Publications by authors named "Janine Diehl"

Mutations in the gene for valosin containing protein (VCP) cause autosomal dominant inclusion body myopathy associated with Paget disease and frontotemporal dementia (IBMPFD). To investigate the role of this novel gene in sporadic forms of frontotemporal dementia (FTD), we genotyped 27 single nucleotide polymorphisms covering the entire VCP genomic region in 198 patients with sporadic FTD and 184 matched controls from Germany. No significant association could be demonstrated.

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Behavioural problems are a key feature of frontotemporal lobar degeneration (FTLD). Also, FTLD patients show impairments in emotion processing. Specifically, the perception of negative emotional facial expressions is affected.

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Objective: Although the clinical course of Alzheimer disease (AD) is gradual, it is useful for a number of reasons to distinguish between different levels of severity. The Clinical Dementia Rating (CDR) has demonstrated high validity and reliability for this purpose, but it requires a considerable amount of data to be collected both from the patient and from an informant. In the present study, the authors mapped Mini-Mental State Examination (MMSE) scores onto CDR categories to determine how well the MMSE performs as a surrogate of the CDR as a timesaving method of staging dementia.

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Background: Until recently, frontotemporal lobar degeneration (FTLD) was considered a rare neurodegenerative disorder that was difficult to diagnose. The publication of consensus criteria for FTLD, however, prompted systematic studies. The criteria categorize FTLD into 3 subgroups: frontotemporal dementia, semantic dementia, and progressive nonfluent aphasia.

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Frontotemporal dementia is a relatively rare neurodegenerative disease. In the majority of cases the onset is at a presenile age, and changes of behavior as well as alterations of personality and social conduct are predominant symptoms whereas cognitive deficits are mild. Therefore interventions for caregivers including self-help groups and educational programs which are usually tailored to Alzheimer's disease often do not meet the needs of caregivers of patients with FTD.

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[Frontotemporal dementia].

Wien Med Wochenschr

June 2002

Frontotemporal dementia is a rare form of progressive intellectual deterioration. Its most prominent clinical features are alterations in personality, motivation, and social conduct whereas memory and orientation remain largely unimpaired. Several underlying neurodegenerative processes may be distinguished which are confined to the cerebral cortex in most cases but occasionally involve the basal ganglia and rarely the anterior horn cells.

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[Vascular dementias].

Fortschr Neurol Psychiatr

March 2002

Cerebrovascular disorders are the second most frequent cause of dementia in late life next to Alzheimer's disease. A recent community-based autopsy study has demonstrated that relevant cerebrovascular changes are much more prevalent in individuals aged 70+ years than previously assumed. Furthermore, the combination between cerebrovascular lesions and Alzheimer-type pathology is the most common neuropathological finding in elderly patients with dementia.

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