Underdiagnosis of frontotemporal lobar degeneration in Brazil

Bahia, Valéria Santoro

doi:10.1590/s1980-57642008dn10400006

Cited by 12 publications

(12 citation statements)

References 34 publications

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“…FTD is considered a rare disorder indistinguishable from AD or psychiatric disorders in the early clinical stages, 13 and is frequently underdiagnosed even in specialist settings. 14 , 15 Accurate diagnosis of FTD is critical, as it has implications for heritability, prognosis, therapeutics and environmental management of patients.…”

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Accuracy of neuropsychological tests and the Neuropsychiatric Inventory in differential diagnosis between Frontotemporal dementia and Alzheimer's disease

Bahia

Viana

2009

Dement. neuropsychol.

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The differential diagnosis between frontotemporal dementia (FTD) and Alzheimer’s disease (AD) is sometimes difficult.ObjectivesTo verify the accuracy of neuropsychological tests and a behavioral disorders scale in the differential diagnosis between FTLD and AD.MethodsRetrospective data on 12 FTD patients and 12 probable AD patients were analyzed. The scores on neuropsychological tests (MMSE score, reverse digit span, delayed recall for drawings, semantic fluency of animals) and the Neuropsychiatric Inventory (NPI) in both groups were compared.ResultsBoth groups had similar performance on neuropsychological tests. All FTD patients and 50% of AD patients had neuropsychiatric abnormalities. The NPI score was 58.0±19.3for the FTD patients, and 3.6±4.7for the AD patients (p< 0.01). Using a NPI cut-off score of 13, the sensitivity and specificity were 100% in this sample. The four most common neuropsychiatric disturbances in FTD patients were: apathy, aberrant motor behavior, disinhibition and eating abnormalities. Apathy and dysphoria/depression were the most common behavioral symptoms among the AD patients.ConclusionsIn this study, NPI was found to be a useful tool for the differential diagnosis between FTD and AD. The neuropsychological tests commonly used in the medical office were unable to distinguish between the two groups.

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Accuracy of neuropsychological tests and the Neuropsychiatric Inventory in differential diagnosis between Frontotemporal dementia and Alzheimer's disease

Bahia

Viana

2009

Dement. neuropsychol.

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“…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].…”

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confidence: 85%

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

Korhonen

Katisko

Cajanus

et al. 2020

Dement Geriatr Cogn Disord

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Introduction: Behavioral variant frontotemporal dementia (bvFTD) is the most common clinical subtype of frontotemporal lobar degeneration. bvFTD is often characterized by changes in behavior and personality, frequently leading to psychiatric misdiagnoses. On the other hand, substantial clinical overlap with other neurodegenerative diseases, such as Alzheimer disease (AD), further complicates the diagnostics. Objective: Our aim was to identify the main differences in early symptoms of bvFTD and AD in the prodromal stages of the diseases. In addition, patients with bvFTD were analyzed separately according to whether they carry the C9orf72 repeat expansion or not. Methods: Patient records of bvFTD (n = 75) and AD (n = 83) patients were analyzed retrospectively for memory and neuropsychiatric symptoms, sleeping disorders, and somatic complaints before the setting of the accurate diagnosis. Results: A total of 84% of bvFTD patients (n = 63) and 98.8% of AD patients (n = 82) reported subjective memory disturbances in the prodromal phases of the disease. bvFTD patients presented significantly more often with sleeping disorders, headache, inexplicable collapses, transient loss of consciousness, somatization, delusions, and hallucinations, suicidality, changes in oral behaviors, and urinary problems. In addition, poor financial judgement was frequently detected in patients with prodromal bvFTD. Aberrant sensations in the nose and throat without any physical explanation, regarded as somatizations, emerged only in bvFTD patients with the C9orf72 repeat expansion. Conclusions: Subjective reporting of impaired episodic memory is a poor indicator in differentiating bvFTD from AD. Sleeping disturbances, delusions, hallucinations, and unexplained somatic complaints in a patient with cognitive disturbances should prompt the clinicians to consider bvFTD as a possible diagnostic option behind these symptoms. The spectrum of symptoms in the prodromal stages of bvFTD may be more diverse than the latest criteria suggest.

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“…To our knowledge, scant studies have discussed the issue of diagnostic delay in bvFTD. 11 - 13 Nonetheless, it was the Brazilian study 13 that reported the longest time delay between disease onset and proper diagnosis. However, all of these reports included PNFA and SD patients, in whom the salient language impairment might have facilitated early referral for specialized assessment.…”

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confidence: 99%

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

Guimarães

Vale

Pimentel

et al. 2013

Dement. neuropsychol.

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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.

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Underdiagnosis of frontotemporal lobar degeneration in Brazil

Cited by 12 publications

References 34 publications

Accuracy of neuropsychological tests and the Neuropsychiatric Inventory in differential diagnosis between Frontotemporal dementia and Alzheimer's disease

Accuracy of neuropsychological tests and the Neuropsychiatric Inventory in differential diagnosis between Frontotemporal dementia and Alzheimer's disease

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

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

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