Work

C9orf72-Related Amyotrophic Lateral Sclerosis and Frontotemporal Dementia

Year: 2015

Type: book-chapter

Abstract: C9orf72 frontotemporal dementia and/or amyotrophic lateral sclerosis (C9orf72-FTD/ALS) is characterized most often by frontotemporal dementia (FTD) and upper and lower motor neuron disease (MND); however, atypical presentations also occur. Age at onset is usually between 50 and 64 years (range: 20-91 years) irrespective of the presenting manifestations, which may be pure FTD, pure amyotrophic lateral sclerosis (ALS), or a combination of the two phenotypes. The clinical presentation is highly heterogeneous and may differ between and within families, causing an unpredictable pattern and age of onset of clinical manifestations. The presence of MND correlates with an earlier age of onset and a worse overall prognosis.

Source: VUBIR (Vrije Universiteit Brussel)

Authors: Marc Cruts, Sebastiaan Engelborghs, Julie van der Zee, Christine Van Broeckhoven

Language: English

Topic: Amyotrophic Lateral Sclerosis Research

Subfield: Clinical Neurology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: green

Funder: Vlaamse regering

OpenAlex ID: W178081570