When a Little Knowledge Can Be Dangerous: False-Positive Diagnosis of Behavioral Variant Frontotemporal Dementia among Community Clinicians

Abstract

Background: Accurate diagnosis of behavioral variant frontotemporal dementia (bvFTD) is important as patients' behavioral symptoms have profound implications for their families and communities. Since the diagnosis of bvFTD derives from behavioral features, accurate identification of patients can be difficult for non-specialists. Concrete rates of diagnostic accuracy among non-specialists are unavailable. Methods: To examine the accuracy of community clinicians' diagnoses of bvFTD and to identify patient characteristics leading to misdiagnosis, we reviewed the charts and referral letters of 3,578 patients who were seen at our specialized center. Referral diagnosis and reasons, manifesting symptoms, demographic data, Mini-Mental State Examination score, Clinical Dementia Rating score and Neuropsychiatric Inventory score were extracted. Results: 60% of patients assigned a single diagnosis of bvFTD by community clinicians did not have bvFTD according to specialists. Compared to specialist-confirmed bvFTD patients, false bvFTD patients were more likely to be depressed and to be non-Caucasian, showed less euphoria, apathy, disinhibition and abnormal eating behaviors, had milder disease severity and better overall cognition. bvFTD was mentioned by referring clinicians in 86% of specialist-confirmed bvFTD cases, but missed cases were called Alzheimer's, Parkinson's or Huntington's disease, or progressive aphasia. Conclusion: These results revealed a widespread lack of familiarity with core diagnostic symptoms among non-specialists and suggest that community clinicians require specialized diagnostic support before providing a definitive diagnosis of bvFTD.

Impaired coherence for semantic but not episodic autobiographical memory in semantic variant primary progressive aphasia

“…These findings are consistent with the link between language and autobiographical memory as demonstrated in studies of language development (e.g. Fivush and Nelson, 2004;Peterson & Rideout, 1998;Simcock & Hayne, 2002), amnesia due to MTL damage (Duff, Hengst, Tranel, & Cohen, 2009;MacKay, Stewart, & Burke, 1998;Kurczek, Brown-Schmidt & Duff, 2013), AD (Appell, Kertesz, & Fisman, 1982;Martin & Fedio, 1983;Rochon, Waters & Caplan, 1994;Croot, Hodges, Xuereb, & Patterson, 2000;Henry, Crawford, & Phillips, 2004;Taler & Phillips, 2016;Shinagawa, Catindig, Block, Miller, & Rankin, 2016), and MCI (Duong, Whitehead, Hanratty, & Chertkow, 2006;Murphy, Rich, & Troyer, 2006;Fleming, 2014;Drummond et al, 2015).…”

Impaired coherence for semantic but not episodic autobiographical memory in semantic variant primary progressive aphasia

“…These findings are consistent with the link between language and autobiographical memory as demonstrated in studies of language development (e.g. Fivush and Nelson, 2004;Peterson & Rideout, 1998;Simcock & Hayne, 2002), amnesia due to MTL damage (Duff, Hengst, Tranel, & Cohen, 2009;MacKay, Stewart, & Burke, 1998;Kurczek, Brown-Schmidt & Duff, 2013), AD (Appell, Kertesz, & Fisman, 1982;Martin & Fedio, 1983;Rochon, Waters & Caplan, 1994;Croot, Hodges, Xuereb, & Patterson, 2000;Henry, Crawford, & Phillips, 2004;Taler & Phillips, 2016;Shinagawa, Catindig, Block, Miller, & Rankin, 2016), and MCI (Duong, Whitehead, Hanratty, & Chertkow, 2006;Murphy, Rich, & Troyer, 2006;Fleming, 2014;Drummond et al, 2015).…”

Apraxia profiles—A single cognitive marker to discriminate all variants of frontotemporal lobar degeneration and Alzheimer's disease

“…Nevertheless, neither unambiguous operationalizations of diagnostic core features (e.g., “loss of empathy” for bvFTD; “semantic memory dysfunction” for svPPA) nor straightforward and brief clinical tests with sufficient differential value to discriminate between these dementia subtypes simultaneously are available. The correct application of the clinical criteria and a clinical differentiation of the FTLD variants from each other and from Alzheimer's disease (AD) thus remains challenging, particularly in nonexpert settings and for patients in early disease stages [12–14].…”