Neurology Clinic, Clinical Center of Serbia, University of Belgrade, School of Medicine, Dr Subotica 6, Belgrade, 11000, Serbia.
Institute of Mental Health, University of Belgrade, School of Medicine, Belgrade, Serbia.
J Neurol. 2018 Jan;265(1):82-88. doi: 10.1007/s00415-017-8667-9. Epub 2017 Nov 15.
The fixed dystonia phenotype was originally established as a prototype of functional dystonia. Nevertheless, in recent reports different functional dystonia phenotypes have been recognized with dystonic movement comprising phasic instead of tonic contraction.
To examine clinical characteristic in all patients with dystonia who fulfilled the criteria for functional movement disorders irrespective of phenotype in an attempt to determine parameters of clinical presentations that might impact the disease progression pattern and outcome.
Patients presented with dystonia features incompatible with organic disease without other features required for the diagnosis of functional movement disorders were analyzed and prospectively followed-up. The two-step cluster analysis was performed to obtain the subgroups of dystonia phenotypes.
The two-step cluster analysis extracted two subgroup of patients. Patients of the first cluster (68.8%) presented with "mobile" dystonia (84.9%), of cranial/neck/trunk localization (90.9%), fluctuated clinical course (69.7%), with frequent additional movement or other functional neurological disorders (63.6%) during follow-up. In the second cluster (31.2%) all of the patients presented with "fixed" dystonia of extremities, and the clinical course was characterized by either the disease progression (60%), or continuous without improvement (26.7%), and rare occurrence of additional functional neurological disorders (13.3%).
In terms of clinical and demographic features as well as pattern of disease progression there are two clinical phenotypes in patients with functional dystonia. Distinctive features of incongruence and inconstancy are characteristic for "mobile" functional dystonia subgroup of patients.
固定性肌张力障碍表型最初被确立为功能性运动障碍的原型。然而，在最近的报告中，已经认识到不同的功能性运动障碍表型，其特征是痉挛性运动而不是强直性收缩。
检查所有符合功能性运动障碍标准但无论表型如何的肌张力障碍患者的临床特征，试图确定可能影响疾病进展模式和结果的临床表现参数。
分析了以不伴有其他功能性运动障碍诊断所需特征的运动障碍为特征但不符合器质性疾病的患者，并对其进行前瞻性随访。采用两步聚类分析获得肌张力障碍表型的亚组。
两步聚类分析提取了两个亚组患者。第一组患者（68.8%）表现为“移动性”肌张力障碍（84.9%），头部/颈部/躯干定位（90.9%），波动性临床病程（69.7%），随访期间经常出现其他运动或其他功能性神经障碍（63.6%）。在第二组（31.2%）中，所有患者均表现为四肢“固定性”肌张力障碍，其病程特征为疾病进展（60%）或持续无改善（26.7%），且罕见发生其他功能性神经障碍（13.3%）。
就临床和人口统计学特征以及疾病进展模式而言，功能性运动障碍患者存在两种临床表型。不协调性和不稳定性的特征是“移动性”功能性运动障碍亚组患者的特征。
