Division of Neurology, Department of Clinical Neurosciences, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada.
Division of Neurology, Department of Medicine, University of British Columbia, Vancouver, BC, Canada.
Can J Neurol Sci. 2024 Jul;51(4):482-486. doi: 10.1017/cjn.2023.268. Epub 2023 Aug 9.
Carpal tunnel syndrome (CTS) is one of the most common extra-cardiac manifestations of wild-type transthyretin amyloidosis (wtATTR); however, the characteristics of CTS in this population remain poorly understood.
This retrospective cohort study reports findings from a single-centre experience of comprehensive neurological screening at the time of wtATTR diagnosis by nerve conduction studies (NCS) and neurologist assessment.
Seventy-nine patients underwent neurological screening, 73 (92%) males, mean age 79.2 ± 7.5 years. Seventy-four (94%) had electrodiagnostic findings of median neuropathy at the wrist (MNW), 37 (50%) of which had a prior diagnosis of CTS and 37 (50%) had a new diagnosis of MNW. Over half of wtATTR patients (42, 53%) had bilateral MNW on screening. Most with pre-existing CTS had bilateral disease (28, 76%) and underwent bilateral carpal tunnel release (CTR) (23, 62%) prior to screening. Twenty-one (19%) wrists had mild MNW, 43 (38%) moderate and 49 (43%) severe. Twenty-one (28%) wtATTR patients with MNW were asymptomatic, 10 of which (48%) had moderate disease. Nineteen (36%) wtATTR patients with symptomatic MNW had recurrent disease despite previous CTR. As a result of screening, 36 (68%) patients with symptomatic MNW were referred for CTR.
MNW is exceptionally common at the time of wtATTR diagnosis, affecting 94% of our patients. Most had severe, bilateral MNW on NCS. Some were asymptomatic, despite having moderate disease. The rate of recurrence following CTR was observed to be higher in wtATTR patients than the general population.
腕管综合征（CTS）是野生型转甲状腺素蛋白淀粉样变性（wtATTR）最常见的心脏外表现之一；然而，该人群的 CTS 特征仍知之甚少。
本回顾性队列研究报告了通过神经传导研究（NCS）和神经科医生评估在 wtATTR 诊断时进行全面神经筛查的单中心经验结果。
79 例患者接受了神经筛查，73 例（92%）为男性，平均年龄 79.2±7.5 岁。74 例（94%）存在腕部正中神经病变（MNW）的电诊断结果，其中 37 例（50%）有 CTS 的既往诊断，37 例（50%）有新诊断的 MNW。超过一半的 wtATTR 患者（42 例，53%）在筛查时存在双侧 MNW。大多数有既往 CTS 的患者（28 例，76%）存在双侧疾病，且在筛查前均接受了双侧腕管松解术（CTR）（23 例，62%）。21 例（19%）腕部 MNW 为轻度，43 例（38%）为中度，49 例（43%）为重度。21 例（28%）有 MNW 但无症状的 wtATTR 患者中，有 10 例（48%）为中度疾病。19 例（36%）有症状性 MNW 的 wtATTR 患者尽管之前进行了 CTR，但疾病仍复发。由于筛查，36 例（68%）有症状性 MNW 的患者被转诊接受 CTR。
在 wtATTR 诊断时，MNW 异常常见，影响了我们 94%的患者。大多数患者的 NCS 显示严重的双侧 MNW。一些患者无症状，尽管患有中度疾病。在 wtATTR 患者中观察到 CTR 后的复发率高于一般人群。
