Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, UCL, Queen Square, London, United Kingdom.
Mov Disord. 2010 Apr 15;25(5):560-9. doi: 10.1002/mds.23019.
Approximately 10% of patients diagnosed clinically with early Parkinson's disease (PD) have normal dopaminergic functional imaging (Scans Without Evidence of Dopaminergic Deficit [SWEDDs]). An important subgroup of SWEDDs are those with asymmetric rest tremor resembling parkinsonian tremor. Clinical and pathophysiological features which could help to distinguish SWEDDs from PD have not been explored. We therefore studied clinical details including non-motor symptoms in 25 tremulous SWEDDs patients in comparison to 25 tremor-dominant PD patients. Blinded video rating was used to compare examination findings. Electrophysiological tremor parameters and also response to a cortical plasticity protocol using paired associative stimulation (PAS) was studied in 9 patients with SWEDDs, 9 with tremor-dominant PD (with abnormal dopamine transporter single photon emission computed tomography findings), 8 with segmental dystonia, and 8 with essential tremor (ET). Despite clinical overlap, lack of true bradykinesia, presence of dystonia, and head tremor favored a diagnosis of SWEDDs, whereas re-emergent tremor, true fatiguing or decrement, good response to dopaminergic drugs, and presence of non-motor symptoms favored PD. A single tremor parameter could not differentiate between groups, but the combination of re-emergent tremor and highest tremor amplitude at rest was characteristic of PD tremor. SWEDDs and segmental dystonia patients exhibited an abnormal exaggerated response to the PAS protocol, in contrast to a subnormal response in PD and a normal response in ET. We conclude that despite clinical overlap, there are features that can help to distinguish between PD and SWEDDs which may be useful in clinical practice. The underlying pathophysiology of SWEDDs differs from PD but has similarities with primary dystonia.
大约 10%临床诊断为早期帕金森病 (PD) 的患者多巴胺能功能成像正常 (无多巴胺能缺陷扫描 [SWEDDs])。SWEDDs 的一个重要亚组是那些具有类似于帕金森震颤的不对称静止性震颤的患者。尚未探索有助于将 SWEDDs 与 PD 区分开来的临床和生理病理特征。因此,我们研究了 25 例震颤 SWEDDs 患者的临床细节,包括非运动症状,并与 25 例以震颤为主的 PD 患者进行了比较。使用盲法视频评分比较了检查结果。在 9 例 SWEDDs 患者、9 例以震颤为主的 PD 患者(多巴胺转运体单光子发射计算机断层扫描发现异常)、8 例节段性肌张力障碍和 8 例特发性震颤患者中研究了电生理震颤参数以及使用配对联想刺激 (PAS) 的皮质可塑性方案的反应。尽管存在临床重叠,但缺乏真正的运动迟缓、存在肌张力障碍和头部震颤有利于 SWEDDs 的诊断,而震颤再现、真性疲劳或减退、对多巴胺能药物的良好反应以及非运动症状的存在有利于 PD 的诊断。单个震颤参数无法区分两组,但震颤再现和静止时最高震颤幅度的组合是 PD 震颤的特征。SWEDDs 和节段性肌张力障碍患者对 PAS 方案表现出异常的过度反应,而 PD 患者的反应低于正常,ET 患者的反应正常。我们得出结论,尽管存在临床重叠,但仍有一些特征可以帮助区分 PD 和 SWEDDs,这在临床实践中可能有用。SWEDDs 的潜在病理生理学与 PD 不同,但与原发性肌张力障碍相似。