Grandas Francisco
Department of Neurology, Hospital Universitario Gregorio Marañón and Parkinson's Disease and Movement Disorders Unit, Hospital Beata Maria Ana, Madrid, Spain.
Handb Clin Neurol. 2011;100:249-60. doi: 10.1016/B978-0-444-52014-2.00017-3.
Hemiballism is a relatively rare hyperkinetic movement disorder characterized by involuntary, violent, coarse and wide-amplitude movements involving ipsilateral arm and leg. Although classically related to lesions in the subthalamic nucleus, in clinical-radiological series of hemiballism most patients had lesions outside this nucleus, involving mainly other basal ganglia structures. It has been suggested that abnormal neuronal firing patterns in the internal segment of the globus pallidus may be related to the pathogenesis of hemiballism. Stroke is the most common cause, but in recent years an increasing number of patients with hemiballism associated with nonketotic hyperglycemia or with complications of human immunodeficiency virus (HIV) infection have been reported. Contrarily to what was stated in older literature, hemiballism has, in general, a relatively good prognosis. Depending on the underlying causes, many patients may experience spontaneous improvements or remissions. Treatment should be directed to the cause of hemiballism. Symptomatic treatment includes the use of drugs, particularly blockers of striatal D2 dopamine receptors and tetrabenazine. Surgical treatment, especially pallidotomy, is a therapeutic option for the minority of patients with severe persistent disabling hemiballism.
偏身投掷症是一种相对罕见的运动亢进性运动障碍，其特征为涉及同侧手臂和腿部的不自主、剧烈、粗大且幅度较大的运动。虽然传统上与丘脑底核病变有关，但在偏身投掷症的临床放射学系列研究中，大多数患者的病变位于该核团之外，主要累及其他基底神经节结构。有人提出，苍白球内侧段异常的神经元放电模式可能与偏身投掷症的发病机制有关。中风是最常见的病因，但近年来，越来越多与非酮症高血糖或人类免疫缺陷病毒（HIV）感染并发症相关的偏身投掷症患者被报道。与旧文献中的说法相反，偏身投掷症总体预后相对较好。根据潜在病因，许多患者可能会自发改善或缓解。治疗应针对偏身投掷症的病因。对症治疗包括使用药物，特别是纹状体D2多巴胺受体阻滞剂和丁苯那嗪。手术治疗，尤其是苍白球切开术，是少数严重持续性致残性偏身投掷症患者的一种治疗选择。
