Analysis of a case series of behavioral variant frontotemporal dementia: Emphasis on diagnostic delay

Abstract

INTRODUCTIONDespite many advances in the characterization of the behavioral variant of frontotemporal dementia (bvFTD), the diagnosis of this syndrome poses a significant challenge, while delays or diagnostic mistakes may impact the proper clinical management of these patients.OBJECTIVETo describe the clinical profile at first evaluation of a sample of patients with bvFTD from a specialized outpatient neurological unit, with emphasis on the analysis of the delay between the onset of symptoms and diagnosis.METHODSWe selected 31 patients that fulfilled international consensus criteria for possible or probable bvFTD. Patients' medical admission sheets were thoroughly reviewed.RESULTSPatients' mean age was 67.9±8.2 years; 16 (51.6%) were men. Mean number of years of formal education was 7.7±4.0 years. Mean age at onset was 62.2±7.7 years, indicating a mean of 5.8 years of diagnostic delay. Thirteen patients (41.9%) presented with initial behavioral complaints only, eleven patients (35.5%) had mixed behavioral and memory complaints, five patients (16.1%) presented with memory complaints only, and two patient (6.4%) had behavioral and speech problems. Nine patients (29%) were admitted with alternative diagnoses. Mean and standard deviation scores for the mini-mental state examination, animal category fluency and memory test for drawings (five-minute delayed recall) were 19.3±6.3, 8.3±4.1and 3.7±2.7, respectively.CONCLUSIONMost patients from this sample were evaluated almost six years after the onset of symptoms and performed poorly on both cognitive screening tests and functional evaluation measures.

Neuropsychiatric or Behavioral and Psychological Symptoms of Dementia (BPSD): Focus on Prevalence and Natural History in Alzheimer's Disease and Frontotemporal Dementia

“…An early and correct identification and evaluation of BPSD are challenges and crucial parts in the clinical approach and management of these disorders. As at the disease onset, BPSD may dominate the clinical picture and may be interpreted as a primary psychiatric disorder, this can delay the proper evaluation and diagnosis of dementia (55). This is an important point as the earlier diagnosis is reached; more families and patients will be able to benefit from the use of appropriate clinical support and possible intervention.…”

Comparison of Prodromal Symptoms of Patients with Behavioral Variant Frontotemporal Dementia and Alzheimer Disease

“…Furthermore, previous studies, including our own, have shown that patients with bvFTD may also manifest with early neuropsychiatric symptoms, such as depression and psychosis [22,23]. Those patients who show only a few core clinical bvFTD features, or primarily present with psychiatric symptoms, are initially often misdiagnosed as having late-onset psychiatric disorders or other neurodegenerative diseases, most often AD [23][24][25][26]. Therefore, misrecognition of symptoms in the early stages of bvFTD frequently leads to diagnostic delay [27][28][29][30].…”

Gaps in clinical research in frontotemporal dementia: A call for diversity and disparities–focused research

“…[15][16][17][18] In other words, later diagnosis of FTD is common in many underrepresented populations. 15,17,19,20 These delays are due to factors at the level of the individual/family, and those at the level of the medical/health system (summarized in Table 1). In preparation for a 2021 externally led Patient-Focused Drug Development meeting on FTD, The Association for Frontotemporal Degeneration and the FTD Disorders Registry collaborated on the FTD Insights Survey, a community-based online survey of nearly 1800 diagnosed patients, care partners, and family members designed to better TA B L E 1 Individual and clinical barriers to FTD diagnosis in diverse populations.…”