CLINICAL-NEUROPATHOLOGICAL CORRELATIONS IN ALZHEIMERS-DISEASE AND RELATED DEMENTIAS

Objective: To compare neurologists' initial clinical diagnoses made ac cording to National Institute of Neurological and Communicative Disord ers and Stroke-Alzheimer's Disease and Related Disorders Association ( NINCDS-ADRDA) and Diagnostic and Statistical Manual of Mental Disorder s, Revised Third Edition guidelines with neuropathological diagnoses o f Alzheimer's disease (AD) and related dementias. Design: Consecutive autopsies in a prospective cohort study. Setting: Community-dwelling p atients with dementia referred to neurologists al an Alzheimer's Disea se Research Center and satellite clinics (n=151) and patients initiall y evaluated when institutionalized (n=19). Patients: Of 204 elderly pa tients who had an autopsy performed, 170 had received a complete demen tia evaluation according to NINCDS-ADRDA guidelines. Main Outcome Meas ures: Percentage agreement between neurologists' initial clinical diag noses and pathological findings. Results: Of 137 patients clinically d iagnosed as having probable or possible AD, 123 (90%) had AD neuropath ological findings; this included 29 with AD accompanied by Lewy bodies , and 14 with AD and one or more infarcts. Cases of vascular and mixed dementia (AD and infarct[s]) had lower rates of agreement with pathol ogical findings. Possible AD cases were more likely than probable AD c ases to show pathological features other than AD. Clinicians predicted the presence or absence of AD pathological findings significantly bet ter than chance. In patients with AD pathological lesions, older age o f onset and male Sender were significantly associated with shorter dur ation from disease onset to death. Conclusions: Clinicians accurately predicted AD pathological findings or their absence in most cases. Att ributing other degenerative dementias to AD, misdiagnosing patients wi th combined AD and Lewy bodies and misjudging the vascular contributio n to dementia were the major areas of inaccuracy. Formal criteria for dementia associated with non-AD lesions, Lewy bodies, and infarcts nee d to be developed and tested.