Psychotic symptoms in frontotemporal dementia: a diagnostic dilemma?

Waldö, María Landqvist; Gustafson, Lars; Passant, Ulla; Englund, Elisabet

doi:10.1017/s1041610214002580

Cited by 77 publications

(44 citation statements)

References 33 publications

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“…This may partly explain why the authors found a lower prevalence (22.7%) in the present cohort, which was characterized by a higher mean age of bvFTD patients (58.95 years). A recent retrospective study on delusions, hallucinations, and paranoid ideas in neuropathologically verified bvFTD patients also revealed a relatively high prevalence of these positive psychotic symptoms (32%): 20.6% of the patients had paranoid ideas, and 17.5% had hallucinations and delusions in equal measure 17. As discussed by the authors of this study, psychotic symptoms may be present for only a short period or occur later during disease course, thereby explaining the relatively high rates in their retrospective study.…”

Section: Discussionmentioning

confidence: 91%

“…Positive psychotic symptoms refer to an excess or distortion of normal functions (eg, paranoia or hearing voices), negative symptoms reflect a diminution or loss of normal functions (eg, reduced motivation or reduced emotion), and formal thought disorders implicit a disorganization of thought 12–14. Previous studies on psychotic symptoms in bvFTD were focused only on positive psychotic symptoms (eg, hallucinations, delusions, and paranoia) and found that these psychotic features were present in 10%–32% of bvFTD patients 3,15–17. Even though negative psychotic symptoms and formal thought disorders have led to frequent misdiagnosis of psychosis in bvFTD patients, comprehensive studies on the full spectrum of psychosis in bvFTD are lacking 5,8,18–20.…”

Section: Introductionmentioning

confidence: 99%

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Psychosis in behavioral variant frontotemporal dementia

Gossink¹,

Vijverberg²,

Krudop³

et al. 2017

NDT

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BackgroundDementia 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.MethodsIn this study, a commonly used and validated clinical scale that quantifies the broad spectrum of psychotic symptoms (Positive and Negative Symptom Scale) was used in patients with probable and definite bvFTD (n=22) and with a primary psychiatric disorder (n=35) in a late-onset frontal lobe cohort. Median symptom duration was 2.8 years, and the patients were prospectively followed for 2 years.ResultsIn total, 22.7% of bvFTD patients suffered from delusions, hallucinatory behavior, and suspiciousness, although the majority of the patients exhibited negative psychotic symptoms such as social and emotional withdrawal and blunted affect (95.5%) and formal thought disorders (81.8%). “Difficulty in abstract thinking” and “stereotypical thinking” (formal thought disorders) differentiated bvFTD from psychiatric disorders. The combined predictors difficulty in abstract thinking, stereotypical thinking, “anxiety”, “guilt feelings,” and “tension” explained 75.4% of variance in the diagnosis of bvFTD versus psychiatric diagnoses (P<0.001).ConclusionDelusions, hallucinatory behavior, and suspiciousness were present in one-fifth of bvFTD patients, whereas negative psychotic symptoms such as social and emotional withdrawal, blunted affect, and formal thought disorders were more frequently present. This suggests that negative psychotic symptoms and formal thought disorders have an important role in the psychiatric misdiagnosis in bvFTD; misdiagnosis in bvFTD might be reduced by systematically exploring the broad spectrum of psychiatric symptoms.

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Section: Discussionmentioning

confidence: 91%

Section: Introductionmentioning

confidence: 99%

Psychosis in behavioral variant frontotemporal dementia

Gossink¹,

Vijverberg²,

Krudop³

et al. 2017

NDT

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“…A recent study utilized pathological data to correlate regions of atrophy at post-mortem to psychotic symptoms reported at any time during life, and found an association with predominantly right-sided brain degeneration but failed to find any link with subcortical atrophy (Landqvist Waldö et al, 2015). Methodological differences may explain the divergent findings between this recent study and the current study.…”

Section: Discussionmentioning

confidence: 99%

The neural correlates and clinical characteristics of psychosis in the frontotemporal dementia continuum and the C9orf72 expansion

Devenney

Landín-Romero

Irish

et al. 2017

NeuroImage: Clinical

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ObjectiveThis present study aims to address the gap in the literature regarding the severity and underlying neural correlates of psychotic symptoms in frontotemporal dementia with and without the C9orf72 gene expansion.MethodsFifty-six patients with behavioural variant frontotemporal dementia (20 with concomitant amyotrophic lateral sclerosis) and 23 healthy controls underwent neuropsychological assessments, detailed clinical interview for assessment of psychosis symptoms, brain MRI and genetic testing. Carers underwent a clinical interview based upon the neuropsychiatric inventory. Patients were assessed at ForeFront, the Frontotemporal Dementia Research Group at Neuroscience Research Australia or at the Brain and Mind Centre, between January 2008 and December 2013.An index of psychosis was calculated, taking into account the degree and severity of psychosis in each case. Voxel-based morphometry analyses were used to explore relationships between the psychosis index and grey matter changes.ResultsThirty-four percent of frontotemporal dementia patients showed psychotic features. C9orf72 expansion cases were more likely to exhibit psychotic symptoms than non-carriers (64% vs. 26%; p = 0.006), which were also more severe (psychotic index 23.1 vs. 8.1; p = 0.002). Delusions comprised persecutory, somatic, jealous and grandiose types and were present in 57% of C9orf72 carriers and 19% of non-carriers (p = 0.006). Auditory, visual or tactile hallucinations were present in 36% of C9orf72 carriers and 17% of non-carriers (p = 0.13). Increased psychotic symptoms in C9orf72 expansion carriers correlated with atrophy in a distributed cortical and subcortical network that included discrete regions of the frontal, temporal and occipital cortices, as well as the thalamus, striatum and cerebellum.ConclusionsThis study underlines the need to consider and assess for psychotic symptoms in the frontotemporal dementia-amyotrophic lateral sclerosis continuum particularly in those with C9orf72 gene expansions. The network of brain regions identified in this study is strikingly similar to that identified in other psychotic disorders such as schizophrenia, which suggests that treatment strategies in psychiatry may be beneficial for the management of psychotic symptoms in frontotemporal dementia.

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“…Other behavioral characteristics, seen in some patients, include hypersensitivity or hyposensitivity to pain, sounds and other sensory stimuli . Moreover, although thought to be rare , it is now recognized that some bvFTD patients experience psychotic symptoms of delusions and hallucinations . Behavioral changes are accompanied by cognitive impairments in “frontal” executive functions such as abstraction, planning, attention, reasoning and judgment.…”

Section: Clinical Syndromesmentioning

confidence: 99%

Frontotemporal lobar degeneration: Pathogenesis, pathology and pathways to phenotype

2017

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Frontotemporal Lobar Degeneration (FTLD) is a clinically, pathologically and genetically heterogeneous group of disorders that affect principally the frontal and temporal lobes of the brain. There are three major associated clinical syndromes, behavioral variant frontotemporal dementia (bvFTD), semantic dementia (SD) and progressive non-fluent aphasia (PNFA); three principal histologies, involving tau, TDP-43 and FUS proteins; and mutations in three major genes, MAPT, GRN and C9orf72, along with several other less common gene mutations. All three clinical syndromes can exist separately or in combination with Amyotrophic Lateral Sclerosis (ALS). SD is exclusively a TDP-43 proteinopathy, and PNFA may be so, with both showing tight clinical, histological and genetic inter-relationships. bvFTD is more of a challenge with overlapping histological and genetic features, involvement of any of the three aggregating proteins, and changes in any of the three major genes. However, when ALS is present, all cases show a clear histological phenotype with TDP-43 aggregated proteins, and familial forms are associated with expansions in C9orf72. TDP-43 and FUS are nuclear carrier proteins involved in the regulation of RNA metabolism, whereas tau protein - the product of MAPT - is responsible for the assembly/disassembly of microtubules, which are vital for intracellular transport. Mutations in TDP-43 and FUS genes are linked to clinical ALS rather than FTLD (with or without ALS), suggesting that clinical ALS may be a disorder of RNA metabolism. Conversely, the protein products of GRN and C9orf72, along with those of the other minor genes, appear to form part of the cellular protein degradation machinery. It is possible therefore that FTLD is a reflection of dysfunction within lysosomal/proteasomal systems resulting in failure to remove potentially neurotoxic (TDP-43 and tau) aggregates, which ultimately overwhelm capacity to function. Spread of aggregates along distinct pathways may account for the different clinical phenotypes, and patterns of progression of disease.

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Psychotic symptoms in frontotemporal dementia: a diagnostic dilemma?

Cited by 77 publications

References 33 publications

Psychosis in behavioral variant frontotemporal dementia

Psychosis in behavioral variant frontotemporal dementia

The neural correlates and clinical characteristics of psychosis in the frontotemporal dementia continuum and the C9orf72 expansion

Frontotemporal lobar degeneration: Pathogenesis, pathology and pathways to phenotype

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