Atee Mustafa, Whiteman Ineka, Lloyd Rebecca, Morris Thomas
The Dementia Centre, HammondCare, Level 2, 302 Selby Street Nth, Osborne Park, WA 6017, Australia.
Sydney Pharmacy School, Faculty of Medicine and Health, The University of Sydney, Sydney, NSW, Australia.
Palliat Care Soc Pract. 2024 Sep 6;18:26323524241273492. doi: 10.1177/26323524241273492. eCollection 2024.
Childhood dementias are a group of rare, fatal neurodegenerative disorders, characterised by global cognitive decline, loss of previously acquired developmental skills and behaviours and psychological symptoms of dementia (BPSD). Batten disease, or neuronal ceroid lipofuscinosis, and Sanfilippo syndrome, or mucopolysaccharidosis type III, are two of the more common forms of childhood dementia disorders worldwide. While psychosocial interventions are the best available therapeutic approach for BPSD management in adult-onset dementia, there is very limited literature or clinical experience in the context of childhood dementia. To address this gap, we conducted a descriptive case analysis of BPSD profiles, associated contributing factors and targeted psychosocial interventions in two cases with childhood dementia disorders (Sanfilippo syndrome and CLN3 (juvenile onset) Batten disease) who were referred to Dementia Support Australia, a national dementia behaviour support service in Australia. Primary BPSD identified in these disorders included physical and verbal aggression and irritability/lability. In these cases, contributing factors to the development of BPSD were not monolithic, encompassing pain, caregiver's approach and over or under-stimulation. Improvement in BPSD were observed using the Neuropsychiatric Inventory-Quesionnaire and globally noted as per the qualitative feedback reported by family and caregivers. Person-centred, multimodal psychosocial interventions were recognised as effective therapies in resolving BPSD in these cases. In conclusion, the case studies described the nature and presentation of BPSD in two common forms of childhood dementia and demonstrated the potential benefits of person-centred psychosocial interventions (delivered through national dementia-specific support programs) in alleviating BPSD such as irritability and aggression in these disorders.
儿童期痴呆是一组罕见的致命性神经退行性疾病，其特征为全面认知衰退、丧失先前获得的发育技能和行为以及痴呆的心理症状（BPSD）。巴滕病（即神经元蜡样脂褐质沉积症）和桑菲利波综合征（即Ⅲ型黏多糖贮积症）是全球范围内儿童期痴呆疾病中两种较常见的形式。虽然心理社会干预是成人期痴呆中管理BPSD的最佳可用治疗方法，但在儿童期痴呆方面的文献和临床经验非常有限。为了填补这一空白，我们对两例儿童期痴呆疾病（桑菲利波综合征和CLN3（青少年型）巴滕病）的BPSD概况、相关促成因素和针对性心理社会干预进行了描述性病例分析，这两例患者被转介至澳大利亚痴呆症支持组织，该组织是澳大利亚一家全国性的痴呆行为支持服务机构。在这些疾病中确定的主要BPSD包括身体攻击和言语攻击以及易怒/情绪不稳定。在这些病例中，BPSD发展的促成因素并非单一的，包括疼痛、照顾者的方式以及刺激过度或不足。使用神经精神科问卷观察到BPSD有所改善，并且根据家庭和照顾者报告的定性反馈总体上也有改善。以患者为中心的多模式心理社会干预被认为是解决这些病例中BPSD的有效疗法。总之，这些案例研究描述了两种常见儿童期痴呆形式中BPSD的性质和表现，并证明了以患者为中心的心理社会干预（通过全国性的特定痴呆症支持项目提供）在减轻这些疾病中易怒和攻击等BPSD方面的潜在益处。
