In the last decade, there has been enormous progress in our understanding of
Frontotemporal Lobar Degeneration (
FTLD). Published clinicopathological series have clearly demonstrated an overlap between the clinical syndromes subsumed under the term
frontotemporal dementia and the
Progressive Supranuclear Palsy (PSP), and the
Corticobasal Degeneration (CBD) syndrome. From a neuropathological point of view, two broad pathological subdivisions of
FTLD are currently recognized: a) tau-positive pathology due to the accumulation of various forms of the
microtubule-associated protein tau, that encompasses
FTLD with Pick bodies, PSP and CBD, and b) tau-negative pathology, mainly characterized by
ubiquitin/TDP-43-immunoreactive inclusions and in some cases due to
Progranulin mutations. Several
biological markers in cerebrospinal fluid and in blood have been evaluated to identify monogenic forms of
FTLD and to differentiate either
FTLD spectrum disorders or
FTLD from other
neurodegenerative disorders. The proposed
biomarkers are primarily related to the mechanisms underlying the accumulation of the abnormal
proteins in
FTLD such as Tau, TDP-43 and
Progranulin. These
biomarkers may support the accurate diagnosis of the specific diseases causing
FTLD, can be useful in assessing efficacy during pharmacological trials, and may help in identifying new molecular targets for treatment approaches. In this review, we summarise the most recent findings on
biological markers and their usefulness in clinical practice for the diagnosis and management of
FTLD.