The pursuit for markers of disease progression in behavioral variant frontotemporal dementia: a scoping review to optimize outcome measures for clinical trials.

Behavioral variant frontotemporal dementia (bvFTD) is a neurodegenerative disorder characterized by diverse and prominent changes in behavior and personality. One of the greatest challenges in bvFTD is to capture, measure and predict its disease progression, due to clinical, pathological and genetic heterogeneity. Availability of reliable outcome measures is pivotal for future clinical trials and disease monitoring.

[Sustainability in Flemish and Dutch mental health care].

Background: The Flemish and Dutch (mental) health sectors cause greenhouse gas emissions and therefore will have to make an effort to reduce their climate impact.

Aim: To assess whether differences can be found in the climate policies of Flemish and Dutch mental health institutions.

Method: Descriptive research based on a sustainability questionnaire, in which concrete actions, objectives and ambitions in the field of sustainability were questioned at Flemish and Dutch mental health institutions.

Diagnostic Instability Over Time in the Late-Onset Frontal Lobe Syndrome: When Can We Say it's FTD?

Objectives: Distinguishing sporadic behavioral variant of frontotemporal dementia (bvFTD) from late-onset primary psychiatric disorders (PPD) remains challenging with the lack of robust biomarkers. An early bvFTD misdiagnosis in PPD cases and vice-versa is common. Little is known about diagnostic (in)stability over longer period of time.

[Automated speech analysis can distinguish frontotemporal dementia from depression: a pilot study].

Background: Differentiating the behavioural variant of frontotemporal dementia from a depression is challenging. Recent development of automated speech analyses might add to diagnostic.

Aim: To investigate the value of automated speech analyses in differentiating bvFTD from a depressive disorder.

Relationship Between Sporadic Behavioral Variant Frontotemporal Dementia and Primary Psychiatric Disorders: A Study in Families.

Because the behavioral variant of frontotemporal dementia (bvFTD) shows major clinical overlap with primary psychiatric disorders (PPD) that affect similar neuroanatomical circuits, a common genetic vulnerability between FTD and PPD was hypothesized. We studied whether PPD are more prevalent in families of patients with sporadic frontotemporal dementia compared with healthy controls (HC), subjects with Alzheimer's disease (AD), and individuals with bipolar disorder (BD). In this case-control study performed between January 2013 and February 2019, we investigated the first-degree family history concerning depression, psychosis (including schizophrenia), BD, and autism spectrum disorder for 73 bvFTD patients, 153 patients with BD, 108 patients with AD, and 101 HC with a semistructured questionnaire (QFTD-NL 1.

Theory of Mind and social functioning among neuropsychiatric disorders: A transdiagnostic study.

Social dysfunction is commonly present in neuropsychiatric disorders of schizophrenia (SZ) and Alzheimer's disease (AD). Theory of Mind (ToM) deficits have been linked to social dysfunction in disease-specific studies. Nevertheless, it remains unclear how ToM is related to social functioning across these disorders, and which factors contribute to this relationship.

[A 51-year-old man with primary progressive aphasia].

Primary progressive aphasia (PPA) is a form of dementia in which brain circuits responsible for language and speech show progressive impairments. Based on consensus criteria PPA is divided into 3 main variants: a nonfluent/agrammatic, a semantic and a logopenic variant. Each variant has specific clinical characteristics, including neuropsychiatric symptoms, and is associated with different neuropathological findings.

The natural history of primary progressive aphasia: beyond aphasia.

Introduction: Primary progressive aphasia (PPA) is divided into three prototypical subtypes that are all characterized by their single core symptom of aphasia. Although later in their course, other cognitive, behavioral, and motor domains may become involved, little is known about the progression profile of each subtype relative to the other subtypes.

Methods: In this longitudinal retrospective cohort study, based on the recent biomarker-supported diagnostic criteria, 24 subjects diagnosed with semantic variant (svPPA), 22 with non-fluent variant (nfvPPA), and 18 with logopenic variant (lvPPA) were collected and followed up for 1-6 years.

[Frontotemporal dementia diagnoses within the psychiatric practice: pitfalls and practical tools].

Background: The behavioural variant of frontotemporal dementia (bvFTD) strongly resembles primary psychiatric disorders. Furthermore, a bvFTD mimic may occur, without neurodegenerative aetiology.

Aim: To offer psychiatrist clinical tools for making or ruling out a bvFTD diagnosis.

The bvFTD phenocopy syndrome: a case study supported by repeated MRI, [F]FDG-PET and pathological assessment.

A clinical syndrome with neuropsychiatric features of bvFTD without neuroimaging abnormalities and a lack of decline is a phenocopy of bvFTD (phFTD). Growing evidence suggests that psychological, psychiatric and environmental factors underlie phFTD. We describe a patient diagnosed with bvFTD prior to the revision of the diagnostic guidelines of FTD.

The Frontotemporal Dementia versus Primary Psychiatric Disorder (FTD versus PPD) Checklist: A Bedside Clinical Tool to Identify Behavioral Variant FTD in Patients with Late-Onset Behavioral Changes.

Background: Differentiating early behavioral variant frontotemporal dementia (bvFTD) and primary psychiatric disorders (PPD) is complex and biomarkers have limited accuracy, leading to inaccurate diagnoses.

Objectives: Develop a simple bedside clinical tool to differentiate bvFTD from PPD.

Methods: A checklist of clinical features differentiating bvFTD from PPD was developed based on literature and clinical experience.

Predicting progression in the late onset frontal lobe syndrome.

ABSTRACTA late onset frontal lobe syndrome (LOF) refers to a clinical syndrome with apathy, disinhibition, or stereotypical behavior arising in middle or late adulthood. Diagnostics are challenging, and both clinicians and patients need reliable predictors of progression to improve clinical guidance. In this longitudinal multicenter and genetically screened prospective study, 137 LOF patients with frontal behavior (FBI score≥11) and/or stereotypical behavior (SRI≥10) were included.

[Behavioural changes as a symptom: diagnosing behavioural variant frontotemporal dementia].

Behavioural variant frontotemporal dementia (bvFTD) is a neurodegenerative disease, the symptoms of which are changes in character, behavioural changes and socio-cognitive changes occurring predominantly at an age between 40 and 70 years. Frontotemporal atrophy is apparent on diagnostic imaging in 70% of patients with bvFTD; a diagnostic dilemma arises if this is not clearly obvious. Validated questionnaires for stereotypical behaviour, depressive symptoms and apathy, and neuropsychological examination can be very helpful in differentiating between bvFTD and psychiatric and other neurological conditions.

Disease trajectories in behavioural variant frontotemporal dementia, primary psychiatric and other neurodegenerative disorders presenting with behavioural change.

Behavioural variant frontotemporal dementia (bvFTD) is characterized by behavioural and social cognitive disturbances, while various psychiatric and neurodegenerative disorders may have similar clinical symptoms. Since neurodegenerative disorders are eventually progressive, whereas primary psychiatric disorders are not, this study aimed to investigate whether the change in clinical symptoms over time differed between groups and which biomarkers predicted rate of decline. Disease trajectories (median follow-up = 3 years) of frontal and stereotyped behaviour, general and frontal cognitive functioning, and social cognition were examined in bvFTD (n = 34), other neurodegenerative (n = 28) and primary psychiatric disorders (n = 43), all presenting with late-onset frontal lobe syndrome (45-75 years), using linear mixed models.

Hypometabolism of the posterior cingulate cortex is not restricted to Alzheimer's disease.

When differential diagnosis of dementia includes both Alzheimer's disease (AD) and the behavioural variant of frontotemporal dementia (bvFTD), distribution of cerebral glucose metabolism as measured using [F]‑2‑fluoro‑2‑deoxy‑d‑glucose positron emission tomography ([F]FDG-PET) may be helpful. One important clue for differentiation is the presence of hypometabolism in the posterior cingulate cortex (PCC), usually associated with AD. PCC hypometabolism however, could also be present in bvFTD.

Social Cognition Differentiates Behavioral Variant Frontotemporal Dementia From Other Neurodegenerative Diseases and Psychiatric Disorders.

Objective: Although deficits in social cognition are established as core features in behavioral variant frontotemporal dementia (bvFTD), it remains unresolved if impaired social cognition distinguishes bvFTD from the broad differential diagnoses in clinical practice. Our aim was to study whether social cognition discriminates bvFTD from other neurodegenerative diseases and psychiatric disorders in patients presenting with late-onset frontal symptoms. Next, we studied the association of social cognition with frontal symptoms and cognitive functioning.

The Diagnostic Challenge of the Late-Onset Frontal Lobe Syndrome: Clinical Predictors for Primary Psychiatric Disorders Versus Behavioral Variant Frontotemporal Dementia.

Objective: Primary psychiatric disorders (PsD) can present with symptomatology identical to that of behavioral variant frontotemporal dementia (bvFTD). To date, clinical guidelines do not provide a solution for this diagnostic challenge. The aim of our study was to prospectively determine which demographic, clinical, neuropsychological, neuroimaging, and cerebrospinal fluid biomarkers are important in distinguishing PsD from bvFTD.

The Pitfall of Behavioral Variant Frontotemporal Dementia Mimics Despite Multidisciplinary Application of the FTDC Criteria.

Background: The behavioral variant of frontotemporal dementia (bvFTD) has a broad differential diagnosis including other neurological and psychiatric disorders. Psychiatric misdiagnoses occur in up to 50% of bvFTD patients. Numbers on misdiagnosis of bvFTD in psychiatric disorders are lacking.

Psychosis in behavioral variant frontotemporal dementia.

Background: Dementia is generally characterized by cognitive impairment that can be accompanied by psychotic symptoms; for example, visual hallucinations are a core feature of dementia with Lewy bodies, and delusions are often seen in Alzheimer's disease. However, for behavioral variant of frontotemporal dementia (bvFTD), studies on the broad spectrum of psychotic symptoms are still lacking. The aim of this study was to systematically and prospectively subtype the wide spectrum of psychotic symptoms in probable and definite bvFTD.

Cerebrospinal fluid biomarker examination as a tool to discriminate behavioral variant frontotemporal dementia from primary psychiatric disorders.

Introduction: To prospectively determine the diagnostic value of cerebrospinal fluid (CSF) levels total-tau (tau) to amyloid-β ratio (Aβ) ratio (tau/Aβ ratio), phosphorylated-tau (p-tau) to tau ratio (p-tau/tau ratio), neurofilament light chain (NfL) and YKL40 in the late-onset frontal lobe syndrome, in particular for the differential diagnosis of behavioral variant frontotemporal dementia (bvFTD) versus primary psychiatric disorders (PSY).

Method: We included patients with a multidisciplinary 2-year-follow-up diagnosis of probable/definite bvFTD ( = 22) or PSY ( = 25), who underwent a detailed neuropsychiatric clinical examination, neuropsychological test battery, and magnetic resonance imaging at baseline. In all cases, CSF was collected through lumbar puncture at baseline.

Identifying Specific Clinical Symptoms of Behavioral Variant Frontotemporal Dementia Versus Differential Psychiatric Disorders in Patients Presenting With a Late-Onset Frontal Lobe Syndrome.

Objective: Early differentiation between psychiatric disorders and behavioral variant frontotemporal dementia (bvFTD) is of paramount importance in patients with the late-onset frontal lobe syndrome. As bvFTD in patients will deteriorate, psychiatric disorders are treatable. To date, misdiagnosis often occurs due to an overlap of symptoms and lack of specific biomarkers.

Late life bipolar disorder evolving into frontotemporal dementia mimic.

Objectives: Although bipolar disorder has been understood classically as a cyclic disease with full recovery between mood episodes, in the last decade, evidence has accumulated supporting progressive features. The clinical picture of advanced or end-stage bipolar disorder is heterogeneous with possible deficits in cognition and behavior, as illustrated by our case series.

Cases: From our neuropsychiatric outpatient clinic, we describe four cases with bipolar disorder gradually developing a clinical syndrome, including apathy, disinhibition, loss of empathy, stereotypical behavior, and compulsiveness, fulfilling the criteria for possible behavioral variant frontotemporal dementia.

Diagnostic Accuracy of MRI and Additional [18F]FDG-PET for Behavioral Variant Frontotemporal Dementia in Patients with Late Onset Behavioral Changes.

Background: Neuroimaging has a reasonable accuracy to differentiate behavioral variant frontotemporal dementia (bvFTD) from other neurodegenerative disorders, its value for the differentiation of bvFTD among subjects with acquired behavioral disturbances is unknown.

Objective: To determine the diagnostic accuracy of MRI, additional [18F]FDG-PET, and their combination for bvFTD among subjects with late onset behavioral changes.

Methods: Patients with late onset behavioral changes referred to a memory clinic or psychiatric services were included.