MOVEMENT DISORDERS IN FRONTOTEMPORAL LOBAR DEGENERATION

Abstract

Frontotemporal Lobar Degeneration (FTLD) consists of a wide spectrum of pathological entities related to the pathological aggregation of certain proteins in the central nervous system. The clinical expression of this spectrum is Frontotemporal Dementia (FTD), which has two subtypes, the behavioral variant of FTD (bvFTD) and Primary Progressive Aphasia (PPA). In both, a variety of movement disorders may occur. These include akinetic-rigid syndrome, Richardson's syndrome, Corticobasal Syndrome (CBS), Motor Neuron Disease (MND), stereotypies, etc. As with other neurodegenerative diseases, proper diagnosis of frontotemporal degeneration is still a challenge, despite the continuous increase in the variety of diagnostic tools. The purpose of this article is to analyze these movement disorders and correlate them with neuropsychological, imaging, genetic and pathologic findings that direct the neurologist to the most likely diagnosis.