Regarding progression, FTD is marked by shorter duration of survival and more rapid decline of cognition and function than Alzheimer's disease. Of the PPA subtypes, male predominance has been reported in svPPA, with female predominance in nfvPPA. Male predominance of bvFTD has been reported across a number of studies and centers. Independent studies investigating the prevalence of FTD in the UK estimated a prevalence of about 15 cases/100,000 in adults between 45 and 64 years of age, which was comparable to the prevalence of early-onset Alzheimer's disease. Recent epidemiological work estimates a range from 15 to 22/100,000. The number of psychiatric and neurological disorders that can resemble the spectrum of FTD symptomology may contribute to misclassification and underestimation. We also discuss best practices for how to manage the challenges associated with FTD.ĭue to the diagnostic challenges associated with FTD, the true prevalence of this disease is likely underestimated. This review underscores the importance of identification of these deficits to aid in early and accurate clinical diagnosis and management. ![]() Recent advances have shed more light on the specific cognitive, behavioral and socio-emotional deficits seen in these syndromes. This review summarizes the clinically relevant findings typically seen in each of the three FTD subtypes. nfvPPA is accompanied by left inferior frontal and insular atrophy expressive speech and syntax difficulties are characteristic of the disease early in its course. Most patients present initially with greater left hemisphere atrophy however, approximately a quarter of cases present with initial right anterior temporal lobe atrophy, and is associated with a more behavioral presentation of symptoms, including: social awkwardness, loss of insight and difficulty with face recognition. The semantic variant is characterized by bilateral anterior temporal lobe atrophy, associated with language, compulsions and dysfunctions in emotional processing. Loss of semantic knowledge is associated with the semantic variant of primary progressive aphasia (svPPA), while agrammatism and motor–speech difficulties are associated with nonfluent variant of primary progressive aphasia (nfvPPA). The remaining subtypes are classified as variants of primary progressive aphasia (PPA), and are marked by initial and prominent disturbance and decline of language functioning. The behavioral variant is characterized by focal and prominent bilateral frontal atrophy, though some reports suggest more right-hemisphere involvement than left. Behavioral variant FTD (bvFTD) accounts for about half of all FTD cases, and involves initial and progressive decline in social functioning and changes in personality. ![]() Currently, FTD incorporates three clinical subtypes. First described by Arnold Pick in 1892, FTD affects brain regions implicated in motivation, reward processing, personality, social cognition, attention, executive functioning and language. FTD defines a heterogeneous group of clinical syndromes marked by the progressive, focal neurodegeneration of the frontal and anterior temporal lobes. In what follows, we review each of the FTD syndromes, highlight current research investigating the cognitive, behavioral and socio-emotional deficits observed with this disease, address common diagnostic challenges and summarize best practices associated with management of FTD.įrontotemporal dementia (FTD) is the third most common dementia for individuals 65 years and older, and is the second most common form for individuals 65 years and younger. Comprehensive clinical assessment in combination with evaluation of language, socio-emotional functioning, cognition and neuroimaging aid in accurate and early diagnosis and treatment planning. While neuropsychiatric symptom measures are helpful, more sensitive assessments delineating the specific behavioral and linguistic deficits accompanying FTD are needed. Psychiatric prodrome, neuropsychiatric symptoms and language difficulties are common in FTD, but the diversity of presentation raises unique diagnostic challenges that can significantly impact patient care and counsel for caregivers regarding clinical status and prognosis. Frontotemporal dementia (FTD) is a progressive neurologic syndrome with diverse clinical presentations and attendant underlying pathologies.
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