Benign tremulous Parkinsonism
Benign tremulous Parkinsonism
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DOI：
10.1001/archneur.63.3.354
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发表时间：
2006-03-01
影响因子：
--
通讯作者：
Ahlskog, JE
中科院分区：
文献类型：
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作者：
Josephs, KA;Matsumoto, JY;Ahlskog, JE
背景：良性震颤性帕金森病从未被精确定义，也没有长期病程的研究。目的：报道我们在运动障碍实践中遇到的良性震颤性帕金森病患者的临床特点和纵向病程。设计：计算机检索病历数据库。地点：明尼苏达州罗切斯特市梅奥诊所患者：116例患者中，16例（10男6女）有至少8年的帕金森病史，经高级运动障碍专家检查，在最初诊断为帕金森病（PD）后最终被诊断为良性震颤性帕金森病。干预措施:没有。主要结局指标：发病年龄、对左旋多巴治疗的反应、震颤特征和家族史。结果：末次随访时平均病程11年（范围8 ~ 25年）。平均发病年龄为58.5岁，比大多数PD系列患者年轻，大多数患者左旋多巴反应较差（尽管一些左旋多巴试验不充分）。16例患者中有13例出现中度至明显的体位性震颤，其中6例为动态性震颤。10例（63%）患者有PD和/或震颤家族史。三名患者需要进行丘脑深部脑手术来治疗震颤。结论：良性震颤性帕金森病可能是一种独特的临床实体，其特征是以震颤为主，并伴有帕金森病其他方面的轻微进展。这种震颤通常对左旋多巴治疗反应不大。在这个系列中，大多数患者有直系亲属被诊断为震颤或PD。
Background: Benign tremulous parkinsonism has never been precisely defined nor has the long-term course been studied.Objective: To report the clinical features and longitudinal course of patients with benign tremulous parkinsonism encountered in our movement disorders practice.Design: Computer search of medical records database.Setting: Mayo Clinic, Rochester, Minn.Patients: Of 116 patients identified, 16 (10 male and 6 female) had at least an 8-year history of this disease, had been examined by a senior movement disorders specialist, and had ultimately been diagnosed as having benign tremulous parkinsonism after an initial diagnosis of Parkinson disease (PD).Interventions: None.Main outcome Measures: Age at onset of disease, response to levodopa therapy, tremor characteristics, and family history.Results: Mean disease duration was 11 years (range, 8-25 years) at last follow-up. Mean age at onset, 58.5 years, was younger than in most PD series, and most patients had a poor levodopa response (although levodopa trials were inadequate in some). A moderate to marked postural tremor was noted in 13 of the 16 patients, including 6 with a kinetic tremor. A family history of PD and/or tremor was reported in 10 (63%) of our patients. Three patients required thalamic deep brain surgery to treat their tremor.Conclusions: Benign tremulous parkinsonism may be a distinct clinical entity characterized by tremor predominance plus minimal progression of other aspects of parkinsonism. The tremor is often not very responsive to levodopa therapy. In this series, most patients had immediate family members with a diagnosis of tremor or PD.