Diagnosis and Management of Behavioral Variant Frontotemporal Dementia

Abstract

Frontotemporal dementia (FTD) was documented over a century ago. The last decade, however, has seen substantial changes in our conceptions of this increasingly recognized disorder. Different clinical variants have been delineated, the most common of which is the behavioral variant (bvFTD). Updated diagnostic criteria have been established. New histopathological findings and genetic etiologies have been discovered. Research continues to uncover molecular mechanisms by which abnormal proteins accumulate in degenerating brain tissue. Novel neuroimaging techniques suggest that functional networks are diminished in bvFTD that may be relevant to empathy and social behavior. Despite rapid advances in our understanding of bvFTD, the disease is still under-recognized and commonly misdiagnosed. The result is inappropriate patient care. Recognizing the various presentations of bvFTD and its histological and genetic subtypes may further diagnosis, treatment and research.

Historical Evolution of the Frontal Lobe Syndrome

“…Tourette's), other behavioral disturbances like stereotypical language, motor or disinhibition occur [39][40][41][42][43][44] . Many of the differential diagnoses show great clinical overlap, especially bvFTD and psychiatric disorders like schizophrenia [45][46][47] . After taking a detailed history and a physical exam, more discriminating instruments might be needed to differentiate between FLS causes.…”

Single Subject Classification of Alzheimer’s Disease and Behavioral Variant Frontotemporal Dementia Using Anatomical, Diffusion Tensor, and Resting-State Functional Magnetic Resonance Imaging

“…Accurate and confident differentiation between these disease types is crucial for the proper management, prognosis, and potential treatment of patients with dementia [4][5][6]. Yet, despite distinct clinical diagnostic criteria [1,7], heterogeneity and overlap of clinical manifestations often complicate differential diagnosis [8].…”

Impulsivity, decreased social exploration, and executive dysfunction in a mouse model of frontotemporal dementia

“…Clinically, FTD is divided into three subtypes: a behavioral variant (bvFTD) that affects social skills, emotions, personal conduct, and self-awareness, semantic dementia that compromises language comprehension, and motor variants leading to muscle wasting (Hodges et al, 2004). BvFTD presents with changes in social behavior and conduct, such as loss of social awareness and social withdrawal, restlessness and poor impulse control leading to compulsive behaviors including stereotyped hair-pulling and skin picking (Eslinger, Moore, Anderson, & Grossman, 2011;Lindau et al 2000;Mendez & Perryman, 2002;Pressman & Miller, 2014;Snowden et al, 2001Snowden et al, , 2003. At later stages, FTD patients develop deficits in executive function: they have problems planning, coordinating and executing simple tasks (Harciarek and Cosentino, 2013;Huey et al, 2009;Johns et al, 2009;Moy et al, 2004;Stopford et al, 2012).…”