Discriminating behavioral and language variants of frontotemporal lobar degeneration


  • C9orf72 expansions, age at onset (AAO) and Northern European ancestry correlates with the behavior variant of frontotemporal lobar degeneration (bvFTD), not primary progressive aphasia (PPA).

Why this matters

  • Frontotemporal lobar degeneration (FTLD) refers to the second most common form of young-onset dementia after Alzheimer’s Disease. The major clinical syndromes are behavior variant (bvFTD) and/or language dysfunctions, known as primary progressive aphasia (PPA).

  • Repeat expansions of C9orf72 have been identified in FTLD and have been reported to be associated with phenotypical and pathological heterogeneity.

International Medical Press is a global provider of independent medical education. Its mission is to provide healthcare professionals with high-quality, trusted medical information with the aim of helping optimize patient care.

No responsibility is assumed by International Medical Press for any injury and/or damage to persons or property through negligence or otherwise, or from any use or operation of any methods, products, instructions, or ideas contained in the material herein. Because of rapid advances in the medical sciences, International Medical Press recommends that independent verification of diagnoses and drug dosages should be made. The opinions expressed do not reflect those of International Medical Press or the sponsor. International Medical Press assumes no liability for any material contained herein.