Article

Delusions and hallucinations in frontotemporal dementia: a clinicopathologic case report.

Department of Clinical Neurological Sciences, Division of Neurology, St Joseph's Hospital, University of Western Ontario, Ontario, Canada.
Cognitive and behavioral neurology: official journal of the Society for Behavioral and Cognitive Neurology (Impact Factor: 1.14). 07/2008; 21(2):107-10. DOI: 10.1097/WNN.0b013e3181799e19
Source: PubMed

ABSTRACT Frontotemporal dementia (FTD) is associated with marked behavior changes, but hallucinations and delusions are rare.
To report a case of FTD with early and persistent delusions, including de Clerambault syndrome.
We describe the clinical, neuroradiologic, and neuropathologic findings of a 76-year-old woman with an 11-year course of FTD with bizarre delusions and hallucinations.
The patient's autopsy examination of the brain revealed pathology consistent with frontotemporal lobar degeneration with ubiquitin-positive and transactive response (TAR)-DNA-binding protein-43-positive inclusions and hippocampal sclerosis.
Delusions and hallucinations can occur in proven frontotemporal pathology.

1 Follower
 · 
382 Views
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Behavioral and psychological symptoms of dementia (BPSD) are commonly observed over the course of Alzheimer's disease (AD). However, it is unclear whether BPSD are part of AD neuropathology or rather represent a psychological reaction to cognitive disabilities. Using voxel-based-morphometry (VBM), we aimed to clarify this issue by investigating patients with AD at different clinical stages. Twenty-seven patients with AD (12 early [ADe] and 15 moderate [ADm]), 19 with amnestic mild cognitive impairment (a-MCI), and 23 healthy controls underwent MRI scanning at 3T. Assessment of BPSD was done in each patient using the Neuropsychiatric Inventory-12 (NPI-12). VBM was used to investigate changes in grey matter (GM) atrophy across groups, and associations between regional GM volumes and occurrence and severity of BPSD in patients. Mood disorders, anxiety, and agitation were present in both a-MCI and AD, while psychotic symptoms were observed mainly in AD. As expected, VBM showed only limited areas of GM atrophy in a-MCI patients, with a progressive extension in ADe and ADm patients (PFWE-corrected-values < 0.05). Disinhibition was strongly associated with GM volume in bilateral cingulate and right middle frontal gyri, while delusions were associated with GM volume in right hippocampus (PFWE-corrected-values < 0.05). This study confirms that BPSD are present since the earliest AD stages. Interesting associations were found in regions traditionally implicated by AD neuropathology. This suggests that BPSD are likely to represent clinical features of AD and should be regarded for their diagnostic and prognostic value.
    Journal of Alzheimer's disease: JAD 01/2010; 21(2):627-39. DOI:10.3233/JAD-2010-100048 · 3.61 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Clinical distinction between Lewy bodies disease (LBD) and frontotemporal dementia (FTD) is sometimes difficult. Nigrostriatal dopaminergic degeneration occurs in both LBD and FTD, limiting helpfulness of DAT imaging to differentiate these forms of dementia. Several studies have emphasized the usefulness of myocardial scintigraphy with (123)Metaiodobenzylguanidine ((123)I-MIGB) in assessing the sympathetic nerve terminals in LBD demonstrating that cardiac (123)I-MIGB uptake is decreased in patients with this disease. We investigated the role of cardiac (123)I-MIBG scintigraphy in differentiating patients with LBD from those with FTD. Clinical diagnosis of LBD and FTD was determined according to established consensus criteria. Nine patients with LBD (1 possible and 8 probable), 6 patients with FTD, and 16 control subjects were involved in the study. The heart to mediastinum ratio (H/M) of (123)I-MIBG uptake was markedly reduced in all patients with LBD (H/M early: 1.25±0.12; delayed: 1.14±0.13) whereas it was normal in patients with FTD (H/M early: 1.86±0.20; delayed: 1.80±0.23) and in controls (H/M early: 1.91±0.17; delayed: 1.99±0.19), suggesting that cardiac (123)I-MIBG scintigraphy can help distinguish patients with LBD from those with FTD.
    Neurobiology of aging 02/2009; 31(11):1903-11. DOI:10.1016/j.neurobiolaging.2008.11.009 · 4.85 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Some patients with frontotemporal dementia (FTD) have concomitant extrapyramidal symptoms and psychosis and may simultaneously meet consensus criteria for FTD and for dementia with Lewy bodies (DLB). Clinicopathologic studies are helpful in understanding the underlying neurodegenerative process in such cases. To describe clinical and pathologic features of 6 patients with signs and symptoms suggestive of both a diagnosis of FTD and DLB at first clinical presentation, of which 2 patients have now undergone autopsy, and to compare them with autopsy-confirmed FTD and Lewy body disease patients. All 6 patients met published consensus criteria for a diagnosis of both FTD and DLB (5 probable and 1 possible). Clinical symptoms of FTD included personality and behavioral changes, whereas those suggestive of DLB included Parkinsonism, fluctuating cognition, parasomnia, and hallucinations. Five patients underwent single photon emission computed tomography ((99m)Tc) imaging, which showed varying degrees of frontal lobe hypoperfusion. Magnetic resonance imaging, electroencephalogram, and electromyogram were not helpful in differentiating FTD from DLB. Histologic examination of the 2 autopsy cases was consistent with a pathologic diagnosis of TDP-43 proteinopathy; specifically frontotemporal lobar degeneration with ubiquitin-only immunoreactive changes, type 1. There were significant differences between these 6 patients and the 2 groups of autopsy confirmed FTD and Lewy body disease patients. We have identified a novel group of FTD patients with clinical features that overlap with DLB, yet seem to be different from both typical FTD and typical Lewy body disease.
    Cognitive and behavioral neurology: official journal of the Society for Behavioral and Cognitive Neurology 10/2008; 21(3):157-63. DOI:10.1097/WNN.0b013e3181864a09 · 1.14 Impact Factor