Cosentino Carlos, Torres Luis, Nuñez Yesenia, Suarez Rafael, Velez Miriam, Flores Martha
Departamento de Enfermedades Neurodegenerativas, Instituto Nacional de Ciencias Neurológicas, Lima, Perú
Tremor Other Hyperkinet Mov (N Y). 2016 Jul 19;6:402. doi: 10.7916/D8DN454P. eCollection 2016.
Hemichorea/hemiballism associated with nonketotic hyperglycemia is a well-recognized syndrome, but few case series have been reported in the literature.
We describe 20 patients with hemichorea/hemiballism associated with hyperglycemia (9 males and 11 females) with mean age of 67.8 years. Ten patients had a previous diagnosis of type 2 diabetes mellitus, and one had type 1 diabetes mellitus. Six of them had documentation of poor diabetic control over at least the last 3 months. Nine patients had new-onset hyperglycemia with a diagnosis of diabetes mellitus made after discharge. Seventeen patients had unilateral chorea/ballism, while three had bilateral chorea/ballism. Eighteen cases had striatal hyperdensities on computed tomography (CT) and/or hyperintense signals on magnetic resonance imaging (MRI). The putamen was affected in all cases, and the caudate nucleus was involved in nine.
Hemichorea/hemiballism associated with nonketotic hyperglycemia can be the presenting sign of diabetes mellitus in almost half of cases or can occur after a few months of poor glycemic control in patients with diagnosed diabetes. This case series is one of the largest to date and adds valuable information about clinical and neuroimaging features that are comparable with published data but also emphasize the role of adequate diabetes mellitus control.
与非酮症高血糖相关的偏侧舞蹈症/偏侧投掷症是一种公认的综合征,但文献中报道的病例系列较少。
我们描述了20例与高血糖相关的偏侧舞蹈症/偏侧投掷症患者(9例男性和11例女性),平均年龄为67.8岁。10例患者既往诊断为2型糖尿病,1例为1型糖尿病。其中6例有记录显示至少在过去3个月血糖控制不佳。9例患者为新发高血糖,出院后诊断为糖尿病。17例患者为单侧舞蹈症/投掷症,3例为双侧舞蹈症/投掷症。18例患者在计算机断层扫描(CT)上有纹状体高密度影和/或在磁共振成像(MRI)上有高信号。所有病例均累及壳核,9例累及尾状核。
与非酮症高血糖相关的偏侧舞蹈症/偏侧投掷症在近半数病例中可能是糖尿病的首发症状,或在已确诊糖尿病患者血糖控制不佳数月后出现。该病例系列是迄今为止最大的病例系列之一,增加了有关临床和神经影像学特征的有价值信息,这些信息与已发表的数据相当,但也强调了充分控制糖尿病的作用。