Frontotemporal dementia (FTD) patients frequently present with psychosis, which complicates diagnosis and management. In this study, we aim to examine the relationship between psychosis and the most common genetic mutations predisposing to FTD, and in the different pathological subtypes of FTD.

We conducted a systematic review, searching the literature up to December 2022, and reviewed 50 articles that met our inclusion criteria. From the reviewed articles, we extracted and summarized data regarding the frequency of psychosis and patient characteristics in each major genetic and pathological subtype of FTD.

Among FTD patients with confirmed genetic mutations or pathological diagnosss, the frequency of psychosis was 24.2%. Among the genetic mutation carriers, C9orf72 mutation carriers had the highest frequency of psychosis (31.4%), whereas GRN (15.0%) and MAPT (9.2%) mutation carriers had lower frequencies of psychosis. MAPT mutation carriers notably developed psychosis at a younger age compared to other genetic groups. The most common psychotic symptoms were delusions among C9orf72 carriers and visual hallucinations among GRN mutation carriers. Among the pathological subtypes, 30% of patients with FUS pathology, 25.3% of patients with TDP-43 pathology, and 16.4% of patients with tau pathology developed psychosis. In the TDP-43 group, subtype B pathology was the most common subtype reported in association with psychosis.

Our systematic review suggests a high frequency of psychosis in specific subgroups of FTD patients. Further studies are required to understand the structural and biological underpinnings of psychosis in FTD.

Carer burden is common in younger-onset dementia (YOD), often due to the difficulty of navigating services often designed for older people with dementia. Compared to Alzheimer’s disease (AD), the burden is reported to be higher in behavioral variant frontotemporal dementia (bvFTD). However, there is little literature comparing carer burden specifically in YOD. This study hypothesized that carer burden in bvFTD would be higher than in AD.

Retrospective cross-sectional study.

Tertiary neuropsychiatry service in Victoria, Australia.

Patient-carer dyads with YOD.

We collected patient data, including behaviors using the Cambridge Behavioral Inventory-Revised (CBI-R). Carer burden was rated using the Zarit Burden Inventory-short version (ZBI-12). Descriptive statistics and Mann-Whitney U tests were used to analyze the data.

Carers reported high burden (ZBI-12 mean score = 17.2, SD = 10.5), with no significant difference in burden between younger-onset AD and bvFTD. CBI-R stereotypic and motor behaviors, CBI-R everyday skills, and total NUCOG scores differed between the two groups. There was no significant difference in the rest of the CBI-R subcategories, including the behavior-related domains.

Carers of YOD face high burden and are managing significant challenging behaviors. We found no difference in carer burden between younger-onset AD and bvFTD. This could be due to similarities in the two subtypes in terms of abnormal behavior, motivation, and self-care as measured on CBI-R, contrary to previous literature. Clinicians should screen for carer burden and associated factors including behavioral symptoms in YOD syndromes, as they may contribute to carer burden regardless of the type.

This study examined the association between loneliness and risk of incident all-cause dementia and whether the association extends to specific causes of dementia.

Longitudinal.

Community.

Participants were from the UK Biobank (N = 492,322).

None.

Loneliness was measured with a standard item. The diagnosis of dementia was derived from health and death records, which included all-cause dementia and the specific diagnoses of Alzheimer’s disease (AD), vascular dementia (VD), and frontotemporal dementia (FTD), over 15 years of follow-up.

Feeling lonely was associated with a nearly 60% increased risk of all-cause dementia (HR = 1.59, 95% CI = 1.51–1.65; n = 7,475 incident all-cause). In cause-specific analyses, loneliness was a stronger predictor of VD (HR = 1.82, 95% CI = 1.62–2.03; n = 1,691 incident VD) than AD (HR = 1.40, 95% CI = 1.28–1.53; n = 3135 incident AD) and was, surprisingly, a strong predictor of FTD (HR = 1.64, 95% CI = 1.22–2.20; n = 252 incident FTD). The associations were robust to sensitivity analyses and were attenuated but remained significant accounting for clinical (e.g. diabetes) and behavioral (e.g. physical activity) risk factors, depression, social isolation, and genetic risk. The association between loneliness and all-cause and AD risk was moderated by APOE ϵ4 risk status such that the increased risk was apparent in both groups but stronger among non-carriers than carriers of the risk allele.

Loneliness is associated with increased risk of multiple types of dementia.

Frontotemporal dementia(FTD) is the prevalent type of primary progressive dementia. Psychiatric symptoms can be seen in FTD. So it can imitate psychiatric disorders and be misdiagnosed. However, few studies have investigated the underlying cause of misdiagnosis.

The primary aim of this study was to identify the prior psychiatric diagnoses of patients before receiving a definitive diagnosis of FTD and the main reasons to cause diagnostic delay.

We screened through the records of patients who were admitted to our psychiatry outpatient or inpatient clinic from January 1st, 2018 to June 30th, 2021. The patients with FTD were included in our study.

Our sample consisted of 13 patients with FTD(mean age= 54.77 ± 12.22, 7 females). Psychiatric misdiagnoses were depression(n=6), psychosis(n=5), bipolar affective disorder (n=5), conversion disorder(n=4), and malingering(n=1). As we looked at the first symptoms of the patients, it was revealed that 9 of 12 patients presented with depressive symptoms or at least experienced a short depressive period at the beginning of their behavioral changes. Interestingly, 8 of 12 patients had given a history of stressful life events just before their complaints emerged, which was thought the main misdirector for physicians. The average delays in diagnosis were 14.58(±16.93) months in the psychiatry clinic, 5.66(±11.02) months in the neurology clinic in our hospital.

Our study suggests that the depressive episode preceding behavioral changes may be the prodromal stage for fully developed FTD. Moreover, the depressive episode and the history of stressful life events appear to mislead clinicians in diagnosing FTD.

No significant relationships.

Frontotemporal dementia (FTD) is common in presenile population. The overlapping symptoms with other psychiatric disorders can lead to wrong/late diagnosis which cause delays/difficulties regarding case-management. Especially, long-standing and/or late-onset depression can descriptively envelop bvFTD (behavioral-variant) and leads to unnecessary treatments and increased distress. It’s important to implement a descriptive diagnostic algorithm which will help clinicians to distinguish the phenomenology of these disorders.

This presentation aims to call attention of the clinicians/researchers to an elaborated effort concerning differential diagnosis of two common disorders with overlapping features through a case-study of a 59-year-old male patient.

One case from an inpatient unit of a psychiatric clinic in Lower Saxony, Germany will be reported.

Case: The patient was referred to our acute-psychiatric-ward from the day-clinic-unit because of treatment-resistant, severe and long-lasting depressive symptoms. He was depressed, desperate, hopeless, listless and had suicidal thoughts. During the first days of treatment, symptoms like apathy, bad hygiene, weird eating-behavior, urinary incontinence, lack of empathy, language disorders and other behavioral symptoms were evident. Brain-MRI yielded frontotemporal lobar atrophy. Trail-Making-Test and Frontal-Assessment-Battery showed pronounced impairment of executive functions. Mini mental state examination and DemTect yielded light to moderate memory dysfunction. Diagnostic Criteria for Probable bvFTD (International-Consensus-Criteria) were fulfilled.

The diagnosis of bvFTD enabled a rapid assignment of a legal representative and relieved the long-lasting discomfort of the patient and his family that was caused by multiple unsuccessful treatment trials against depression. The differential diagnostic frame between bvFTD and depression will be discussed in view of the current literature.

No significant relationships.

Obsessive-compulsive symptoms (OCS) have been described in many neurological disorders, including dementia. A meta-analysis by the authors (2021) reported a prevalence of OCS in dementia of approx. 35.8%, and a higher percentage in frontotemporal dementia (FTD) (46.7%). The literature also points that obsessive-compulsive disorder with late-life onset is rare, but those cases are frequently associated with neurologic injury, and some authors suggest a role of cognitive disfunction.

Our main goal was to describe the neurobiologic and cognitive underpinnings of OCS in patients with dementia.

MEDLINE, CENTRAL and PsycNet databases were searched for articles about obsessive-compulsive symptoms in dementia. Search terms included “obsessive”, “compulsive”, “OCD”, “cognitive decline”, “cognitive dysfunction” and “dementia”. Titles, abstracts and full texts were screened independently by 2 reviewers.

Correlations between dysfunction / lesions in various circuits in the context of dementia and OCS were found, such as (1) frontal regions (specially the orbitofrontal cortex) and anterior cingulate cortex (2) fronto-striatal-thalamic circuits (3) temporal structures; (4) cerebellar structures; (5) serotoninergic, dopaminergic, and cholinergic neurotransmission. A high proportion of studies concerned FTD. Regarding cognitive mechanisms, there is a focus on the importance subjective concerns about cognitive functioning, which could exacerbate obsessional beliefs and maladaptive responses to intrusions.

The main brain circuits implicated in dementia, specially FTD, and OCS are those involving frontal regions and the fronto-striatal-thalamic circuits, with areas such as the temporal and cerebellar structures algo being studied. The correlation between dysfunctional circuits in dementia and OCS could give us new hints about OCD and its treatment.

No significant relationships.

54-year-old female patient who came to hospital due to psychopathological decompensation of her Obsessive-Compulsive Disorder (OCD), after 35 years under follow-up. Parkinson´s disease. Psychopharmacological treatment: sertraline 100 mg (1-0-0); lorazepam 2 mg (1-1-1); Levodopa/carbidopa 100/25 mg (1-1-1). Distressed at first examination. She described increase in rituals, important intake restriction, weight impact and difficulties in home management with functional repercussions. Psychopathological exploration: conscious, oriented, and approachable. Circumstantial speech with no obsessive ideas. Increased frequency of repetitive behaviours led to a functional deterioration, becoming dependent for activities of daily living. Elevated anxiety. No major mood disorder. No psychotic symptoms. Bradykinesia. Hypophagia without anorexia. Admission is carried out. Good evolution: improvement in motor symptoms and intake restoration. No changes in repetitive behaviours.

To discuss the differential diagnosis between OCD and Frontotemporal Dementia.

Repetitive behaviours were initially understood as rituals typical of OCD. However, the absence of both a fixed pattern of behaviour and a structured obsessive ideation, made us consider the possibility of frontal perseveration behaviours. For this reason, a neuropsychological evaluation and a functional neuroimaging test were performed: Test Mo-CA: 9/30 with striking failures in executive functions. SPECT: mild uptake defect in the left frontotemporal region.

Finally, in view of the impairment in executive functions and the frontal defects in neuroimaging, we change the initial diagnosis of OCD towards a Neurocognitive Disorder of probable frontotemporal origin.

The presented case evidenced the importance of differentiating obsessive compulsions from frontal perseverance to guide the differential diagnosis, given the implications for therapeutic management and prognosis.

No significant relationships.

Frontotemporal Demential (FTD) is a neurodegenerative disorder evolving the frontal or temporal brain lobes. They have been described six variants. Behaviour variant (BvFTD) is the most common, and is characterized by changes in social behaviour and conduct, with loss of social awareness and poor impulse control. Hebephrenic schizophrenia (HSz), or disorganized schizophrenia, was recognized as a schizophrenia subtype, characterized by desorganized behaviour and a cognitive deteriorization. Subtypes of schizophrenia are no longer recognized as separate conditions neither in the Diagnostic and Statistical Manual of Mental Disorders, nor in the new International Statistical Classification of Diseases.

To review the literature about the concepts of hebephrenic schizophrenia and their similarities with the concept of frontotemporal dementia

Narrative review of the literature on PubMed/MEDLINE, using the keywords “hebephrenic szchizophrenia” AND “frontotemporal dementia”. Only articles in English were included.

Some authors described dificulty in establish a diferential diagnosis between HSz and BvFTD. HSz has an earlier onset. However, BvFTD is an early age dementia. The fenomenology of both diseases is similar, and schizophrenia was historical conceptualized as praecox dementia. Frontotemporal abnormalities are common neuroimagiological findings in schizophrenia. Clinically, FTD shows a profound alteration in personality and social conduct, emotional blunting and loss of insight. Memory, intellectual functions, executive and attentional abilities may be disturbed in both.

A diferential diagnosis between HSz and BvFTD is dificult to establish (clinically and imagiologically). The response to treatment is weak in both. It should be investigated the possibility they could be the same syndrome, onseting in diferent ages.

No significant relationships.