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Neurocase
Behavior, Cognition and Neuroscience
Volume 21, 2015 - Issue 1
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Original Articles

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

N.-M. SuhonenInstitute of Clinical Medicine, Neurology, University of Oulu, Oulu, Finland;Department of Neurology, Oulu University Hospital, Oulu, Finland
,
A.-L. KaivorinneInstitute of Clinical Medicine, Neurology, University of Oulu, Oulu, Finland;Department of Neurology, Oulu University Hospital, Oulu, Finland
,
V. MoilanenInstitute of Clinical Medicine, Neurology, University of Oulu, Oulu, Finland
,
M. BodeDepartment of Diagnostic Radiology, Oulu University Hospital, Oulu, Finland
,
R. TakaloDivision of Nuclear Medicine, Department of Diagnostic Radiology, Oulu University Hospital, Oulu, Finland
,
T. HänninenInstitute of Clinical Medicine – Neurology, University of Eastern Finland, Kuopio, Finland;Department of Neurology, Kuopio University Hospital, Kuopio, Finland
&
A.M. RemesInstitute of Clinical Medicine – Neurology, University of Eastern Finland, Kuopio, Finland;Department of Neurology, Kuopio University Hospital, Kuopio, FinlandCorrespondence[email protected]
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Pages 85-89 | Received 22 Jun 2013, Accepted 29 Sep 2013, Published online: 14 Jan 2014
 
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Abstract

A hexanucleotide expansion in chromosome 9 open-reading frame 72 (C9ORF72) has been found to be a major cause of frontotemporal lobar degeneration (FTLD). 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.

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