Fig 4.
Clinical symptoms and signs distinguish pathological subgroups of frontotemporal dementia (FTD). (A) The percentage of patients and/or informants reporting the presence of the clinical features indicated. When stratified by pathological diagnosis, the presenting symptoms showed patterns that distinguish the various pathological subgroups. Behavioral/social (white bars) = disinhibition, change in personality, apathy, decreased motivation, and psychiatric illness diagnosed later in life near the onset of symptoms; language disorders (striped bars) = naming or word-finding difficulty, decline in speech or grammar; cognitive deficits (black bars) = memory loss, decline in activities of daily living, attention deficits, executive dysfunction, or visuospatial complaints; movement problems (gray bars) = abnormal gait, falling, rigidity, apraxia, or dysarthria. (B, C) The percentage of patients diagnosed at first (B) and last (C) clinical examination in the major pathological subgroups with language (striped bars), behavioral/social (black bars), extrapyramidal (white bars), and/or pyramidal dysfunction (gray bars) is indicated. Although the pattern of clinical signs did not significantly distinguish the pathological subgroups, the presence of both social dysfunction and aphasia at first examination was specific for frontotemporal lobar degeneration with ubiquitin-positive inclusions/dementia lacking distinctive histopathology (FTLD-U/DLDH) compared with Alzheimer’s disease (AD) and tauopathy groups. Extrapyramidal symptoms were more common in the tauopathy subgroups (58%) relative to the FTLD-U/DLDH subgroups (21%), and pyramidal symptoms were frequently identified in FTLD-U/ DLDH patients, although this difference did not reach statistical significance. With disease progression, the incidence of social dysfunction, aphasia, extrapyramidal signs, and pyramidal signs increased in all subgroups to a common status of global impairment in all pathology subgroups.