ACCURACY OF CLINICAL-DIAGNOSIS OF ALZHEIMER-DISEASE AND CLINICAL-FEATURES OF PATIENTS WITH NON-ALZHEIMER DISEASE NEUROPATHOLOGY

Neuropathological examination confirmed the clinical diagnosis of poss ible or probable Alzheimer disease (AD) in 90 of the first 100 patient s who came to autopsy at the Johns Hopkins Alzheimer's Disease Researc h Center. In 10 cases, postmortem brain examination did not confirm AD but revealed variable patterns of neuronal loss in neocortex and limb ic structures without amyloid deposits. The most common pattern of deg eneration was relatively isolated hippocampal sclerosis CHS). Despite the finding that the 10 patients with non-AD neuropathology were ill f or less time and were less cognitively impaired at study entry than th ose patients with definite AD, they had shorter survival times and sho wed equal behavioral disturbance at study entry (on a standardized mea sure). The clinical case reports included here suggest early and progr essive prominent behavioral disturbance and other indexes of rapid ill ness progression in three of the four HS patients and two other non-AD patients. We conclude that the criteria of the National Institute of Neurological Disorders and Stroke/Alzheimer Disease and Related Disord ers Association for possible or probable AD are highly accurate and th at misdiagnosis is most likely to occur early in the course of illness and in patients with prominent behavioral disturbance or other atypic al features.