Significats de multiple system tauopathy with presenile dementia en anglès

In semantic dementia, there was asymmetrical temporal lobe atrophy, with greater left-sided damage.

Three groups of 10 subjects were studied: semantic dementia patients, Alzheimer's disease patients, and control subjects.

Patients with bvFTD and semantic dementia had a strong sucrose preference compared with the other groups.

She had semantic dementia, a rare disease that slowly and inescapably takes a person's language away.

This study explored this question by comparing three patients with semantic dementia to 40 normal controls performing different everyday activities.

Recent research on frontotemporal dementia highlights the potential value of these approaches.

Results: The frontotemporal dementia subgroup represented approximately half of all FTLD diagnoses.

Other diseases which may involve tau deposition include frontotemporal dementia and corticobasal degeneration.

Neuroimaging features were consistent with the diagnosis of frontotemporal dementia.

The authors conclude that PSEN1 mutations can be associated with clinical features of frontotemporal dementia.

Hierarchical cluster analysis after exclusion of these 12 genes differentiated 4 of the 6 PD cases from progressive supranuclear palsy and frontotemporal dementia with parkinsonism.

This has now changed with the discovery of close to 20 mutations in the tau gene in frontotemporal dementia with Parkinsonism linked to chromosome 17.

Mary Johnson, age fifty-seven, Frontotemporal lobe dementia

Methods: Electronic databases were searched using terms related to frontotemporal lobar degeneration and movement disorders.

Mutations in the gene encoding PGRN give rise to shortage of PGRN and cause familial frontotemporal lobar degeneration.

Summary: This represents the first systematic review and meta-analysis of the occurrence of movement disorder phenomenology in genetic frontotemporal lobar degeneration.

These findings further highlight that plasma PGRN levels may not accurately predict clinical features or response to future frontotemporal lobar degeneration therapies.

Mutations in various RBPs cause several diseases of the central nervous system, including frontotemporal lobar degeneration, amyotrophic lateral sclerosis and fragile X syndrome.