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Ebook Clinical manual of alzheimer disease and other dementias: Part 2

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Ebook Clinical manual of alzheimer disease and other dementias: Part 2

Frontotemporal Dementia and Other TauopathiesAnne M. Lipton, M.D., Ph.D.Adam Boxer, M.D., Ph.D.I n its broadest sense, the term frontotemporal dementi

Ebook Clinical manual of alzheimer disease and other dementias: Part 2ia (FTD) refers to a number of neurodegenerative diseases that vary in clinical presentation and pathological findings. FID is also known as frontotem

poral lobar degeneration (FTLD) (Neary et al. 1998). The clinical and research nosology for this disease continue to evolve and sometimes create contr Ebook Clinical manual of alzheimer disease and other dementias: Part 2

oversy or confusion. Frontal-variant FTD (fvFTD) refers to rhe specific F I D clinical subtype characterized by executive dysfunction and apathy. Alth

Ebook Clinical manual of alzheimer disease and other dementias: Part 2

ough the clinical syndromes vary, they characteristically involve problems with language, behavior, and/or motor findings, such220 Clinical Manual of

Frontotemporal Dementia and Other TauopathiesAnne M. Lipton, M.D., Ph.D.Adam Boxer, M.D., Ph.D.I n its broadest sense, the term frontotemporal dementi

Ebook Clinical manual of alzheimer disease and other dementias: Part 2ques, has led to remarkable advances.HistoryThe archetypal FTD is Pick disease, first clinically delineated by Arnold Pick (1892), who described langu

age impairments and behavioral disturbances in the setting of focal brain atrophy. Alois Alzheimer (1911) provided rhe first histopathological descrip Ebook Clinical manual of alzheimer disease and other dementias: Part 2

tion of Pick disease with argyrophilic inclusions (later called Pick bodies) and swollen, achromatic cells (later called Pick cells). The Lund-Manches

Ebook Clinical manual of alzheimer disease and other dementias: Part 2

ter criteria (Lund and Manchester Groups 1994) delineated the clinical features of FTD; these criteria were later refined by a consensus panel that us

Frontotemporal Dementia and Other TauopathiesAnne M. Lipton, M.D., Ph.D.Adam Boxer, M.D., Ph.D.I n its broadest sense, the term frontotemporal dementi

Ebook Clinical manual of alzheimer disease and other dementias: Part 2 2001).FTD occurs, on average, in individuals in their 50s and may be the most common cause of dementia in this age group (Knopman er al. 2004). Onset

before age 65 years is one of the clinical diagnostic criteria for FTD (Neary et al. 1998).Clinical Subtypes of FTDPatients with FTD present with the Ebook Clinical manual of alzheimer disease and other dementias: Part 2

insidious onset of a behavioral syndrome or a language variant. FTD progresses gradually, but survival is generally shorter than for Alzheimer diseas

Ebook Clinical manual of alzheimer disease and other dementias: Part 2

e. Hodges et al. (2003) reported that median survival from symptom onset and from diagnosis was about 6 years for fvFTD and about 3 years for FTD asso

Frontotemporal Dementia and Other TauopathiesAnne M. Lipton, M.D., Ph.D.Adam Boxer, M.D., Ph.D.I n its broadest sense, the term frontotemporal dementi

Ebook Clinical manual of alzheimer disease and other dementias: Part 2and problems with social conduct and interpersonal skills associated with abnormalities of the right frontotemporal lobe on neuroimaging (Mychack et a

l. 2001). Lack of insight is a hallmark of the fvFTD subtype. Patients are often impulsive and oblivious to societal or other limitations on -.1. •.. Ebook Clinical manual of alzheimer disease and other dementias: Part 2

.•. ..... I •. I. . . j:.JJ . .1I.r... ..-I.....Frontotemporal Dementia and other Tauopathies 221An individual with fvFTD may display disinhibition, a

Ebook Clinical manual of alzheimer disease and other dementias: Part 2

pathy, or both. Patients with orbitofrontal dysfunction are more “disagreeable” and less modest and altruistic (Rankin et al. 2004). Damage in the ven

Frontotemporal Dementia and Other TauopathiesAnne M. Lipton, M.D., Ph.D.Adam Boxer, M.D., Ph.D.I n its broadest sense, the term frontotemporal dementi

Ebook Clinical manual of alzheimer disease and other dementias: Part 2 have some aspects of a Kliiver-Bucy syndrome, including eating (or drinking) to excess, with an emphasis on carbohydrate-laden junk food.Primary Prog

ressive AphasiaPatients with a language variant of FTD—either progressive nonfluent aphasia or semantic dementia—frequently have one or more extensive Ebook Clinical manual of alzheimer disease and other dementias: Part 2

evaluations for stroke due to their aphasia. The aphasia worsens, and they may become mute. Some also develop behaviors similar to those seen in fvFT

Ebook Clinical manual of alzheimer disease and other dementias: Part 2

D or in motor dysfunctions such as amyotrophic lateral sclerosis (ALS) or parkinsonism.Artistic abilities often manifest in patients with a language v

Frontotemporal Dementia and Other TauopathiesAnne M. Lipton, M.D., Ph.D.Adam Boxer, M.D., Ph.D.I n its broadest sense, the term frontotemporal dementi

Ebook Clinical manual of alzheimer disease and other dementias: Part 2or as a modification of a skill previously evident in an individual.Progressive Nonfluetit AphasiaProgressive nonfluent aphasia involves expressive ap

hasia with word finding difficulty, agrammatism, and phonemic paraphasias. Unlike patients with the other forms of FTD, patients with progressive nonf Ebook Clinical manual of alzheimer disease and other dementias: Part 2

luent aphasia usually have little functional or behavioral impairment until late in their disease.Semantic DementiaSemantic dementia, also called the

Ebook Clinical manual of alzheimer disease and other dementias: Part 2

temporal lobe variant of FTD, is caused by a progressive loss of information about the world and is associated with degeneration of the anterior tempo

Frontotemporal Dementia and Other TauopathiesAnne M. Lipton, M.D., Ph.D.Adam Boxer, M.D., Ph.D.I n its broadest sense, the term frontotemporal dementi

Ebook Clinical manual of alzheimer disease and other dementias: Part 2meaning of words) and an associative agnosia (e.g., difficulty in stating or demonstrating the function of an object, such as a tool or utensil) in in

dividuals with more left temporal lobe involvement. Prosopagnosia (inability to recognize faces) may rarely occur and is associated with right tempora Ebook Clinical manual of alzheimer disease and other dementias: Part 2

l lobe damage. More commonly, behavioral problems similar to those in222 Clinical Manual of Alzheimer Disease and other DementiasOverlap of FTD Clinic

Ebook Clinical manual of alzheimer disease and other dementias: Part 2

al SyndromesBecause the three FTD clinical syndromes often overlap (as can be seen in some of the above examples), and because they may also overlap w

Frontotemporal Dementia and Other TauopathiesAnne M. Lipton, M.D., Ph.D.Adam Boxer, M.D., Ph.D.I n its broadest sense, the term frontotemporal dementi

Ebook Clinical manual of alzheimer disease and other dementias: Part 2 authors suggest rhe term Pick complex to encompass all of these syndromes.The current consensus clinical criteria for FTD are useful but still lack p

recision. New guidelines are in development. The current clinical criteria fail to account for many neurogenetic and neuroimaging aspects of the diagn Ebook Clinical manual of alzheimer disease and other dementias: Part 2

osis of FTD. Rosen et al. (2002b) found that the Neary er al. (1998) clinical consensus criteria efficiently separated 30 autopsy-proven cases of Alzh

Ebook Clinical manual of alzheimer disease and other dementias: Part 2

eimer disease and 30 autopsy-proven cases of FTLD. They found that the following five clinical features best distinguished FTLD from Alzheimer disease

Frontotemporal Dementia and Other TauopathiesAnne M. Lipton, M.D., Ph.D.Adam Boxer, M.D., Ph.D.I n its broadest sense, the term frontotemporal dementi

Ebook Clinical manual of alzheimer disease and other dementias: Part 2 number of diseases overlap clinically and pathologically with FTD, including motor neuron disease/ALS, corticobasal syndrome, and PSP.Motor Neuron Di

sease/Amyotrophic Lateral SclerosisOf 100 ALS patients studied prospectively with extensive neuropsychological assessment, about one-third met criteri Ebook Clinical manual of alzheimer disease and other dementias: Part 2

a for FTLD (Lomen-Hoerth et al. 2003). Many patients clinically diagnosed with FTLD have motor neurontype inclusions on histopathology, either with or

Ebook Clinical manual of alzheimer disease and other dementias: Part 2

without clinical motor neuron disease (Bigio et al. 2003). Moreover, both chronic traumatic encephalopathy and FTLD may include TAR-DNA binding prote

Frontotemporal Dementia and Other TauopathiesAnne M. Lipton, M.D., Ph.D.Adam Boxer, M.D., Ph.D.I n its broadest sense, the term frontotemporal dementi

Ebook Clinical manual of alzheimer disease and other dementias: Part 2y associated with motor neuron disease (McKee et al. 2010).Corticobasal SyndromeCorticobasal syndrome \s the current nomenclature used to describe the

unifyingFrontotemporal Dementia and other Tauopathies 223 Ebook Clinical manual of alzheimer disease and other dementias: Part 2

Frontotemporal Dementia and Other TauopathiesAnne M. Lipton, M.D., Ph.D.Adam Boxer, M.D., Ph.D.I n its broadest sense, the term frontotemporal dementi

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