Mastain B, Cassim F, Guieu J D, Destée A
Service de Neurologie A, Hôpital Roger Salengro, Universitaire de Lille.
Rev Neurol (Paris). 1998 May;154(4):322-9.
Orthostatic tremor is an unusual kind of potentially disabiling tremor appearing immediately when standing. Clinical examination is normal in primary form except for wide base standing and unsteadiness which disappear when walking. Arm tremor resembling essential tremor is found present in one third of cases. Electrophysiological exploration is necessary for diagnosis and shows a regular rapid tremor (frequency around 16 Hz). We present 10 new cases, 3 men and 7 women, 37 to 74 years old. Unsteadiness when standing was the predominant complaint in 9 cases, the other first described pains in the lower limbs. All had visible or palpable tremor predominant in thighs. Four patients had postural arm tremor, one had neurogen syndrome in the lower limbs corresponding to toxic polyneuropathy which developed after tremor. Electromyographic study found high frequency (13-17.3 Hz) rhythmic discharge in weight-bearing muscles. Orthostatic tremor cannot be considered as a clinical variant of postural essential tremor. Its pathophysiology is unknown but the efficacy of clonazepam, primidone or barbiturates suggests the impairment of the gabaergic system.
直立性震颤是一种特殊的、可能导致残疾的震颤,站立时立即出现。原发性直立性震颤除了站立时双脚分开较宽和行走时消失的步态不稳外,临床检查通常正常。三分之一的病例会出现类似特发性震颤的手臂震颤。诊断需要进行电生理检查,结果显示有规律的快速震颤(频率约为16赫兹)。我们报告10例新病例,3名男性和7名女性,年龄在37至74岁之间。9例患者以站立不稳为主要症状,另1例最初描述为下肢疼痛。所有患者大腿均有明显或可触及的震颤。4例患者有姿势性手臂震颤,1例下肢出现与中毒性多发性神经病相符的神经源性综合征,该综合征在震颤后出现。肌电图研究发现负重肌肉中有高频(13 - 17.3赫兹)节律性放电。直立性震颤不能被视为姿势性特发性震颤的临床变异型。其病理生理学尚不清楚,但氯硝西泮、扑米酮或巴比妥类药物的疗效提示γ-氨基丁酸能系统受损。