1、非酮症性高血糖所致的舞蹈样非酮症性高血糖所致的舞蹈样投掷运动投掷运动non-ketotic hyperglycaemia induced chorea-ballismHemiballism-hemichorea(HB-HC)Hemiballism-hemichorea(HB-HC)A clinical spectrum of continuous,A clinical spectrum of continuous,nonpatterned,and involuntary nonpatterned,and involuntary movements involving one side of t
2、he movements involving one side of the bodybody Focal vascular lesion in the contralateral Focal vascular lesion in the contralateral basal ganglia basal ganglia Metabolic derangements(e.g.,non-ketotic Metabolic derangements(e.g.,non-ketotic hyperglycemia or hyperthyroidism)hyperglycemia or hyperthy
3、roidism)brain neoplasm brain neoplasm infectious diseases of the central nervous infectious diseases of the central nervous system(e.g.,human immunodeficiency virus system(e.g.,human immunodeficiency virus infection)infection)non-ketotic hyperglycemia is the second most common cause of HB-HCPresenta
4、tion of striatal hyperintensity on T1-weighted MRI in patients Presentation of striatal hyperintensity on T1-weighted MRI in patients with hemiballism-hemichorea caused by non-ketotic hyperglycemia:Report with hemiballism-hemichorea caused by non-ketotic hyperglycemia:Report of seven new cases and a
5、 review of literature.of seven new cases and a review of literature.J Neurol(2001)248presentation of ballistic or choreiform presentation of ballistic or choreiform movements of at least two of the unilateral face,movements of at least two of the unilateral face,neck,upper limb and lower limb region
6、s;neck,upper limb and lower limb regions;a markedly elevated blood glucose level at the a markedly elevated blood glucose level at the onset of HB-HC;onset of HB-HC;(3)a hyperintensive lesion in the contralateral(3)a hyperintensive lesion in the contralateral striatum on brain CT and/or MRI;striatum
7、 on brain CT and/or MRI;(4)an abrupt cessation of the dyskinesia after(4)an abrupt cessation of the dyskinesia after achieving hyperglycemic control;achieving hyperglycemic control;(5)no evidence of acute cerebrovascular,infectious,(5)no evidence of acute cerebrovascular,infectious,or inflammatory l
8、esions on brain CT and/or MRI;or inflammatory lesions on brain CT and/or MRI;(6)no evidence of other metabolic derangement,(6)no evidence of other metabolic derangement,recreational drug use,or a known history of recreational drug use,or a known history of degenerative disorder.degenerative disorder
9、.Diagnosis of non-ketotic hyperglycemia Diagnosis of non-ketotic hyperglycemia hyperosmolar syndrome(NKHHS)hyperosmolar syndrome(NKHHS)was made based on the observation of was made based on the observation of hyperglycemia hyperglycemia(blood sugar levels greater than 500(blood sugar levels greater
10、than 500 mg/dl)mg/dl),the the absence of ketonemiaabsence of ketonemia and and a serum osmolality greater than 350 mmol/kga serum osmolality greater than 350 mmol/kg 1995 1995年一例年一例7474岁老年女性，急性起病，左舞蹈动作。血岁老年女性，急性起病，左舞蹈动作。血糖糖296mg/dl296mg/dl，血渗透压，血渗透压296mOmsm/L.296mOmsm/L.尿酮阴性，尿糖阳性。舞尿酮阴性，尿糖阳性。舞蹈动作持续了蹈
11、动作持续了3737天，天，T1T1高信号、高信号、T2T2低信号持续低信号持续1010个月消失。个月消失。SPECTSPECT显示为高灌注。作者推测为小梗死和钙沉积为显示为高灌注。作者推测为小梗死和钙沉积为MRIMRI异常异常信号的原因。信号的原因。19991999年一例，症状同样，偏侧舞蹈。年一例，症状同样，偏侧舞蹈。MRIMRI信号同前例，但信号同前例，但有强化，强化范围同有强化，强化范围同T1T1异常信号区域。推测异常信号区域。推测BBBBBB破坏在先，然破坏在先，然后形成类似后形成类似MRIMRI异常信号区。异常信号区。20012001年，年，9292岁男性。症状、影像学同前。尸检证实
12、：多岁男性。症状、影像学同前。尸检证实：多灶性小梗死灶、反应性胶质增生、神经元间反应灶性小梗死灶、反应性胶质增生、神经元间反应（interneuronal response.interneuronal response.）1999 1999年另一例，年另一例，2222岁。症状同前。岁。症状同前。CTCT示稍高密度影。示稍高密度影。MRIMRI同前。同前。20012001年年5 5例。诱因及症状均同前。例为以前未发现患例。诱因及症状均同前。例为以前未发现患糖尿病。症状持续糖尿病。症状持续6 6月到月到5 5年，病程年，病程2 2天天1 1月。例有典型的月。例有典型的MRIMRI表现，一例无明显表
13、现，一例无明显MRIMRI异常信号灶。异常信号灶。20042004年，有人对此病的为微量出血的发病机制提出一些年，有人对此病的为微量出血的发病机制提出一些疑问，最终推测为进展性梗死，并与星形细胞反应性增生有疑问，最终推测为进展性梗死，并与星形细胞反应性增生有关关 2002 2002年，年，Oh,S.HOh,S.H等综述了等综述了19851985年年20012001年间报道年间报道的的5353例（包括报道新发例病人）并进行了例（包括报道新发例病人）并进行了MetaMeta分析，分析，指出了指出了CHBGCHBG的特点为：的特点为：老年女性受累多（女老年女性受累多（女/男比为男比为30/1730/
14、17），），71.171.1岁（岁（22 22 9292）平均血糖水平为）平均血糖水平为481.5mg/dl(169 481.5mg/dl(169 1264),HbAlc 1264),HbAlc 为为14.414.4（9.99.919.219.2），血浆渗透压为），血浆渗透压为305.9mmol/kg305.9mmol/kg。绝大部分为单侧舞蹈，少部分发展成双侧。绝大部分为单侧舞蹈，少部分发展成双侧。影像学显示：所有病例均有壳核受累。除一例外，内囊影像学显示：所有病例均有壳核受累。除一例外，内囊前肢基本不受累。前肢基本不受累。2222例随访显示，症状与影像学同步性逐渐缓解。例随访显示，症状与影
15、像学同步性逐渐缓解。3939例痊例痊愈，愈，1414例好转。例好转。7 7例症状复发。例症状复发。CT showed an increased density in the CT showed an increased density in the contralateral putamen and/or caudate contralateral putamen and/or caudate MRI revealed abnormal hyperintensity MRI revealed abnormal hyperintensity on T1-weighted and hypointe
16、nsity on T2-on T1-weighted and hypointensity on T2-weighted images weighted images The striatal hyperdensity in the The striatal hyperdensity in the brain CT completely resolved within 3 brain CT completely resolved within 3 months and in 6 months on MRI.months and in 6 months on MRI.A review reveal
17、ed a total of 35 casesA review revealed a total of 35 cases There was no gender difference and the average There was no gender difference and the average age at the on-set of dyskinesia was 72 years.age at the on-set of dyskinesia was 72 years.Prognosis of all the reported cases was Prognosis of all
18、 the reported cases was excellent and their hyperkinetic states all excellent and their hyperkinetic states all abruptly ceased after hyperglycemic control had abruptly ceased after hyperglycemic control had been achieved.been achieved.Twelve cases had follow up neuroimaging Twelve cases had follow
19、up neuroimaging examination.These showed complete resolution examination.These showed complete resolution within 11 months in 9 cases,partial resolution within 11 months in 9 cases,partial resolution after 6 months in 1 case,and no change was seen after 6 months in 1 case,and no change was seen in 2
20、 cases 6 months later.in 2 cases 6 months later.男性，男性，57岁。尿糖岁。尿糖(+)二次血糖分别为二次血糖分别为15.76mmol/L和和 14.89/mmol/LThe nature of the characteristic The nature of the characteristic CT/MRI signal changesCT/MRI signal changes still debated still debated Based on the evolution of clinical manifestations Based
21、on the evolution of clinical manifestations and the findings of the neuroimages,Chang et al.and the findings of the neuroimages,Chang et al.suggested that suggested that putaminal petechial hemorrhageputaminal petechial hemorrhage might might be the pathological mechanism.be the pathological mechani
22、sm.among neuronal subtypes,striatal medium spiny among neuronal subtypes,striatal medium spiny neurones are highly vulnerable to neurones are highly vulnerable to energy depletionenergy depletion.The hypothesis of a reversible metabolic impairment The hypothesis of a reversible metabolic impairment
23、may explain the transient MRI alterations.may explain the transient MRI alterations.studies by SPECT and PET have revealed the studies by SPECT and PET have revealed the reduction of blood flow and metabolism in the reduction of blood flow and metabolism in the contralateral striatumcontralateral st
24、riatum MR spectroscopy has also demonstrated the MR spectroscopy has also demonstrated the presence of pronounced energy depletion and presence of pronounced energy depletion and neuronal dysfunction in the contralateral neuronal dysfunction in the contralateral striatumstriatum proton MRI spectrosc
25、opy and diffusion weighted proton MRI spectroscopy and diffusion weighted MRI studies suggest a hyperviscosity syndrome,MRI studies suggest a hyperviscosity syndrome,possibly caused by hyperglycaemia,and possibly caused by hyperglycaemia,and concomitant concomitant cytotoxic edemacytotoxic edema cou
26、ld be the cause of could be the cause of the MRI changes.the MRI changes.MRI MRI的信号变化可以用该部位的点状出血及随后的高的信号变化可以用该部位的点状出血及随后的高铁血红蛋白形成和含铁血黄素的沉积来解释铁血红蛋白形成和含铁血黄素的沉积来解释 因为很多高血糖患者的周围神经都有髓鞘的损害，所因为很多高血糖患者的周围神经都有髓鞘的损害，所以壳核中的高信号可能与损害的神经髓鞘有关，它可以选以壳核中的高信号可能与损害的神经髓鞘有关，它可以选择性地混合髓鞘结合水与轴突游离水使择性地混合髓鞘结合水与轴突游离水使T1T1像缩短像缩
27、短 biopsy specimen from the hyperintense biopsy specimen from the hyperintense putamenputamen revealed a slight atrocytosis and vacuolization revealed a slight atrocytosis and vacuolization or a fragment of gliotic brain tissue with abundant or a fragment of gliotic brain tissue with abundant gemisto
28、cytes,but was without deposition of gemistocytes,but was without deposition of hemosiderin.hemosiderin.标本检查发现病变部位仅为轻度的星形胶质细胞增生和标本检查发现病变部位仅为轻度的星形胶质细胞增生和空泡形成，而没有铁或钙的沉积空泡形成，而没有铁或钙的沉积 标本中发现了含有原浆性星形胶质细胞的脑胶质碎片，标本中发现了含有原浆性星形胶质细胞的脑胶质碎片，并认为并认为MRIMRI短短T1T1信号是由于肿胀的原浆性星形胶质细胞中蛋信号是由于肿胀的原浆性星形胶质细胞中蛋白水化层所致白水化层所致 病理生
29、理基础不明，可能与糖尿病脑血管病变所致的病理生理基础不明，可能与糖尿病脑血管病变所致的急性血脑屏障功能障碍及高血糖后的代谢紊乱有关急性血脑屏障功能障碍及高血糖后的代谢紊乱有关 雌激素可以降低黑质纹状体系统多巴胺的功能、增加雌激素可以降低黑质纹状体系统多巴胺的功能、增加多巴胺受体的密度，使多巴胺受体产生超敏现象，更年期多巴胺受体的密度，使多巴胺受体产生超敏现象，更年期妇女雌激素减少，故本病多见于老年女性妇女雌激素减少，故本病多见于老年女性 从神经影像上来看本病的发生与尾状核及豆状核损害从神经影像上来看本病的发生与尾状核及豆状核损害有关，波谱分析发现病灶部位的有关，波谱分析发现病灶部位的N-N-乙
30、酰天冬氨酸（乙酰天冬氨酸（NAANAA）/肌酸（肌酸（CrCr）比值低于对侧、胆碱（）比值低于对侧、胆碱（ChoCho）/Cr/Cr比值高于对侧比值高于对侧且有乳酸盐峰出现，这三种变化分别预示了神经原的丢失且有乳酸盐峰出现，这三种变化分别预示了神经原的丢失和损害、神经胶质的增生和潜在的慢性局灶性脑血管病和损害、神经胶质的增生和潜在的慢性局灶性脑血管病 SPECT SPECT研究发现基底节区血流灌注减低，研究发现基底节区血流灌注减低，PETPET研究证实病研究证实病灶部位糖的代谢显著降低，证实了病灶部位存在缺血现象灶部位糖的代谢显著降低，证实了病灶部位存在缺血现象 因为本病多见于糖尿病非酮症高血
31、糖患者，有学者认为因为本病多见于糖尿病非酮症高血糖患者，有学者认为此类患者细胞能量代谢以无氧代谢为主，三羧酸循环被抑制，此类患者细胞能量代谢以无氧代谢为主，三羧酸循环被抑制，脑细胞以脑细胞以GABAGABA为能量来源，导致为能量来源，导致 GABAGABA被很快耗竭，基底节被很快耗竭，基底节正常活动受到损害，临床上出现偏侧舞蹈症正常活动受到损害，临床上出现偏侧舞蹈症 解释很多，如解释很多，如：1)肥大星形胶质细胞肥大星形胶质细胞；2)迟发缺血高迟发缺血高信号信号；3)二氧化锰歧化酶二氧化锰歧化酶 非酮症性高血糖所致的舞蹈样投掷运动非酮症性高血糖所致的舞蹈样投掷运动1，见于糖尿病患者；，见于糖尿
32、病患者；2，以急起的舞蹈样投掷运动为特征，一般无神经系统其他，以急起的舞蹈样投掷运动为特征，一般无神经系统其他的症状和体征；的症状和体征；3，发病时血糖高，但血酮体，发病时血糖高，但血酮体阴性阴性；4，CT基基底底节区密度稍高，节区密度稍高，MRI表现为短表现为短T1信号，信号，T2像改变像改变不明显，也可出现稍短不明显，也可出现稍短T2的表现；的表现；5，疾病病理不明，可能与，疾病病理不明，可能与高血糖导致局部血管通透性增加高血糖导致局部血管通透性增加导致血管渗血导致血管渗血有关，也可能与有关，也可能与高血糖所致的局部代谢障碍高血糖所致的局部代谢障碍有有关；关；6，首先应尽快纠正高血糖，同时可予氟哌啶醇等对症，首先应尽快纠正高血糖，同时可予氟哌啶醇等对症处理；处理；7，预后：纠正高血糖后大部分病人症状消失，预后：纠正高血糖后大部分病人症状消失
