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Neurodegenerative Diseases: Fronto-temporal Lobar Degeneration

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Imaging Brain Diseases

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Abstract

Fronto-temporal dementia (FTD) or fronto-temporal lobar dementia (FTLD) includes four clinical subgroups, i.e., semantic dementia, progressive non-fluent aphasia, FTD-behavioral variant, and FTD with motor neuron disease/ALS.

A variety of neuropathologic subgroups is recognized based on involved genes (TDP-43, granulin (GRN) VCP C9ORF MAPT FUS tau 3R, 4R) and inclusions (neuronal cytoplasmic inclusions, neuronal intranuclear inclusions, dystrophic neurites, glial inclusions).

There is no specific treatment; symptomatic treatment includes cholinesterase inhibitors, memantine, selective serotonin reuptake inhibitors, and l-dopa. Clinical outcome is poor as the disease progresses with development of akinetic mutism during the end stages of the disease.

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Authors and Affiliations

  1. Division of Neuropathology, Neuromed Campus, Kepler University Hospital, Johannes Kepler University, Linz, Austria

    Serge Weis

  2. Department of Neuroradiology, Neuromed Campus, Kepler University Hospital, Johannes Kepler University, Linz, Austria

    Michael Sonnberger, Eva Voglmayr & Raimund Kleiser

  3. Department of Neuro-Nuclear Medicine, Neuromed Campus, Kepler University Hospital, Johannes Kepler University, Linz, Austria

    Andreas Dunzinger

  4. Department of Neurosurgery, Neuromed Campus, Kepler University Hospital, Johannes Kepler University, Linz, Austria

    Martin Aichholzer

  5. PMU University Institute for Medical & Chemical Laboratory Diagnostics, Salzburg, Austria

    Peter Strasser

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Weis, S. et al. (2019). Neurodegenerative Diseases: Fronto-temporal Lobar Degeneration. In: Imaging Brain Diseases. Springer, Vienna. https://doi.org/10.1007/978-3-7091-1544-2_34

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