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非酮症高血糖性舞蹈症临床特点分析

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  【摘要】 目的 探討非酮症高血糖性舞蹈症患者的临床特点及影像学特点。方法 选取8例非酮症高血糖性舞蹈症患者作为研究对象, 动态观察其临床症状、实验室及影像学检查结果, 应用降糖药物、多巴胺受体阻滞剂治疗舞蹈样症状, 观察其预后。结果 入院时急查血糖8.56~34.30 mmol/L, 平均血糖(16.60±7.49)mmol/L。血浆渗透压286.44~342.42 mOsm/L, 平均血浆渗透压(302.15±6.35)mOsm/L;尿酮体除1例阳性(+)、第2天复查阴性外, 余均为阴性。舞蹈样症状表现:紧张会加重、安静时会减轻、睡眠中消失。舞蹈侧肢体肌张力增加、病理反射阳性者(脑梗死后遗症)2例, 其余均未发现其他神经系统阳性体征。8例患者颅脑CT均表现为基底节区稍高密度影, CT值36~48 Hu。双侧受累2例, 患侧肢体对侧基底节区受累6例。壳核全部受累8例, 尾状核头同时受累4例。其中5例行头磁共振成像(MRI)检查, 基底节区均发现异常信号5例, 表现为患侧肢体对侧壳核、尾状核或者两侧壳核、尾状核T1高信号, 边界清析, 无占位效应及水肿, 增强并无强化。3例于患病后2个月~1年复查颅脑CT或MRI, 显示基底节区高密度影减低或消失, 而且并无软化灶形成。所有患者均予活血化淤、营养神经等药物治疗。血糖控制后舞蹈样症状在2~3周以内逐渐消失。结论 非酮症高血糖性舞蹈症影像学表现特异, 控制血糖可迅速改善病情, 预后良好。
  【关键词】 舞蹈症性障碍;非酮症高血糖;多巴胺受体阻滞剂
  【Abstract】 Objective To discuss the clinical and imaging characteristics of nonketotic hyperglycemic chorea. Methods A total of 8 patients with non-ketotic hyperglycemic chorea were selected as the study subjects, and their clinical symptoms, laboratory and imaging findings were dynamically observed. Hypoglycemic drugs and dopamine receptor blockers were used to treat chorea-like symptoms, and the prognosis was observed. Results At admission, the blood glucose level was 8.56~34.30 mmol/L, with average blood glucose as (16.60± 7.49) mmol/L. Plasma osmotic pressure was 286.44~342.42 mOsm/L, with mean plasma osmotic pressure as (302.15±6.35) mOsm/L. The urine ketone body was negative except 1 case (+) positive on 1st day and negative on the 2nd day. Chorea-like symptoms: stress increases, quietness decreases, and sleep disappears. There were 2 cases with increased muscle tension and positive pathological reflex (sequelae of cerebral infarction) in the dancing limbs. No other positive signs of nervous system were found in the rest. CT findings of 8 patients were slightly high density in basal ganglia, with CT values 36 Hu~48 Hu. There were 2 cases of bilateral involvement, and 6 cases of involvement of the affected side of the basal ganglia. There were 8 cases of putamen involvement and 4 cases of caudate nucleus head involvement. The other 5 cases underwent head magnetic resonance imaging (MRI). Abnormal signals were found in basal ganglia in 5 cases, showing high signal intensity on T1 in contralateral putamen, caudate nucleus or bilateral putamen and caudate nucleus of the affected limbs, with clear boundary, no space-occupying effect and edema, and no enhancement. The CT or MRI findings of 3 patients at 2 months ~1 year after the onset of the disease showed that the basal ganglia had decreased or disappeared in high density, and no softening lesions were found. All patients were treated with drugs such as activating blood circulation and removing blood stasis and nourishing nerve. Choreoid symptoms gradually disappeared within 2~3 weeks after blood glucose control. Conclusion Nonketotic hyperglycemic chorea has specific imaging features. Controlling blood glucose can improve the condition rapidly and have a good prognosis.   【Key words】 Chorean disorder; Non-ketotic hyperglycemia; Dopamine receptor blockers
  非酮癥高血糖性舞蹈症患者大多糖尿病病史长且血糖控制不良, 部分为初发老年糖尿病患者。临床表现多样, 舞蹈症是其较少见的临床症状。发病机制迄今为止仍不十分清楚。本研究根据本院收治的8例非酮症高血糖性舞蹈症患者的临床资料, 并结合相关文献探讨其临床表现、影像学特点和治疗效果, 报告如下。
  1 资料与方法
  1. 1 一般资料 选取2010年1月~2018年6月本院收治的8例非酮症高血糖性舞蹈症患者作为研究对象, 排除药物或一氧化碳(CO)中毒、遗传性舞蹈病、肝豆状核变性、慢性酒精中毒等其他疾病。8例患者中男3例, 女5例, 年龄52~78岁, 平均年龄(65.8±5.5)岁。2例首次确诊糖尿病, 6例糖尿病病史3~18年, 均血糖控制欠佳。5例患者既往脑梗死、高血压病史, 病史7~12年。2例合并冠心病, 病程分别为4、6年。
  1. 2 方法 动态观察8例非酮症高血糖性舞蹈症患者的临床症状、实验室及影像学检查, 应用降糖药物、多巴胺受体阻滞剂治疗舞蹈样症状。
  2 结果
  2. 1 实验室检查 入院时急查血糖8.56~34.30 mmol/L, 平均血糖(16.60±7.49)mmol/L。血浆渗透压286.44~342.42 mOsm/L, 平均血浆渗透压(302.15±6.35)mOsm/L;尿酮体除1例阳性(+)、第2天复查阴性外, 余均为阴性。
  2. 2 临床表现 偏身舞蹈样症状6例, 左侧偏身舞蹈2例, 右侧偏身舞蹈4例, 头面部、口唇、舌亦受累2例。双侧舞蹈样症状2例。仅上肢受累3例, 上下肢同时受累5例。舞蹈样症状表现:紧张会加重、安静时会减轻、睡眠中消失。舞蹈侧肢体肌张力增加、病理反射阳性者(脑梗死后遗症) 2例, 其余均未发现其他神经系统阳性体征。
  2. 3 影像学 8例患者均于就诊后行颅脑CT检查, 检查时间距发病时间间隔3~18 d。8例患者颅脑CT均表现为基底节区稍高密度影, CT值36~48 Hu。双侧受累2例, 患侧肢体对侧基底节区受累6例。壳核全部受累8例, 尾状核头同时受累4例。其中5例行头MRI检查, 检查时间距发病时间间隔4~25 d, 基底节区均发现异常信号5例, 表现为患侧肢体对侧壳核、尾状核或者两侧壳核、尾状核T1高信号, 边界清析, 无占位效应及水肿, 增强并无强化。3例于患病后 2个月~1年复查颅脑CT或MRI, 显示基底节区高密度影减低或消失, 而且并无软化灶形成。
  2. 4 治疗及预后 5例患者使用胰岛素治疗;1例患者因原治疗方案血糖控制好未予以调整;2例患者因家属不同意使用胰岛素, 故予口服降糖药治疗。6例患者仅用氟哌啶醇(0.5~2.0 mg, 3次/d口服)控制舞蹈样症状;2例患者同时使用氟哌啶醇、硫必利(100 mg, 3次/d口服)治疗。所有患者均予活血化淤、营养神经等药物治疗。血糖控制后舞蹈样症状在2~3周以内逐渐消失。
  3 讨论
  非酮症高血糖性舞蹈症影像学表现特异, 均表现为颅脑MRI T1基底节区尤其壳核呈高信号, 而且随病情好转T1高信号逐渐减弱或消失[1]。颅脑CT病初表现为高密度影, 可在短时间内消失。本组患者影像学表现均符合上述特点。这种特异性影像学变化的病理生理机制目前仍不清楚。因大多数患者的特异性影像学表现在短时间内减弱或消失, 故基底节区高信号不大可能是钙化[2]。据起病急、CT纹状体高密度、MRI T1高信号, 部分学者推测可能为斑片状出血或梗死后出血[3]。最近1例尸检报告认为可能是纹状体区矿物质沉积和微出血[4]。与常规血肿相比, 病变主要在壳核、尾状核头部、苍白球, 内囊、丘脑等结构并未受累, 无水肿、占位效应, 部分患者双侧同时受累, MRI T1高信号持续时间较长, 上述因素均不支持出血性病变。Wintermark等[5]认为高血糖或高渗透压引起急性壳核内白质不同程度的华勒变性, 导致蛋白质脱水, 从而引起上述影像学变化。Mestre等[6]认为长期血糖控制不佳导致血-脑屏障功能障碍, 深部灰质结构中毛细血管内红细胞经血管壁漏出引起斑点状出血, 这可能是与常规血肿影像学表现不同的原因。
  绝大多数非酮症高血糖性舞蹈症患者预后较好, 仅少数临床症状持续存在[7, 8]。主要治疗方案为控制血糖, 血糖控制后临床症状通常会明显好转甚至消失[9]。Branea等[10]报道1例患者血糖水平与舞蹈样症状评定量表分值呈正相关, 证明血糖水平与舞蹈样症状呈正相关。临床医生常用多巴胺受体拮抗剂控制舞蹈样症状, 如奋乃静、氟哌啶醇等, 疗效不佳者可合用氯硝西泮、地西泮。因多巴胺受体拮抗剂会导致迟发性运动障碍, 治疗方案应个体化。
  综上所述, 非酮症高血糖、舞蹈病、颅脑MRI T1基底节区高信号是非酮症高血糖性舞蹈症的特异性临床表现, 该病虽发病率低, 但临床表现特异, 正规诊治预后良好, 应引起广大临床医生的重视。
  参考文献
  [1] Ohmori H, Hirashima K, Ishihara D, et al. Two casas of hemiballism hemichorea with T1-weighted MR image hyperintensites. Intern Med, 2005(44):1280-1285.
  [2] Shobha N, Sinha S, Taly AB, et al. Diabetic nonketotic hyperosmolar state:interesting imaging observations in 2 patients with involuntary movements and seizures. Neurol India, 2006, 54(4):440-442.   [3] Nath J, Jambhekar K, Rao C, et al. Radiological and pathological changes in hemiballism hemichorea with striatal hyperintensity. J Magn Reson Imaging, 2006, 23(4):564-568.
  [4] Pisani A, Diomedi M, Rum A, et al. Acanthocytosis as a predisposing factor for nonketotic hyperglycaemia induced chorea-ballista. J Neurol Ncurosurg Psychiatry, 2005, 76(12):1717-1719.
  [5] Wintermark M, Fischbein NJ, Mukherjee P, et al. Unilateral putaminal CT, MR, and difusion abnormalities secondary to nonketotic hyperglycemia in the setting of acute neurologic symptoms mimicking stroke. Am J Neuroradiol, 2004, 25(6):975-976.
  [6] Mestre T, Ferreira J, Pimentel J. Putaminal petechial haemorrhage as the cause of non-ketotic hyperglycaemic chorea: a neuropathological case correlated with MRI findings. Journal of Neurology Neurosurgery & Psychiatry, 2007, 78(5):549-550.
  [7] Jagota P, Bhidayasiri R, Lang AE. Movement disorders in patients with diabetes mellitus. Journal of the Neurological Sciences, 2012, 314(1-2):5-11.
  [8] Hawley JS, Weiner WJ. Hemiballismus: current concepts and review. Parkinsonism & Related Disorders, 2012, 18(2):125-129.
  [9] Massaro F, Palumbo P, Falcini M, et al. Generalized chorea-ballism in acute non ketotic hyperglycemia: Findings from diffusion-weighted magnetic resonance imaging. Parkinsonism & Related Disorders, 2012, 18(8):998-999.
  [10] Branca D, Gervasio O, Le PE, et al. Chorea induced by non-ketotic hyperglycaemia: a case report. Neurological Sciences Official Journal of the Italian Neurological Society & of the Italian Society of Clinical Neurophysiology, 2005, 26(4):275-277.
  [收稿日期:2018-11-27]
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