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Empirically derived from review of 35 pathologically confirmed cases; three diagnostic categories (A,B,C) requiring one, two, or three of hallucinations, unspecified Parkinsonism, fluctuating course, or rapid progression.

PDD, Parkinsonism disease and dementia; DLB, dementia with Lewy bodies.

(Adapted with permission from Litvan I, Bhatia KP, Burn DJ, et al. SIC Task Force appraisal of clinical diagnostic criteria for Parkinsonian disorders. Mov Dis 2003;18:467-486.)

PDD, Parkinsonism disease and dementia; DLB, dementia with Lewy bodies.

(Adapted with permission from Litvan I, Bhatia KP, Burn DJ, et al. SIC Task Force appraisal of clinical diagnostic criteria for Parkinsonian disorders. Mov Dis 2003;18:467-486.)

gene is located on chromosome 17. Because many other disorders have been associated with abnormalities in tau expression, it has been suggested that the clinical spectrum of FTD may involve disorders such as progressive supranuclear palsy and corticobasal degeneration. In AD, tau is associated with neurofibrillary tangles, implying a classification of the dementias based on understanding of molecular pathology may be forthcoming in the future.

FTD has two major clinical presentations. The most common form is a behavioral syndrome. Individuals develop early changes in social and personal functioning. Symptoms may include disinhibition, impulsive and inappropriate behavior, and breakdown of social conventions. There may be stereotyped or repetitive actions admixed with these aforementioned behaviors. Memory may be affected, but tends not to be (clinically) the most significant abnormality.

In the language variant, individuals with FTD may develop expressive language dysfunction, with frequent anomia. Problems in reading comprehension and written expression may follow. Eventually, such patients may be mute. Other patients may have pronounced difficulty with naming and verbal comprehension. Patients with FTD may also develop motor abnormalities including a motor neuron disease syndrome or Parkinsonism.

Possibly because the full extent of FTD is still evolving, several sets of diagnostic criteria have been proposed. McKhann et al. have proposed relatively simple clinical criteria, which are shown in Table 50. The same work group also described five basic patterns of neuropathological change, and gave appropriate neurological differential diagnosis for each type, summarized in Table 51. (Note that the wide variety of syndromic names and varying neuropathology may make identification of a patient's syndrome obscure even in well-studied cases. Despite the wide-ranging phenotypes, in a mixed dementia population [autopsy-defined], there was excellent inter-rater reliability of the FTD diagnostic criteria.)

The current criteria (Table 50) contrasts with the comprehensive criteria proposed by an international consortium (Tables 52-55). The Appendix contains explanations of the terms used in Table 55.

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