Romero Blanco M, João G, Monteiro E
Servicio de Neurología; Hospital Distrital Barlavento-Algarvio, Portimao, Portugal.
Rev Neurol. 2002;34(3):256-8.
Hyperglycaemia, usually not ketotic, may cause hemichorea hemiballism. The mechanism of this physiopathological syndrome is still not clear. It usually occurs in elderly ladies and is associated with hyperdensity in the striate area contralateral to the affected side, on CT and MR potentiated in T1.
Three days before hospital admission, and 86 year old woman presented with the acute onset of choreiform movements of her left limbs, particularly her left arm. She had not taken neuroleptic drugs and there was no family history of chorea. On neurological examination there were no other unusual findings. Laboratory tests showed hyperglycemia with metabolic acidosis and ketone bodies in the urine. On the CT scan there was a hyperdense lesion in the right striate area, but this did not act as a space occupying lesion. Treatment was started with intravenous insulin and the symptoms disappeared 48 hours later.
We emphasize the rarity of the association of hemichorea and diabetic ketoacidosis. From the histological studies done in some of these patients, it seems that the hyperdensity of the striate area may be due to the proliferation of hypertrophic astrocytes secondary to small ischemic lesions. When the hyperglycaemia is treated, the chorea disappears within a few days and it is unusual for neuroleptic treatment to be required. In cases of hemichorea, hyperglycaemia should be ruled out, as should structural causes (tumors, infarcts, hematomas, traumatic lesions, etc.).
高血糖症通常无酮血症,可导致偏侧舞蹈症-偏侧投掷症。这种生理病理综合征的机制尚不清楚。它通常发生在老年女性中,在CT和T1加权磁共振成像上,与患侧对侧纹状体区域的高密度有关。
入院前三天,一名86岁女性出现左侧肢体,尤其是左臂的舞蹈样动作急性发作。她未服用过抗精神病药物,且无舞蹈症家族史。神经系统检查未发现其他异常。实验室检查显示血糖升高伴代谢性酸中毒及尿酮体。CT扫描显示右侧纹状体区域有高密度病变,但该病变未表现为占位性病变。开始静脉注射胰岛素治疗,48小时后症状消失。
我们强调偏侧舞蹈症与糖尿病酮症酸中毒关联的罕见性。从对其中一些患者进行的组织学研究来看,纹状体区域的高密度可能是由于小缺血性病变继发的肥大星形胶质细胞增殖所致。当高血糖得到治疗时,舞蹈症在几天内消失,通常不需要抗精神病药物治疗。对于偏侧舞蹈症病例,应排除高血糖症以及结构性病因(肿瘤、梗死、血肿、创伤性病变等)。