Salgado Paula, Taipa Ricardo, Domingos Joana, Dias Daniel, Pires Manuel Melo, Magalhães Marina
Department of Neurology Centro Hospitalar do Porto Porto Portugal.
Portuguese Brain Bank Neuropathology Unit Centro Hospitalar do Porto Porto Portugal.
Mov Disord Clin Pract. 2017 Sep 8;4(6):819-823. doi: 10.1002/mdc3.12528. eCollection 2017 Nov-Dec.
Chorea may occur as a manifestation of an acute stroke. Patients with vascular-related chorea typically present with an acute or subacute onset of hemichorea, contralateral to the lesion.
In this clinico-pathological case, we report a 90-year-old female who presented, at age 81, with a transient episode of generalized chorea. Over the years, the patient continued to have intermittent episodes of generalized chorea or hemichorea, followed by a progressive dementia syndrome with gait and sphincter disturbance. There was no family history of chorea or dementia. Laboratory tests for paraneoplastic or autoimmune disorders and genetic testing for Huntington's disease were normal or negative. Magnetic resonance imaging showed subcortical and basal ganglia atrophy associated with ischemic leukoencephalopathy and lacunar infarcts. The post-mortem examination identified multiple lacunar infarcts (cortex, white matter, thalamus, basal ganglia) and minor Alzheimer's disease neuropathological changes.
Vascular disease, affecting the basal ganglia, is included in most lists of causes of generalized chorea. Proven cases are difficult to find. We present a rare case of vascular pathology causing late onset generalized and intermittent chorea. We highlight the intermittent nature of the chorea that could be explained by cumulative vascular lesions or functional disconnection in a previous deficient circuit.
舞蹈症可能作为急性中风的一种表现出现。血管相关性舞蹈症患者通常表现为病变对侧急性或亚急性发作的偏侧舞蹈症。
在这个临床病理病例中，我们报告了一名90岁女性，她在81岁时出现了一次短暂的全身性舞蹈症发作。多年来，患者持续出现间歇性全身性舞蹈症或偏侧舞蹈症发作，随后出现伴有步态和括约肌障碍的进行性痴呆综合征。患者无舞蹈症或痴呆症家族史。副肿瘤性或自身免疫性疾病的实验室检查以及亨廷顿病的基因检测均正常或为阴性。磁共振成像显示与缺血性白质脑病和腔隙性梗死相关的皮质下和基底节萎缩。尸检发现多处腔隙性梗死（皮质、白质、丘脑、基底节）以及轻度阿尔茨海默病神经病理改变。
影响基底节的血管疾病在大多数全身性舞蹈症病因列表中都有提及。已证实的病例很难找到。我们报告了一例罕见的血管病变导致迟发性全身性间歇性舞蹈症的病例。我们强调了舞蹈症的间歇性特点，这可能是由先前存在缺陷的回路中累积的血管病变或功能连接中断所解释的。
