Iranzo Alex, Stefani Ambra, Serradell Monica, Martí Maria Jose, Lomeña Francisco, Mahlknecht Philipp, Stockner Heike, Gaig Carles, Fernández-Arcos Ana, Poewe Werner, Tolosa Eduard, Högl Birgit, Santamaria Joan
From the Neurology Service (A.I., M.S., M.J.M., C.G., A.F.-A., E.T., J.S.) and Nuclear Medicine Service (F.L.), Hospital Clinic de Barcelona, IDIBAPS, CIBERNED, Spain; and Department of Neurology (A.S., P.M., H.S., W.P., B.H.), Medical University Innsbruck, Austria.
Neurology. 2017 Jul 18;89(3):242-248. doi: 10.1212/WNL.0000000000004121. Epub 2017 Jun 14.
To evaluate the presence of prodromal markers of Parkinson disease (PD) in patients with longstanding idiopathic REM sleep behavior disorder (IRBD), a small subgroup of individuals with IRBD with long-term follow-up thought not to be at risk of developing PD.
Demographic, clinical, and neuroimaging markers of PD were evaluated in 20 patients with polysomnographic-confirmed longstanding IRBD and in 32 matched controls.
Patients were 16 men and 4 women with mean age of 72.9 ± 8.6 years and mean follow-up from IRBD diagnosis of 12.1 ± 2.6 years. Patients more often had objective smell loss (35% vs 3.4%, = 0.003), constipation (50% vs 15.6%, = 0.008), and mild parkinsonian signs (45% vs 18.8%, = 0.042) than controls. Unified Parkinson's Disease Rating Scale motor score was higher in patients than in controls (5.6 ± 3.5 vs 2.0 ± 2.1, < 0.0001). Dopamine transporter imaging showed decreased striatal uptake in 82.4% of the patients and transcranial sonography found substantia nigra hyperechogenicity in 35.3%. α-Synuclein aggregates were found in 3 of 6 patients who underwent colon or submandibular gland biopsies. All 20 patients showed clinical, neuroimaging, or histologic markers of PD. Probability of prodromal PD (according to recent Movement Disorders Society research criteria) was higher in patients than in controls (<0.0001), and 45% of patients surpassed 80% probability.
Prodromal PD markers are common in individuals with longstanding IRBD, suggesting that they are affected by an underlying neurodegenerative process. This observation may be useful for the design of disease-modifying trials to prevent PD onset in IRBD.
评估长期特发性快速眼动睡眠行为障碍（IRBD）患者中帕金森病（PD）前驱标志物的存在情况，IRBD的一个小亚组经长期随访被认为无发生PD的风险。
对20例经多导睡眠图证实的长期IRBD患者和32例匹配对照进行PD的人口统计学、临床和神经影像学标志物评估。
患者中男性16例，女性4例，平均年龄72.9±8.6岁，从IRBD诊断开始的平均随访时间为12.1±2.6年。与对照组相比，患者更常出现客观嗅觉减退（35%对3.4%，P=0.003）、便秘（50%对15.6%，P=0.008）和轻度帕金森体征（45%对18.8%，P=0.042）。患者的统一帕金森病评定量表运动评分高于对照组（5.6±3.5对2.0±2.1，P<0.0001）。多巴胺转运体成像显示82.4%的患者纹状体摄取减少，经颅超声检查发现35.3%的患者黑质回声增强。在6例接受结肠或颌下腺活检的患者中，有3例发现α-突触核蛋白聚集。所有20例患者均显示出PD的临床、神经影像学或组织学标志物。患者前驱PD的概率（根据最近运动障碍协会的研究标准）高于对照组（P<0.0001），45%的患者超过80%的概率。
前驱PD标志物在长期IRBD患者中很常见，表明他们受到潜在神经退行性过程的影响。这一观察结果可能有助于设计疾病修饰试验以预防IRBD患者发生PD。
