Delusions in frontotemporal lobar degeneration

Omar, Rohani; Sampson, Elizabeth L; Loy, Clement T.; Mummery, Catherine J.; Fox, Nick C.; Rossor, Martin N.; Warren, Jason D.

doi:10.1007/s00415-009-0128-7

Cited by 48 publications

(38 citation statements)

References 26 publications

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“…31 Interestingly, we observed differences in symptoms across both types, with psychosis being more common in behavioral-dominant FTD-MND and parkinsonism and limb apraxia being more common in language-dominant FTD-MND. Likewise, psychosis is associated with the behavioral variant of FTD but not the language variant of FTD, 32 and therefore our findings in FTD-MND mirror findings in FTD. Limb apraxia has not been emphasized previously in patients with FTD-MND, but in our study we showed that almost 50% of patients with language-dominant FTD-MND have limb apraxia.…”

Section: Discussionsupporting

confidence: 78%

Predicting survival in frontotemporal dementia with motor neuron disease

Coon¹,

Sorenson²,

Whitwell³

et al. 2011

Neurology

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Objective: To determine whether clinical and demographic features are associated with prognosis in patients with frontotemporal dementia and motor neuron disease (FTD-MND).Methods: This was a case series of FTD-MND categorized according to behavioral-or languagedominant symptoms at presentation and throughout the disease course. Demographic, clinical, imaging, and survival data were analyzed with respect to dominant FTD-MND type. Voxel-based morphometry was used to assess and compare regional patterns of atrophy in behavioral-and language-dominant FTD-MND types. Results:Of the 56 patients with FTD-MND who were identified, 31 had dominant behavioral symptoms and 25 had dominant language symptoms; 53 patients had died. A survival difference was present between types, with patients with behavioral-dominant symptoms surviving 506 days longer than patients with language-dominant symptoms (mean 1,397 vs 891 days; p ϭ 0.002). There was also a difference in time from diagnosis to death (p ϭ 0.02) between groups. Patients with language-dominant disease were more likely to have bulbar-onset than limb-onset motor neuron disease (MND) (p ϭ 0.01). There was a similar pattern of frontal and temporal lobe atrophy in both types, although there was some evidence for the behavioral type to have more frontal atrophy and the language type to have more left temporal atrophy. Conclusions:In our series of patients with FTD-MND, language-dominant FTD-MND was associated with bulbar-onset MND and a shorter survival. There was also evidence that the dominant FTD-MND type is related to differences in brain atrophy patterns. Neurology Frontotemporal dementia (FTD) encompasses clinical syndromes characterized by progressive and insidious behavioral changes and language deficits.1,2 Clinical symptoms of motor neuron disease (MND) can develop in patients with FTD, whereas patients with MND may manifest behavioral or language symptoms in the disease course.3-6 Beyond the clinical features, a pathologic overlap exists between FTD and MND with the presence of ubiquitin/TAR DNAbinding protein (TDP-43)-immunoreactive inclusions identified in both disorders, suggesting a TDP-43 proteinopathy continuum. [7][8][9] In FTD, behavioral and language deficits are well-characterized with established language syndromes of progressive nonfluent aphasia and semantic dementia.2 Although behavioral and language symptoms often overlap in FTD, the predominant subtype has implications for the disease course with demographic differences existing between behavioral and language groups with respect to gender, age at onset, and survival. 10 Distinguishing clinical features in FTD also has implications for pathophysiology because various profiles of tau and TDP-43 deposition have been associated with different clinical syndromes.

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Section: Discussionsupporting

confidence: 78%

Predicting survival in frontotemporal dementia with motor neuron disease

Coon¹,

Sorenson²,

Whitwell³

et al. 2011

Neurology

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“…55 VHs are more common in the right than in the left temporal variant frontotemporal lobar degeneration, 56 often associated with delusions. 57 Imaging Features. While atrophy in the anterior temporal and medial frontal lobes is characteristic of frontotemporal lobar degeneration (On-line Fig 4), 58 specific imaging findings can reflect underlying subtypes.…”

Section: 45mentioning

confidence: 99%

Distinguishing Neuroimaging Features in Patients Presenting with Visual Hallucinations

Winton-Brown¹,

Ting

Mocellin³

et al. 2016

American Journal of Neuroradiology

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SUMMARY:Visual hallucinations are relatively uncommon presentations in medical and psychiatric clinics, where they are generally regarded as a marker of possible underlying "organic" brain disease. Thus, patients with visual hallucinations are often referred for imaging of the brain. This article presents a pragmatic approach for the radiologist reviewing such imaging. Because conditions that can present with visual hallucinations are legion, a familiarity with the features of the hallucinations themselves, which can serve as clues to the underlying cause, can be helpful in interpreting such cases. We consider the nature of visual hallucinations and the mechanisms underlying their formation. We then provide a framework to guide the search for their cause, first in terms of focal lesions along the visual pathway and then global conditions affecting Ͼ1 region. ABBREVIATIONS:CJD ϭ Creutzfeldt-Jakob disease; VH ϭ visual hallucination

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“…Delusions are false beliefs that are incomprehensible, incorrigible, and held with subjective conviction despite proof or evidence to the contrary [27]. Although they are infrequent [10,36], they may occur in up to 14% of patients with bvFTD, particularly if they belong to specific subgroups [31,44,58]. Two of our patients have morbid jealousy or the Othello syndrome, a delusional belief that their spouse or sexual partner was unfaithful.…”

Section: Discussionmentioning

confidence: 97%

False Reports from Patients with Frontotemporal Dementia: Delusions or Confabulations?

Mendez

Fras

Kremen

et al. 2011

Behavioural Neurology

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Abstract.Patients with behavioral variant frontotemporal dementia (bvFTD) can make false statements consistent with delusions or confabulations. It is unclear whether bvFTD is primarily associated with either delusions or with confabulations and whether they can be explained by the pathophysiology of this disease. In order to clarify this, we retrospectively surveyed the records of 48 patients with bvFTD for the presence of any false reports and identified four patients. Their false reports included continued interaction with a favorite but dead relation, fictitious marriages with movie stars, and two who claimed that their partner was having an affair. When confronted with the falsity of their statements, the patients conveyed a lack of certainty regarding their external or internal source but persisted in the constancy of their reports. On functional neuroimaging, the patients had predominant frontal involvement. This report found that patients with bvFTD can have both fantastic, wish fulfilling confabulations and typical content-specific delusions. We propose that both phenomena result from known disturbances of ventromedial prefrontal cortex in bvFTD, including deficits in source monitoring and in activating an automatic "doubt tag" for false reports.

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Delusions in frontotemporal lobar degeneration

Cited by 48 publications

References 26 publications

Predicting survival in frontotemporal dementia with motor neuron disease

Predicting survival in frontotemporal dementia with motor neuron disease

Distinguishing Neuroimaging Features in Patients Presenting with Visual Hallucinations

False Reports from Patients with Frontotemporal Dementia: Delusions or Confabulations?

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