Slowly progressive frontotemporal lobar degeneration caused by the C9ORF72 repeat expansion: a 20-year follow-up study.

We describe a 20-year follow-up of a unique case with very slowly progressive FTLD caused by the C9ORF72 repeat expansion. In serial neuropsychological examinations, the patient's cognitive decline was exceptionally slow and after 20 years the patient still was mainly independent in activities of daily living. Our case indicates that there is great individual variation in the progression and duration of C9ORF72-associated FTLD, and also language variants or mixed phenotypes may be present.
PMID: 24417314 [PubMed - as supplied by publisher]

Source: Neurocase - January 14, 2014 Category: Neurology Authors: Suhonen NM, Kaivorinne AL, Moilanen V, Bode M, Takalo R, Hänninen T, Remes AM Tags: Neurocase Source Type: research