foundation, The “IMK Almene Fond”, and The Danish Alzheimer Research Foundation for supporting the clinical research in the research unit. We are grateful to SPECT technician Gerda Thomsen for providing SPECT images for this report.

Notes

We wish to thank the 1991 Pharmacy Foundation, The Health Insurance Foundation, The “IMK Almene Fond”, and The Danish Alzheimer Research Foundation for supporting the clinical research in the research unit. We are grateful to SPECT technician Gerda Thomsen for providing SPECT images for this report.