Huntington's Disease Centre, UCL Queen Square Institute of Neurology, University College London, London, United Kingdom.
The National Hospital for Neurology and Neurosurgery (NHNN), Queen Square, London, United Kingdom.
Mov Disord Clin Pract. 2024 Aug;11(8):998-1007. doi: 10.1002/mdc3.14130. Epub 2024 Jun 9.
Clinically assisted nutrition and hydration via percutaneous endoscopic gastrostomy (PEG) is a therapeutic option to ameliorate the difficulties associated with enhanced catabolism, weight loss, and dysphagia in Huntington's disease (HD).
The objective is to provide insights into demographics, staging (Shoulson-Fahn), complications, weight trajectories, and survival rates in people with HD (pwHD) who underwent PEG.
This retrospective study included 705 consecutive pwHD who attended our HD clinic between July 2006 and March 2024, of whom 52 underwent PEG. A control group (n = 52), comprising pwHD without PEG, were closely matched for sex, stage, age, CAG length, and disease burden score at PEG. The study was registered as a service evaluation at the National Hospital for Neurology and Neurosurgery.
PEG prevalence was 15.0% (n = 52/347) among manifest pwHD: 4.8% (n = 3/62) for Stage 3; 33.3% (n = 16/48) for stage 4; and 44.1% (n = 30/68) for stage 5. Commonest indications were dysphagia, weight loss, and inadequate oral intake. Complications included chest infection, tube dislodgement, and peristomal and skin infections. Modeling of weight trajectories after PEG found no difference between PEG and non-PEG groups. Mortality rate was 34.6% (n = 18/52) in the PEG and 36.5% (n = 19/52) in the non-PEG groups (P = 0.84). Treatment duration (until study endpoint or death) was 3.48 years (interquartile range = 1.71-6.02; range = 0.23-18.8), with 65.4% (n = 34/52) alive at the study endpoint.
PEG in pwHD at-risk for weight loss may help slow weight loss. Prospective studies are required to strengthen PEG decision-making in pwHD. PEG survival was much longer than other dementias, highlighting the need to consider PEG independently in pwHD.
经皮内镜胃造口术（PEG）辅助临床营养和水合作用是一种治疗选择，可以改善亨廷顿病（HD）患者与增强的分解代谢、体重减轻和吞咽困难相关的困难。
本研究旨在提供有关接受 PEG 的 HD 患者（pwHD）的人口统计学、分期（Shoulson-Fahn）、并发症、体重轨迹和生存率的见解。
这项回顾性研究纳入了 2006 年 7 月至 2024 年 3 月期间在我们的 HD 诊所就诊的 705 例连续 pwHD，其中 52 例接受了 PEG。对照组（n=52）由没有接受 PEG 的 pwHD 组成，在性别、分期、年龄、CAG 长度和 PEG 时的疾病负担评分方面与接受 PEG 的 pwHD 进行了密切匹配。该研究在国立神经病学与神经外科学院作为服务评估进行了注册。
在表现出的 pwHD 中，PEG 的患病率为 15.0%（n=52/347）：3 期为 4.8%（n=3/62）；4 期为 33.3%（n=16/48）；5 期为 44.1%（n=30/68）。最常见的指征是吞咽困难、体重减轻和口服摄入不足。并发症包括胸腔感染、管移位、造口周围和皮肤感染。PEG 后体重轨迹的建模发现 PEG 组和非 PEG 组之间没有差异。PEG 组的死亡率为 34.6%（n=18/52），非 PEG 组为 36.5%（n=19/52）（P=0.84）。治疗持续时间（直到研究终点或死亡）为 3.48 年（四分位距=1.71-6.02；范围=0.23-18.8），研究终点时 65.4%（n=34/52）仍存活。
在有体重减轻风险的 pwHD 中进行 PEG 可能有助于减缓体重减轻。需要进行前瞻性研究来加强 pwHD 中进行 PEG 的决策。PEG 的生存率远高于其他痴呆症，这突出表明需要在 pwHD 中独立考虑 PEG。
