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1、,非酮症性高血糖所致的舞蹈样投掷运动non-ketotic hyperglycaemia induced chorea-ballism,Hemiballism-hemichorea(HB-HC)A clinical spectrum of continuous,nonpatterned,and involuntary movements involving one side of the body,Focal vascular lesion in the contralateral basal ganglia Metabolic derangements(e.g.,non-ketotic hy
2、perglycemia or hyperthyroidism)brain neoplasm infectious diseases of the central nervous system(e.g.,human immunodeficiency virus infection),non-ketotic hyperglycemia is the second most common cause of HB-HC,Presentation of striatal hyperintensity on T1-weighted MRI in patients with hemiballism-hemi
3、chorea caused by non-ketotic hyperglycemia:Report of seven new cases and a review of literature.J Neurol(2001)248,presentation of ballistic or choreiform movements of at least two of the unilateral face,neck,upper limb and lower limb regions;a markedly elevated blood glucose level at the onset of HB
4、-HC;(3)a hyperintensive lesion in the contralateral striatum on brain CT and/or MRI;(4)an abrupt cessation of the dyskinesia after achieving hyperglycemic control;(5)no evidence of acute cerebrovascular,infectious,or inflammatory lesions on brain CT and/or MRI;(6)no evidence of other metabolic deran
5、gement,recreational drug use,or a known history of degenerative disorder.,Diagnosis of non-ketotic hyperglycemia hyperosmolar syndrome(NKHHS)was made based on the observation of hyperglycemia(blood sugar levels greater than 500 mg/dl),the absence of ketonemia and a serum osmolality greater than 350
6、mmol/kg,1995年一例74岁老年女性，急性起病，左舞蹈动作。血糖296mg/dl，血渗透压296mOmsm/L.尿酮阴性，尿糖阳性。舞蹈动作持续了37天，T1高信号、T2低信号持续10个月消失。SPECT显示为高灌注。作者推测为小梗死和钙沉积为MRI异常信号的原因。1999年一例，症状同样，偏侧舞蹈。MRI信号同前例，但有强化，强化范围同T1异常信号区域。推测BBB破坏在先，然后形成类似MRI异常信号区。2001年，92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（interneuronal response.）,1999年另一例，22岁。症状同前。
7、CT示稍高密度影。MRI同前。2001年5例。诱因及症状均同前。例为以前未发现患糖尿病。症状持续6月到5年，病程2天1月。例有典型的MRI表现，一例无明显MRI异常信号灶。2004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关,2002年，Oh,S.H等综述了1985年2001年间报道的53例（包括报道新发例病人）并进行了Meta分析，指出了CHBG的特点为：老年女性受累多（女/男比为30/17），71.1岁（22 92）平均血糖水平为481.5mg/dl(169 1264),HbAlc 为14.4（9.919.2），血浆渗透压为305.9m
8、mol/kg。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，内囊前肢基本不受累。22例随访显示，症状与影像学同步性逐渐缓解。39例痊愈，14例好转。7例症状复发。,CT showed an increased density in the contralateral putamen and/or caudate MRI revealed abnormal hyperintensity on T1-weighted and hypointensity on T2-weighted images The striatal hyperdensity in the
9、brain CT completely resolved within 3 months and in 6 months on MRI.,A review revealed a total of 35 cases There was no gender difference and the average age at the on-set of dyskinesia was 72 years.Prognosis of all the reported cases was excellent and their hyperkinetic states all abruptly ceased a
10、fter hyperglycemic control had been achieved.Twelve cases had follow up neuroimaging examination.These showed complete resolution within 11 months in 9 cases,partial resolution after 6 months in 1 case,and no change was seen in 2 cases 6 months later.,男性，57岁。尿糖(+)二次血糖分别为15.76mmol/L和 14.89/mmol/L,The
11、 nature of the characteristic CT/MRI signal changes still debated Based on the evolution of clinical manifestations and the findings of the neuroimages,Chang et al.suggested that putaminal petechial hemorrhage might be the pathological mechanism.among neuronal subtypes,striatal medium spiny neurones
12、 are highly vulnerable to energy depletion.The hypothesis of a reversible metabolic impairment may explain the transient MRI alterations.,studies by SPECT and PET have revealed the reduction of blood flow and metabolism in the contralateral striatum MR spectroscopy has also demonstrated the presence
13、 of pronounced energy depletion and neuronal dysfunction in the contralateral striatum proton MRI spectroscopy and diffusion weighted MRI studies suggest a hyperviscosity syndrome,possibly caused by hyperglycaemia,and concomitant cytotoxic edema could be the cause of the MRI changes.,MRI的信号变化可以用该部位的
14、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 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，它可以选择性地混合髓鞘结合水与轴突游离水使T1像缩短,biopsy specimen from the hyperintense putamen revealed a slight atrocytosis and vacuolization or a fragment of gliotic brain tissue with abundant gemistocytes,but was without deposition of hemosiderin
15、.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 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MRI短T1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,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 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有关，波谱分析发现病灶部位的N-乙酰天冬氨酸（NAA）/肌酸（Cr）比值低于对侧、胆碱（Cho）/Cr比值高于对侧且有乳酸盐峰出现
16、，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,SPECT研究发现基底节区血流灌注减低，PET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 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，脑细胞以GABA为能量来源，导致 GABA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 解释很多，如：1)肥大星形胶质细胞；2)迟发缺血高信号；3)二氧化锰歧化酶,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；2，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；3，发病时血糖高，但血酮体阴性；4，CT基底节区密度稍高，MRI表现为短T1信号，T2像改变不明显，也可出现稍短T2的表现；5，疾病病理不明，可能与高血糖导致局部血管通透性增加导致血管渗血有关，也可能与高血糖所致的局部代谢障碍有关；6，首先应尽快纠正高血糖，同时可予氟哌啶醇等对症处理；7，预后：纠正高血糖后大部分病人症状消失,
