El Otmani H, Moutaouakil F, Fadel H, El Ouafi N, Abdoh Rafai M, El Moutaouakil B, Slassi I
Department of Neurology, Al Kortobi Hospital, Tangie, Morocco.
Funct Neurol. 2009 Jul-Sep;24(3):129-32.
Diabetic patients during hyperglycaemic crises may present a rare syndrome characterised by a typical triad: unilateral involuntary movements (hemichoreahemiballism), radiological contralateral striatal abnormality, and rapid resolution of symptoms after glycae - mic correction. This study reports a series of patients showing less usual aspects and also discusses the pathophysiology of this clinical-radiological syndrome. We included in this study four patients presenting choreic or ballic involuntary movements and in whom aetiological assessment revealed frank non-ketotic hyperglycaemia, without other abnormalities that could explain the movement disorder. All the patients underwent CT or MR brain imaging. The typical triad was present in only one case. Less classical aspects were more frequently found: movement disorders revealed diabetes in two patients and one patient had generalised chorea and strictly normal neuroimaging. Correction of blood glucose was not sufficient to improve symptoms in two cases. In one, abnormal movements persisted despite treatment with tetrabenazine. The clinical, radiological and outcome spectrum of the syndrome of chorea-ballismus induced by non-ketotic hyperglycaemia is heterogeneous and not restricted to a typical triad.
糖尿病患者在高血糖危象期间可能会出现一种罕见综合征,其特征为典型的三联征:单侧不自主运动(偏侧舞蹈症-偏侧投掷症)、影像学上对侧纹状体异常以及血糖纠正后症状迅速缓解。本研究报告了一系列表现出不常见特征的患者,并讨论了这种临床-放射学综合征的病理生理学。本研究纳入了4例出现舞蹈样或投掷样不自主运动的患者,病因评估显示为明显的非酮症高血糖,无其他可解释运动障碍的异常情况。所有患者均接受了脑部CT或MRI检查。仅1例出现典型的三联征。更常见的是不太典型的表现:2例患者的运动障碍提示患有糖尿病,1例患者出现全身性舞蹈症且神经影像学完全正常。2例患者血糖纠正后症状并未改善。其中1例患者尽管使用丁苯那嗪治疗,异常运动仍持续存在。非酮症高血糖所致舞蹈症-投掷症综合征的临床、放射学及转归谱具有异质性,并不局限于典型的三联征。