Russell Angela, Hahn Christopher, Chhibber Sameer, Korngut Lawrence, Fine Nowell M
Division of Neurology, Department of Clinical Neurosciences, University of Calgary, Calgary, Alberta, Canada.
Division of Cardiology, Department of Cardiac Sciences, Libin Cardiovascular Institute, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.
Can J Neurol Sci. 2021 Sep;48(5):607-615. doi: 10.1017/cjn.2020.271. Epub 2020 Dec 21.
Wild-type transthyretin amyloidosis (wtATTR) is an important cause of heart failure (HF); however, the prevalence and clinical significance of neurologic complications remains uncertain.
This analysis reports findings from a single-centre experience of routine neuropathy screening at the time of wtATTR diagnosis by nerve conduction studies and neurologist assessment, compared with age-matched controls.
Forty-one wtATTR patients were included, 39 (95%) males, mean age 78.4 ± 7.7 years, 22 (54%) New York Heart Association (NYHA) class III-IV HF, along with 15 age-matched controls (mean age 77.1 ± 4.2 years, 80% male). Twenty-one (51%) wtATTR patients were diagnosed with polyneuropathy, 15 (37%) with spinal stenosis, 36 (88%) with carpal tunnel syndrome (CTS) and 14 (34%) with ulnar neuropathy. Comparison diagnoses among controls were 1 (7%), 0, 1 (7%) and 3 (20%), respectively. Among patients with NYHA class III-IV HF, 16 (73%) had polyneuropathy compared with 5 (26%) with class I-II (p < 0.01), odds ratio of 7.5 (95% confidence interval 1.9-29.9). After neuropathy screening, 19 (46%) patients were offered neurologic therapy and/or additional diagnostic evaluation. This included CTS release surgery (16, 39%), neuropathic pain medication (3, 7%), nerve block (1, 2%), wrist splinting (2, 5%) and foot care (1, 2%). Spine imaging was performed for 3 (7%) patients, and deltoid muscle and sural nerve biopsy for 1 (2%) patient.
Screening of wtATTR patients for neurologic complications resulted in a management change for nearly half. CTS, polyneuropathy and ulnar neuropathy were common. This approach warrants consideration as part of routine assessment for newly diagnosed wtATTR patients.
野生型转甲状腺素蛋白淀粉样变性（wtATTR）是心力衰竭（HF）的一个重要病因；然而，神经并发症的患病率及临床意义仍不明确。
本分析报告了一项单中心经验，即在wtATTR诊断时通过神经传导研究和神经科医生评估进行常规神经病变筛查，并与年龄匹配的对照组进行比较。
纳入41例wtATTR患者，39例（95%）为男性，平均年龄78.4±7.7岁，22例（54%）为纽约心脏协会（NYHA）III-IV级HF，另有15例年龄匹配的对照组（平均年龄77.1±4.2岁，80%为男性）。21例（51%）wtATTR患者被诊断为多发性神经病，15例（37%）为椎管狭窄，36例（88%）为腕管综合征（CTS），14例（34%）为尺神经病变。对照组的相应诊断分别为1例（7%）、0例、1例（7%）和3例（20%）。在NYHA III-IV级HF患者中，16例（73%）有多发性神经病，而I-II级患者中有5例（26%）（p<0.01），比值比为7.5（95%置信区间1.9-29.9）。在进行神经病变筛查后，19例（46%）患者接受了神经治疗和/或进一步诊断评估。这包括CTS松解手术（16例，39%）、神经性疼痛药物治疗（3例，7%）、神经阻滞（1例，2%）、手腕夹板固定（2例，5%）和足部护理（1例，2%）。3例（7%）患者进行了脊柱成像，1例（2%）患者进行了三角肌和腓肠神经活检。
对wtATTR患者进行神经并发症筛查使近半数患者的治疗方案发生了改变。CTS、多发性神经病和尺神经病变很常见。这种方法作为新诊断的wtATTR患者常规评估的一部分值得考虑。
