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Womack KB, Diaz-Arrastia R, Aizenstein HJ, et al. Temporoparietal Hypometabolism in Frontotemporal Lobar Degeneration and Associated Imaging Diagnostic Errors. Arch Neurol. 2011;68(3):329–337. doi:https://doi.org/10.1001/archneurol.2010.295
Frontotemporal lobar degeneration (FTLD) is the third most common degenerative dementia, behind Alzheimer disease (AD) and dementia with Lewy bodies.1 It is a heterogeneous disorder with at least 3 recognized clinical presentations,2 multiple histopathologic subtypes,3,4 and familial cases associated with mutations in 4 different genes5-9 with an additional genetic linkage on chromosome 9p.10-12
Despite the existence of consensus clinical diagnostic criteria, patients with FTLD are commonly misdiagnosed as having AD or a psychiatric illness.2,13-15 These mistakes are understandable given the insidious, progressive nature of both FTLD and AD and their shared symptoms.16 Both illnesses may have prominent behavioral changes, which can overlap symptoms typically seen in psychiatric disorders.17-19 While amnesia as the initial symptom of a progressive dementing disease strongly favors a diagnosis of AD, it also occurs in some patients with FTLD.20 Frontotemporal lobar degeneration may present with language deficits, but prominent language deficits also occur in AD.2,21-24 The difficulty in obtaining a detailed and reliable clinical history in some situations is a further challenge to accurate diagnosis and highlights the value of validated diagnostic biomarkers.
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