Abstract
Alzheimer’s disease typically presents with episodic, short-term memory impairment. There are three principle variants or atypical forms of Alzheimer’s disease: (1) posterior cortical atrophy, (2) logopenic aphasia, and (3) frontal Alzheimer’s disease. Each of these syndromes has a characteristic phenotype that can be identified clinically. Each of the phenotypes can be caused by other neurological disorders besides Alzheimer’s disease and differential diagnosis is an important part of the approach to neurological care. Mixed dementias are common, particularly the association of Alzheimer’s disease with cerebrovascular disease. Mixed dementia may also occur with Alzheimer’s disease in combination with Lewy body pathology and TDP-43 protein inclusions. Amyloid angiopathy is a vascular disorder with amyloid accumulation in the blood vessels and usually presenting with lobar hemorrhage. When there is prominent inflammation, patients may improve with treatment with steroids or anti-metabolites.
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References
Dubois B, Feldman HH, Jacova C, Dekosky ST, Barberger-Gateau P, Cummings J, et al. Research criteria for the diagnosis of Alzheimer's disease: revising the NINCDS-ADRDA criteria. Lancet Neurol. 2007;6(8):734–46.
Crutch SJ, Schott JM, Rabinovici GD, Murray M, Snowden JS, van der Flier WM et al. Consensus classification of posterior cortical atrophy. Alzheimers Dement. 2017. doi:10.1016/j.jalz.2017.01.014.
Gorno-Tempini ML, Hillis AE, Weintraub S, Kertesz A, Mendez M, Cappa SF, et al. Classification of primary progressive aphasia and its variants. Neurology. 2011;76(11):1006–14.
Johnson JK, Head E, Kim R, Starr A, Cotman CW. Clinical and pathological evidence for a frontal variant of Alzheimer disease. Arch Neurol. 1999;56(10):1233–9.
Tekin S, Mega MS, Masterman DM, Chow T, Garakian J, Vinters HV, et al. Orbitofrontal and anterior cingulate cortex neurofibrillary tangle burden is associated with agitation in Alzheimer disease. Ann Neurol. 2001;49(3):355–61.
Larner AJ. “Frontal variant Alzheimer’s disease”: a reappraisal. Clin Neurol Neurosurg. 2006;108(7):705–8.
Dubois B, Feldman HH, Jacova C, Hampel H, Molinuevo JL, Blennow K, et al. Advancing research diagnostic criteria for Alzheimer’s disease: the IWG-2 criteria. Lancet Neurol. 2014;13(6):614–29.
Roman GC, Tatemichi TK, Erkinjuntti T, Cummings JL, Masdeu JC, Garcia JH, et al. Vascular dementia: diagnostic criteria for research studies. Report of the NINDS-AIREN International Workshop. Neurology. 1993;43(2):250–60.
Lim A, Tsuang D, Kukull W, Nochlin D, Leverenz J, McCormick W, et al. Clinico-neuropathological correlation of Alzheimer’s disease in a community-based case series. J Am Geriatr Soc. 1999;47(5):564–9.
Sperling R, Salloway S, Brooks DJ, Tampieri D, Barakos J, Fox NC, et al. Amyloid-related imaging abnormalities in patients with Alzheimer’s disease treated with bapineuzumab: a retrospective analysis. Lancet Neurol. 2012;11(3):241–9.
Knudsen KA, Rosand J, Karluk D, Greenberg SM. Clinical diagnosis of cerebral amyloid angiopathy: validation of the Boston criteria. Neurology. 2001;56(4):537–9.
Auriel E, Charidimou A, Gurol ME, Ni J, Van Etten ES, Martinez-Ramirez S, et al. Validation of clinicoradiological criteria for the diagnosis of cerebral amyloid angiopathy-related inflammation. JAMA Neurol. 2016;73(2):197–202.
Kinnecom C, Lev MH, Wendell L, Smith EE, Rosand J, Frosch MP, et al. Course of cerebral amyloid angiopathy-related inflammation. Neurology. 2007;68(17):1411–6.
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Dr. Cummings has provided consultation to Abbvie, Acadia, Accera, Actinogen, ADAMAS, Alkahest, Alzheon, Anavex, Astellas, Astra Zeneca, Avanir, Axovant, Biogen-Idec, Biotie, Boehinger-Ingelheim, Chase, Eisai, Forum, GE Healthcare, Genentech, Grifols, Intracellular Therapies, IRIS, Ionis Pharmaceuticals, Lilly, Lundbeck, MedAvante, Merck, Neurotrope, Novartis, Nutricia, Otsuka, Pfizer, Probiodrug, QR Pharma, Resverlogix, Roche, Servier, Sunovion, Suven, Takeda, Toyoma, Transition Therapeutics, and United Neuroscience companies.
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Cummings, J. (2017). Atypical Alzheimer’s Disease, Mixed Dementia, and Amyloid Angiopathy. In: Tousi, B., Cummings, J. (eds) Neuro-Geriatrics. Springer, Cham. https://doi.org/10.1007/978-3-319-56484-5_5
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