Caviness J N, Forsyth P A, Layton D D, McPhee T J
Department of Neurology, Mayo Clinic, Scottsdale, Arizona 85259.
Mov Disord. 1995 Jan;10(1):22-7. doi: 10.1002/mds.870100106.
We present three cases of the adult opsoclonus-myoclonus syndrome in patients with systemic carcinoma. In addition to opsoclonus and myoclonus, other clinical components of the syndrome can include ataxia, tremor, gait and stance dysfunction, altered mental status, and head and face dyskinesias. The most common etiologies are idiopathic, paraneoplastic, and infectious encephalitis. Radiographic and pathological studies suggest brain-stem dysfunction with associated cerebellar and/or cerebellar pathway dysfunction. In many cases, there is evidence for the involvement of immunologic and/or inflammatory processes in the pathogenesis of this syndrome. The timely recognition of this syndrome is important because of its implications for the underlying etiology and prognosis. The appearance of this syndrome should prompt the search for an occult malignancy.
我们报告了3例患有系统性癌症的成年眼阵挛-肌阵挛综合征患者。除眼阵挛和肌阵挛外,该综合征的其他临床症状还可包括共济失调、震颤、步态和姿势功能障碍、精神状态改变以及头面部运动障碍。最常见的病因是特发性、副肿瘤性和感染性脑炎。影像学和病理学研究提示脑干功能障碍伴相关小脑和/或小脑通路功能障碍。在许多病例中,有证据表明免疫和/或炎症过程参与了该综合征的发病机制。及时识别该综合征很重要,因为它对潜在病因和预后有影响。该综合征的出现应促使医生寻找隐匿性恶性肿瘤。