OBJECTIVE While patients with primary progressive aphasia (PPA) typically present with predominant language impairment, behavioural symptoms, such as apathy, are often under-recognised. We aimed to systematically characterise apathy across the three recognised subtypes of PPA, plus atypical right-lateralised presentations of semantic dementia, and to evaluate the impact of apathy on carer burden and psychological wellbeing. METHODS Baseline assessments from 114 PPA patients were included: 31 left semantic dementia (left SD) 16 right semantic dementia (right SD), 30 progressive nonfluent aphasia (PNFA) and 37 logopenic progressive aphasia (LPA). Clinician (Neuropsychiatric Inventory; NPI) and carer rated (Cambridge Behavioural Inventory; CBI, Frontal Systems Behaviour Scale; FrSBe) measures were used to quantify symptoms of apathy, and carer burden and psychological wellbeing were determined using the Zarit Burden Interview and Depression, Anxiety and Stress Scale. RESULTS On the NPI, symptoms of apathy were present in 39% left SD, 56% right SD, 33% PNFA and 43% LPA patients. Multiple regression analysis revealed that 17.9% of the variance in carer burden was uniquely explained by scores on carer rated measures of apathy (CBI, FrSBe), even after accounting for diagnosis, disease duration, and cognitive and language impairment. CONCLUSIONS Apathy is much more common than current diagnostic criteria for PPA would suggest, though the severity of apathy is similar across patient groups. Increased awareness and routine assessments of apathy symptoms are needed, together with targeted pharmacological and behavioural interventions. Moreover, as apathy substantially contributes to carer burden in PPA, psychoeducation addressing behavioural symptoms may be beneficial.

中文翻译：

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冷漠及其对原发性进行性失语症照顾者负担和心理健康的影响

目的 虽然原发性进行性失语症 (PPA) 患者通常表现为主要的语言障碍，但行为症状（如冷漠）往往未被充分认识。我们旨在系统地描述三种公认的 PPA 亚型的冷漠，以及语义性痴呆的非典型右偏侧表现，并评估冷漠对照顾者负担和心理健康的影响。方法 包括来自 114 名 PPA 患者的基线评估：31 名左侧语义性痴呆（左侧 SD）、16 名右侧语义性痴呆（右侧 SD）、30 名进行性非流畅性失语症（PNFA）和 37 名语言减少性进行性失语症（LPA）。临床医生（神经精神病学量表；NPI）和护理人员评级（剑桥行为量表；CBI，额叶系统行为量表；FrSBe）用于量化冷漠症状，使用 Zarit 负担访谈和抑郁、焦虑和压力量表确定照顾者的负担和心理健康。结果 在 NPI 中，39% 的左侧 SD、56% 的右侧 SD、33% PNFA 和 43% LPA 患者存在冷漠症状。多元回归分析显示，即使在考虑了诊断、疾病持续时间以及认知和语言障碍之后，照料者负担的 17.9% 的差异可以通过照料者评价的冷漠量度（CBI、FrSBe）的分数来唯一解释。结论 冷漠比目前的 PPA 诊断标准所建议的要普遍得多，尽管冷漠的严重程度在患者组中相似。需要提高对冷漠症状的认识和常规评估，以及有针对性的药理学和行为干预。而且，