Bhattacharyya K B, Basu S, Roy A D, Bhattacharya S
Department of Neurology, Calcutta National Medical College and Hospital, India.
Neurol India. 2003 Mar;51(1):91-3.
Orthostatic tremor is a rare movement disorder characterized by tremulousness of the lower limbs on standing that disappears on walking, sitting or on lying down and a distinctive electromyographic burst of 14 to 16 Hz. On inspection, fine ripples can sometimes be seen over the quadriceps on standing. The tremor has a tendency to reappear even in the supine posture if the lower limb muscles are put to an isometric contraction state, indicating thereby that in spite of the fact that the tremor occurs on standing, it is essentially 'orthostasis independent' and the central factor is the contraction of the muscles. As a matter of fact, the tremor is abolished if the subject is suspended by harness, thus relieving him of muscle contraction. Doubts are being cast whether it is a variant of essential tremor since a number of families are being reported to be suffering from this disease as well. Positron emission tomography reveals hyperactivity of cerebellum in orthostatic tremor as it shows in essential tremor, therefore lending credence to such a hypothesis. However, lack of response to alcohol, propranolol and primidone in orthostatic tremor stands out as a serious challenge to such a view. Lack of positive family history, synchrony of contracting group of muscles and negative 'resetting' of the tremor by increasing peripheral load-phenomena consistently observed in orthostatic tremor and not in essential tremor, are other features that often help to distinguish between the two conditions. We report a case of orthostatic tremor that presented with the classical clinical and electromyographic features. Relevant literature in this regard is also being reviewed.
直立性震颤是一种罕见的运动障碍,其特征为站立时下肢震颤,行走、坐下或躺下时消失,以及有特征性的14至16赫兹肌电图爆发。检查时,站立时有时可在股四头肌上看到细微波动。如果下肢肌肉处于等长收缩状态,即使在仰卧姿势下震颤也有再次出现的倾向,这表明尽管震颤在站立时出现,但本质上是“与直立无关的”,核心因素是肌肉收缩。事实上,如果受试者用吊带悬吊,震颤就会消失,从而解除其肌肉收缩。由于也有一些家庭被报道患有这种疾病,因此有人质疑它是否是特发性震颤的一种变体。正电子发射断层扫描显示直立性震颤时小脑活动亢进,如同在特发性震颤中所见,因此支持了这样一种假说。然而,直立性震颤对酒精、普萘洛尔和扑米酮无反应,这对这种观点构成了严峻挑战。缺乏阳性家族史、收缩肌群的同步性以及通过增加外周负荷使震颤出现阴性“重置”现象(在直立性震颤中持续观察到而在特发性震颤中未观察到),是其他常有助于区分这两种情况的特征。我们报告一例具有典型临床和肌电图特征的直立性震颤病例。同时也对这方面的相关文献进行综述。