糖尿病高渗偏身不自主运动ppt课件

1、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non-ketotic hyperglycaemia induced chorea-ballism1;.Hemiballism-hemichorea (HB-HC)Hemiballism-hemichorea (HB-HC) A clinical spectrum of continuous, nonpatterned, and involuntary A clinical spectrum of continuous, nonpatterned, and involuntary movements involving one

2、 side of the bodymovements involving one side of the body2;. Focal vascular lesion in the contralateral basal ganglia Focal vascular lesion in the contralateral basal ganglia Metabolic derangements (e. g., non-ketotic hyperglycemia or hyperthyroidism) Metabolic derangements (e. g., non-ketotic hyper

3、glycemia or hyperthyroidism) brain neoplasm brain neoplasm infectious diseases of the central nervous system (e. g., human infectious diseases of the central nervous system (e. g., human immunodeficiency virus infection)immunodeficiency virus infection)non-ketotic hyperglycemia is the second most co

4、mmon cause of HB-HC3;.Presentation of striatal hyperintensity on T1-weighted MRI in patients with Presentation of striatal hyperintensity on T1-weighted MRI in patients with hemiballism-hemichorea caused by non-ketotic hyperglycemia: Report of seven new hemiballism-hemichorea caused by non-ketotic h

5、yperglycemia: Report of seven new cases and a review of literature. cases and a review of literature. J Neurol (2001) 2484;.presentation of ballistic or choreiform movements of at least two of the presentation of ballistic or choreiform movements of at least two of the unilateral face, neck, upper l

6、imb and lower limb regions; unilateral face, neck, upper limb and lower limb regions; a markedly elevated blood glucose level at the onset of HB-HC; a markedly elevated blood glucose level at the onset of HB-HC; (3)a hyperintensive lesion in the contralateral striatum on brain CT and/or (3)a hyperin

7、tensive lesion in the contralateral striatum on brain CT and/or MRI; MRI; (4)an abrupt cessation of the dyskinesia after achieving hyperglycemic (4)an abrupt cessation of the dyskinesia after achieving hyperglycemic control; control; (5)no evidence of acute cerebrovascular, infectious, or inflammato

8、ry lesions (5)no evidence of acute cerebrovascular, infectious, or inflammatory lesions on brain CT and/or MRI; on brain CT and/or MRI; (6)no evidence of other metabolic derangement, recreational drug use, or a (6)no evidence of other metabolic derangement, recreational drug use, or a known history

9、of degenerative disorder.known history of degenerative disorder.5;.Diagnosis of non-ketotic hyperglycemia hyperosmolar syndrome (NKHHS)Diagnosis of non-ketotic hyperglycemia hyperosmolar syndrome (NKHHS) was made based on the observation of hyperglycemia (blood sugar levels was made based on the obs

10、ervation of hyperglycemia (blood sugar levels greater than 500 mg/dl),greater than 500 mg/dl), the absence of ketonemia the absence of ketonemia and a serum osmolality greater than 350 mmol/kg and a serum osmolality greater than 350 mmol/kg6;. 1995 1995年一例年一例7474岁老年女性，急性起病，左舞蹈动作。血糖岁老年女性，急性起病，左舞蹈动作。血

11、糖296mg/dl296mg/dl，血渗透压，血渗透压296mOmsm/L.296mOmsm/L.尿酮阴性，尿糖阳性。舞蹈动作持续了尿酮阴性，尿糖阳性。舞蹈动作持续了3737天，天，T1T1高信号、高信号、T2T2低信号持续低信号持续1010个月个月消失。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异常信号的原因。异常信号的原因。 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，但有强化，强化范围同信号同前例，但有强化，强化范围同T1T1异常信异常信号区域。推测号区域。推测

12、BBBBBB破坏在先，然后形成类似破坏在先，然后形成类似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：多灶性小梗死灶、反应性胶质增生、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（神经元间反应（interneuronal response. interneuronal response. ）7;. 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。例为以前未发现患糖尿

13、病。症状持续例。诱因及症状均同前。例为以前未发现患糖尿病。症状持续6 6月到月到5 5年，病年，病程程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疑问，最终推测为进展性梗死，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并与星形细胞反应性增生有关8;. 2002 2002年，年，Oh, S. HOh, S. H等综述了等综述了19851985年年20012001年间报道的年间报道的5353例（包括报道新

14、发例病例（包括报道新发例病人）并进行了人）并进行了MetaMeta分析，指出了分析，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30/1730/17），），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

15、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。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。2222例随访显示，症状与影像学同步性逐渐缓解。例随访显示，症状与影像学同步性逐渐缓解。3939例痊愈，例痊愈，1414例好转。例好转。7 7例症状复发。例症状复发。9;.10;. CT showed an increased density in the contralateral putamen and/or CT showed an increased density in the contralateral putamen and/or caudate

16、 caudate MRI revealed abnormal hyperintensity on T1-weighted and MRI revealed abnormal hyperintensity on T1-weighted and hypointensity on T2-weighted images hypointensity on T2-weighted images The striatal hyperdensity in the brain CT completely resolved within The striatal hyperdensity in the brain

17、 CT completely resolved within 3 months and in 6 months on MRI.3 months and in 6 months on MRI.11;.A review revealed a total of 35 casesA review revealed a total of 35 cases There was no gender difference and the average age at the on-set of There was no gender difference and the average age at the

18、on-set of dyskinesia was 72 years.dyskinesia was 72 years. Prognosis of all the reported cases was excellent and their hyperkinetic Prognosis of all the reported cases was excellent and their hyperkinetic states all abruptly ceased after hyperglycemic control had been achieved. states all abruptly c

19、eased after hyperglycemic control had been achieved. Twelve cases had follow up neuroimaging examination. These showed Twelve cases had follow up neuroimaging examination. These showed complete resolution within 11 months in 9 cases, partial resolution after 6 complete resolution within 11 months in

20、 9 cases, partial resolution after 6 months in 1 case, and no change was seen in 2 cases 6 months later.months in 1 case, and no change was seen in 2 cases 6 months later.12;.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/L13;.14;.15;.The nature of the characteristic CT/MRI signal change

21、sThe nature of the characteristic CT/MRI signal changes still debated still debated Based on the evolution of clinical manifestations and the findings of the Based on the evolution of clinical manifestations and the findings of the neuroimages, Chang et al. suggested that putaminal petechial hemorrh

22、age might neuroimages, Chang et al. suggested that putaminal petechial hemorrhage might be the pathological mechanism .be the pathological mechanism . among neuronal subtypes, striatal medium spiny neurones are highly among neuronal subtypes, striatal medium spiny neurones are highly vulnerable to e

23、nergy depletion. The hypothesis of a reversible metabolic vulnerable to energy depletion. The hypothesis of a reversible metabolic impairment may explain the transient MRI alterations.impairment may explain the transient MRI alterations.16;.17;. studies by SPECT and PET have revealed the reduction o

24、f blood flow and studies by SPECT and PET have revealed the reduction of blood flow and metabolism in the contralateral striatummetabolism in the contralateral striatum MR spectroscopy has also demonstrated the presence of pronounced energy MR spectroscopy has also demonstrated the presence of prono

25、unced energy depletion and neuronal dysfunction in the contralateral striatumdepletion and neuronal dysfunction in the contralateral striatum proton MRI spectroscopy and diffusion weighted MRI studies suggest a proton MRI spectroscopy and diffusion weighted MRI studies suggest a hyperviscosity syndr

26、ome, possibly caused by hyperglycaemia, and hyperviscosity syndrome, possibly caused by hyperglycaemia, and concomitant cytotoxic edema could be the cause of the MRI changes.concomitant cytotoxic edema could be the cause of the MRI changes.18;. MRI MRI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的信号变化可以用该部位的点状出

27、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T1T1像缩短像缩短 19;.biopsy specimen from the hyperintense putamenbiopsy specimen from the hyperintense putamen revealed a slight atrocytosis a

28、nd vacuolization or a fragment of gliotic revealed a slight atrocytosis and vacuolization or a fragment of gliotic brain tissue with abundant gemistocytes, but was without deposition of brain tissue with abundant 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

29、质细胞增生和空泡形成，而没有铁或钙的沉积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MRIMRI短短T1T1信号是由于肿胀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的原浆性星形胶质细胞中蛋白水化层所致 20;.21;. 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的N-N-乙酰天冬氨酸（乙