Neuropsychiatric Symptoms in Behavioral Variant Frontotemporal Dementia and Primary Progressive Aphasia

Abstract

Neuropsychiatric symptoms are well defined in behavioral variant frontotemporal dementia but are not as well studied in primary progressive aphasia. This study compared caregiver reported neuropsychiatric symptoms in these 2 forms of dementia at short and long disease duration. Patients with behavioral variant frontotemporal dementia had more symptoms than patients with primary progressive aphasia. However, when divided by duration of disease, patients with primary progressive aphasia with long duration had a similar number of symptoms to patients with behavioral variant frontotemporal dementia at either duration. Furthermore, this group of patients with primary progressive aphasia had more symptoms typical of behavioral variant frontotemporal dementia and less mood-related symptoms which were more common in patients with primary progressive aphasia with shorter duration. This study illustrates the emergence of neuropsychiatric symptoms as primary progressive aphasia progresses and highlights the increasing overlap with behavioral variant frontotemporal dementia because the disease affects areas outside of the language network. Keywords dementia; neuropsychiatric symptoms; primary progressive aphasia; frontotemporal dementia Neuropsychiatric symptoms are common in the neurodegenerative dementias 1 and are related to specific biochemical and anatomical changes that occur with these diseases. 2 These symptoms include mood disturbances, psychoses, compulsions, anxiety, and agitation, symptoms that constitute a major source of morbidity and caregiver burden. 3,4 Neuropsychiatric symptoms are more prevalent in certain types of dementia. Frontotemporal dementia (FTD) is an umbrella term for a group of dementia syndromes that present clinically without amnesia, especially in the early stages. FTD affects between 4% and 20% of patients presenting to dementia clinics 5 and is found to be most prevalent in individuals below 60 years of age. 6-8 FTD can be broadly divided into 2 clinical categories 9 : a behavioral variant frontotemporal dementia (bvFTD) and an aphasic variant known as primary progressive aphasia (PPA). Neuropsychiatric symptoms are the earliest manifestation in bvFTD and may occur in isolation or in addition to executive dysfunction. Different symptoms of aphasia occur early in PPA 10-13 and remain most prominent over the course of illness. Although the postmortem neuropathological findings are varied in both PPA and bvFTD, they typically affect the prefrontal and anterolateral temporal areas of the brain and are therefore subsumed under the rubric of frontotemporal lobar degeneration (FTLD). PPA refers to the class of clinical dementia syndromes in which aphasia is the earliest and most salient symptom, especially within the first 2 years of symptoms onset. Any type of aphasia can be manifested. 12 PPA has recently been classified into subtypes based on distinctive clinical and neuroimaging features: labelled agrammatic or nonfluent, semantic dementia (SD), and logopenic. [14][15][16][17] Prior t...

Neuropsychiatric Aspects of Primary Progressive Aphasia

“…Night time behavior was not significantly associated with PPA. Our findings are consistent with what had been observed by Banks and Weintraub who were perhaps the first to systematically examine NPS in PPA 13 . They used NPI-Q to measure NPS in PPA (n = 42) then compared it with bvFTD (n = 28).…”

“…Banks and Weintraub compared the frequency of NPS in PPA with that of bvFTD 13, 14 . We conducted a similar study, but we sought to examine as to whether NPS occurs in PPA over and above expected in the general population by conducting a case-control study in which the controls were sampled from an ongoing population-based study.…”

“…Our study is likely to contribute to the budding literature on the neuropsychiatric aspects of PPA. This is important as NPS may be clinical markers for various phases of the underlying neurodegenerative disorder manifesting with PPA and subsequently in PPA-plus 13 .…”

Déficits Primários e Secundários de Funções Executivas Pós-TCE: análise de dissociações

“…Estes dados sugerem que o funcionamento executivo pode estar mediando os prejuízos mnemô-nicos. Outros estudos têm sugerido que o prejuízo em um domínio cognitivo pode mediar o prejuízo de outras funções cognitivas (Borosh & Johnson, 2007;Henry, Rendell, Kliegel, & Altgassen, 2007). O controle inibitório é responsável por inibir estímulos e respostas irrelevantes ao contexto.…”

“…Outra maneira de aprimorar a avaliação dos déficits pode ser com a magnitude do prejuízo encontrado, pois quanto maior for o déficit maiores as probabilidades de que seja primário. Consideram-se déficits primários aqueles atribuídos exclusivamente a um prejuízo na função identificada, enquanto os secundários seriam os que se encontram prejudicados devido ao prejuízo em outra função cognitiva (Borosh & Johnson, 2007).…”