J Mol Neurosci. 2011 Nov;45(3):656-62. doi: 10.1007/s12031-011-9636-x. Epub 2011 Oct 5.
Clinical phenomenology and neuroimaging correlates in ALS-FTD.
Source
Department
of Neurology, University of California, San Francisco, 350 Parnassus
Avenue, Suite 500, San Francisco, CA 94117, USA.
catherine.lomen-hoerth@ucsf.edu
Abstract
The
overlap of frontotemporal dementia (FTD) and amyotrophic lateral
sclerosis (ALS) has been well documented in FTD patients with co-morbid
motor neuron degeneration and in ALS patients with frontotemporal
dysfunction. Up to 15% of FTD patients and 30% of ALS patients
experience the overlap syndrome. The syndrome may be difficult to
identify since patients often present either to a neuromuscular clinic
or a memory disorder's center, each which may have limited expertise in
the other specialty. Survival is greatly impacted for both disorders in
the co-morbid condition, making identification of this syndrome
critical. The clinical characteristics of the overlap syndrome with new
diagnostic criteria will be discussed along with screening strategies,
including the UCSF Screening battery and clinical neurophysiology
techniques. Treatable mimics of this disorder will also be described and
management techniques. Neuroimaging findings will be summarized, which
show that the frontotemporal impairment in ALS patients lies on a
continuum. Identification of the overlap syndrome also provides a unique
opportunity to study very early signs of FTD and conversely, very early
signs of ALS, to gain greater insight into both disorders.
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