Dementia in First-Degree Relatives of Patients with Frontotemporal Dementia

Gräsbeck, Anne; Horstmann, Vibeke; Nilsson, Karin; Sjöbeck, Martin; Sjöström, Hans; Gustafson, Lars

doi:10.1159/000082886

Cited by 10 publications

(4 citation statements)

References 77 publications

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“…were included into the positive ones in all the calculations [27] . This resulted in 3 groups for each of the diseases; ill cases (yes/ probable), healthy cases (no), and cases with lack of information for a diagnosis (unknown).…”

Section: Diagnosticsmentioning

confidence: 99%

Occurrence of Depression in Families with Frontotemporal Dementia: A Family History Study

Horstmann¹,

Gräsbeck²

2009

Neuroepidemiology

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Background: There are clinical similarities between frontotemporal dementia (FTD) and depression. The aim is to study co-aggregation of depression in families with FTD, indicating the existence of common aetiological factors. Methods: The study included 74 index patients with FTD and their 540 first-degree relatives above the age of 15 years. Occurrence of depression was studied at 3 different levels. Results: The incidence of depression in first-degree relatives of FTD patients was not higher than that of a general population. Occurrence of depression was not higher in families where parents had FTD compared to families with parents having no indications of FTD. Individuals with FTD had not suffered from depression to a greater extent than those without FTD. Conclusions: The hypothesis of a common aetiological factor of FTD and depression was not supported.

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Section: Diagnosticsmentioning

confidence: 99%

Occurrence of Depression in Families with Frontotemporal Dementia: A Family History Study

Horstmann¹,

Gräsbeck²

2009

Neuroepidemiology

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“…Some 38 to 50% of clinical FTD cases appear to be familial (Stevens et al 1998;Grasbeck et al 2005). Countless kindred have been described that demonstrate segregation of a disease in an autosomal dominant pattern of inheritance.…”

Section: Geneticsmentioning

confidence: 99%

A review on primary progressive aphasia

Léger

Johnson²

2008

NDT

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Primary progressive aphasia (PPA) is a neurodegenerative disease of insidious onset presenting with progressive isolated loss of language function, without signifi cant impairment in other cognitive domains. Current diagnostic criteria require the language dysfunction to remain isolated for at least two years, and to remain the salient feature as the disease progresses, usually to involve other domains such as behavior, executive functions, and judgment. Although PPA in its early stages can usually be differentiated from probable Alzheimer's disease (PRAD) and the behavioral variant of frontotemporal lobar degeneration by the absence of signifi cant changes in memory and behavior, and the preservation of activities daily living, progression of the disease often leads to defi cits more consistent with the latter. Underlying etiologies remain heterogeneous: the neuropathological characteristics associated with frontotemporal lobar degeneration, cortocobasal degeneration, and motor neuron disease are usually found. There is a strong genetic susceptibility with affl iction of fi rst-degree relatives with similar disease in up to 40 to 50% in some series. Pathogenic mutations in genes coding for the proteins tau and progranulin have been isolated. These are leading to a better understanding of the neuropathological mechanisms and hopefully targeted disease-modifying therapy. Current therapy is limited to improving mood symptoms and targeting behavior changes as they develop. Referral to specialized centers where speech therapy, counseling, and education for both patient and caregiver are available may be helpful.

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“…The probability of a patient's first-degree relatives developing a similar disorder is much higher in FTD than it is in AD, estimated at 40% to 45% [65][66][67]. Mutations in the MAPT gene encoding microtubule-associated protein tau were discovered to cause FTD in 1998 [68][69][70][71].…”

Section: Geneticsmentioning

confidence: 99%

Frontotemporal dementia

Roberson

2006

Curr Neurol Neurosci Rep

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Frontotemporal dementia (FTD) and related conditions are often considered daunting because of the numerous inter-related clinical syndromes and their apparently heterogeneous pathologic substrates. Although the labyrinthine complexity of the disease seemingly continues to grow, recent discoveries have made the maze of FTD somewhat more navigable, spurring new interest in the field. This review begins by surveying the fascinating clinical presentations of these conditions and the few currently available treatments, then turns to recent progress in understanding the pathophysiology of FTD. Among the important advances surveyed are clinicopathologic correlations that enable prediction of the pathologic substrate of certain clinical subtypes, and genetic studies that have been particularly fruitful in identifying new causes of FTD.

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Dementia in First-Degree Relatives of Patients with Frontotemporal Dementia

Cited by 10 publications

References 77 publications

Occurrence of Depression in Families with Frontotemporal Dementia: A Family History Study

Occurrence of Depression in Families with Frontotemporal Dementia: A Family History Study

A review on primary progressive aphasia

Frontotemporal dementia

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