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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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U. Hohl, P. Tiraboschi, L. A. Hansen, L. J. Thal, and J. Corey-Bloom Diagnostic Accuracy of Dementia With Lewy Bodies Arch Neurol, March 1, 2000; 57(3): 347 - 351. [Abstract] [Full Text] [PDF]
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M. N. Sabbagh, J. Corey-Bloom, P. Tiraboschi, R. Thomas, E. Masliah, and L. J. Thal Neurochemical Markers Do Not Correlate With Cognitive Decline in the Lewy Body Variant of Alzheimer Disease Arch Neurol, December 1, 1999; 56(12): 1458 - 1461. [Abstract] [Full Text] [PDF]
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J. Verghese, H. A. Crystal, D. W. Dickson, and R. B. Lipton Validity of clinical criteria for the diagnosis of dementia with Lewy bodies Neurology, December 1, 1999; 53(9): 1974 - 1974. [Abstract] [Full Text] [PDF]
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T. Gomez-Isla, W. B. Growdon, M. McNamara, K. Newell, E. Gomez-Tortosa, E. T. Hedley-Whyte, and B. T. Hyman Clinicopathologic correlates in temporal cortex in dementia with Lewy bodies Neurology, December 1, 1999; 53(9): 2003 - 2003. [Abstract] [Full Text] [PDF]
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N. Hirono, E. Mori, S. Tanimukai, H. Kazui, M. Hashimoto, T. Hanihara, and T. Imamura Distinctive Neurobehavioral Features Among Neurodegenerative Dementias J Neuropsychiatry Clin Neurosci, November 1, 1999; 11(4): 498 - 503. [Abstract] [Full Text] [PDF]
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E. Gomez-Tortosa, K. Newell, M. C. Irizarry, M. Albert, J. H. Growdon, and B. T. Hyman Clinical and quantitative pathologic correlates of dementia with Lewy bodies Neurology, October 1, 1999; 53(6): 1284 - 1284. [Abstract] [Full Text]
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O. L. Lopez, I. Litvan, K. E. Catt, R. Stowe, W. Klunk, D. I. Kaufer, J. T. Becker, and S. T. DeKosky Accuracy of four clinical diagnostic criteria for the diagnosis of neurodegenerative dementias Neurology, October 1, 1999; 53(6): 1292 - 1292. [Abstract] [Full Text] [PDF]
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C. F. Lippa, K. Ozawa, D. M. A. Mann, K. Ishii, T. W. Smith, S. Arawaka, and H. Mori Deposition of {beta}-Amyloid Subtypes 40 and 42 Differentiates Dementia With Lewy Bodies From Alzheimer Disease Arch Neurol, September 1, 1999; 56(9): 1111 - 1118. [Abstract] [Full Text] [PDF]
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W. P. Goldman, J. D. Baty, V. D. Buckles, S. Sahrmann, and J. C. Morris Motor dysfunction in mildly demented AD individuals without extrapyramidal signs Neurology, September 1, 1999; 53(5): 956 - 956. [Abstract] [Full Text] [PDF]
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K. Shiozaki, E. Iseki, H. Uchiyama, Y. Watanabe, T. Haga, K. Kameyama, T. Ikeda, T. Yamamoto, and K. Kosaka Alterations of muscarinic acetylcholine receptor subtypes in diffuse Lewy body disease: relation to Alzheimer's disease J. Neurol. Neurosurg. Psychiatry, August 1, 1999; 67(2): 209 - 213. [Abstract] [Full Text] [PDF]
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C. Ballard, C. Holmes, I. McKeith, D. Neill, J. O'Brien, N. Cairns, P. Lantos, E. Perry, P. Ince, and R. Perry Psychiatric Morbidity in Dementia With Lewy Bodies: A Prospective Clinical and Neuropathological Comparative Study With Alzheimer's Disease Am J Psychiatry, July 1, 1999; 156(7): 1039 - 1045. [Abstract] [Full Text]
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A. Heyman, G. G. Fillenbaum, M. Gearing, S. S. Mirra, K. A. Welsh-Bohmer, B. Peterson, and C. Pieper Comparison of Lewy body variant of Alzheimer's disease with pure Alzheimer's disease: Consortium to Establish a Registry for Alzheimer's Disease, Part XIX Neurology, June 1, 1999; 52(9): 1839 - 1839. [Abstract] [Full Text] [PDF]
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G. S. Zubenko, H. B. Hughes III, and J. S. Stiffler Neurobiological correlates of a putative risk allele for Alzheimer's disease on chromosome 12q Neurology, March 1, 1999; 52(4): 725 - 725. [Abstract] [Full Text]
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M. Gearing, M. Lynn, and S. S. Mirra Neurofibrillary Pathology in Alzheimer Disease With Lewy Bodies: Two Subgroups Arch Neurol, February 1, 1999; 56(2): 203 - 208. [Abstract] [Full Text] [PDF]
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T. Shimomura, E. Mori, H. Yamashita, T. Imamura, N. Hirono, M. Hashimoto, S. Tanimukai, H. Kazui, and T. Hanihara Cognitive Loss in Dementia With Lewy Bodies and Alzheimer Disease Arch Neurol, December 1, 1998; 55(12): 1547 - 1552. [Abstract] [Full Text] [PDF]
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S. Kuwabara, M. Asahina, M. Koga, M. Mori, N. Yuki, and T. Hattori Two patterns of clinical recovery in Guillain-Barre syndrome with IgG anti-GM1 antibody Neurology, December 1, 1998; 51(6): 1656 - 1660. [Abstract] [Full Text] [PDF]
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A. Pestronk Multifocal Motor Neuropathy: Diagnosis and Treatment Neurology, December 1, 1998; 51(6_Suppl_5): S22 - S24. [Abstract] [Full Text] [PDF]
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J. F. Howard Jr. Intravenous Immunoglobulin for the Treatment of Acquired Myasthenia Gravis Neurology, December 1, 1998; 51(6_Suppl_5): S30 - S36. [Abstract] [Full Text] [PDF]
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E. Gomez-Tortosa, K. Newell, M. Irizarry, and B. T. Hyman Clinical and neuropathological features of dementia with Lewy bodies American Journal of Alzheimer's Disease and Other Dementias, November 1, 1998; 13(6): 284 - 290. [Abstract] [PDF]
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J. Engel Jr. Etiology as a risk factor for medically refractory epilepsy: A case for early surgical intervention Neurology, November 1, 1998; 51(5): 1243 - 1244. [Full Text] [PDF]
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F. Semah, M.-C. Picot, C. Adam, D. Broglin, A. Arzimanoglou, B. Bazin, D. Cavalcanti, and M. Baulac Is the underlying cause of epilepsy a major prognostic factor for recurrence? Neurology, November 1, 1998; 51(5): 1256 - 1262. [Abstract] [Full Text] [PDF]
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L. D'Olhaberriague, S. R. Levine, L. Salowich-Palm, D. Tanne, K. L. Sawaya, T. K. Aurora, M. Perry, M. Day, T. Spencer, and L. Schultz Specificity, isotype, and titer distribution of anticardiolipin antibodies in CNS diseases Neurology, November 1, 1998; 51(5): 1376 - 1380. [Abstract] [Full Text] [PDF]
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R. P. Grewal, E. Tayag, K. P. Figueroa, L. Zu, A. Durazo, C. Nunez, and S. M. Pulst Clinical and genetic analysis of a distinct autosomal dominant spinocerebellar ataxia Neurology, November 1, 1998; 51(5): 1423 - 1426. [Abstract] [Full Text] [PDF]
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T. Ernst, L. Chang, I. Walot, and K. Huff Physiologic MRI of a tumefactive multiple sclerosis lesion Neurology, November 1, 1998; 51(5): 1486 - 1488. [Abstract] [Full Text] [PDF]
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J. R. Keane and J. Ahmadi Most diabetic third nerve palsies are peripheral Neurology, November 1, 1998; 51(5): 1510 - 1510. [Full Text] [PDF]
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D. I. Kaufer, K. E. Catt, O. L. Lopez, and S. T. DeKosky Dementia with Lewy bodies: Response of delirium-like features to donepezil Neurology, November 1, 1998; 51(5): 1512 - 1512. [Full Text] [PDF]
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 C. F. Lippa, H. Fujiwara, D. M.A. Mann, B. Giasson, M. Baba, M. L. Schmidt, L. E. Nee, B. O'Connell, D. A. Pollen, P. St. George-Hyslop, et al. Lewy Bodies Contain Altered {alpha}-Synuclein in Brains of Many Familial Alzheimer's Disease Patients with Mutations in Presenilin and Amyloid Precursor Protein Genes Am. J. Pathol., November 1, 1998; 153(5): 1365 - 1370. [Abstract] [Full Text] [PDF]
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L. J. Willmore Epilepsy emergencies: The first seizure and status epilepticus Neurology, November 1, 1998; 51(5_Suppl_4): S34 - S38. [Abstract] [Full Text] [PDF]
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