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Departments of Neurosciences (Drs. Hansen, Galasko, Masliah, Katzman, and Thal, Mr. DeTeresa and Mr. Alford), Psychiatry (Dr. Butters), and Community and Family Medicine (Dr. Klauber), and the Alzheimer's Disease Research Center (Drs. Salmon, Katzman, and Hofstetter, Ms. Pay and Ms. Rice), University of California, San Diego; the Neurology (Drs. Galasko and Thal) and Psychology (Dr. Butters) Services, Veterans Administration Medical Center, San Diego; and Department of Political Science (Dr. Hofstetter), San Diego State University, San Diego, CA.
Thirty-six clinically diagnosed and pathologically confirmed Alzheimer's disease (AD) patients included 13 with cortical and subcortical Lewy bodies (LBs). The patients with LBs appeared to constitute a distinct neuropathologic and clinical subset of AD, the Lewy body variant (LBV). The LBV group showed gross pallor of the substantia nigra, greater neuron loss in the locus ceruleus, substantia nigra, and substantia innominata, lower neocortical ChAT levels, and fewer midfrontal tangles than did the pure AD group, along with a high incidence of medial temporal lobe spongiform vacuolization. Analysis of neuropsychological tests from 9 LBV subjects and 9 AD patients matched for age and degree of dementia revealed greater deficits in attention, fluency, and visuospatial processing in the LBV group. Similar comparisons of neurologic examinations showed a significant increase in masked facies; in addition there was an increase in essential tremor, bradykinesia, mild neck rigidity, and slowing of rapid alternating movements in the LBV group. Extremity rigidity, flexed posture, resting tremor, or other classic parkinsonian features were not characteristic of the LBV patient. In some cases, it may be possible to diagnose LBV premortem on the basis of the clinical and neuropsychological features.
Address correspondence and reprint requests to Dr. Lawrence Hansen, Department of Neurosciences, M-024, School of Medicine, University of California, San Diego, La Jolla, CA 92093.
Supported by grants from the ADRC (AGO5131), the NIA (AGO8201), and the California state-funded SOCARE/ADDTC.
Received May 19, 1989. Accepted for publication in final form June 22, 1989.
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