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获得性肝脑变性的临床与影像学特点 被引量:2

The characteristics of the clinical and imaging findings in acquired hepatocerebral degeneration
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摘要 目的分析获得性肝脑变性的临床和影像学特征,探讨其规律性。方法分析9例获得性肝脑变性病例的临床表现、实验室检查和影像学改变的数据。结果5例既往有明确的肝硬化病史,4例没有明确的肝病病史,发病后检查分别发现肝血管病、肝胰脾等弥漫钙化或者肝硬化。主要临床表现为:精神异常、认知能力下降、帕金森病样综合征、构音障碍、共济失调、睡眠障碍。肝酶学轻中度异常,而血氨均升高。脑电图提示脑电活动呈弥漫性减慢,4例出现三相波节律。影像学改变为苍白球、大脑脚、小脑和大脑皮层下对称性短T_1加权像信号,以及基底节、中脑、脑桥、延髓橄榄核对称性长T_2加权像信号。结论获得性肝脑变性是肝细胞和肝血管病变引起的神经功能损害综合征,肝酶改变、血氨升高和脑电图符合代谢性疾病改变,结合特征性影像学改变,对疾病的诊断具有重要意义。 Objective To explore the regularities of acquired hepatoeerebral degeneration (AHD) by analyzing the clinical and neuroimaging characteristics. Methods The data of clinical manifestation,laboratory examination and neuroimaging findings in 9 cases of AHD had been collected. Results 5 cases had definite previous cirrhosis,while 4 cases had no previous chronic liver disease,which afterward turned out to be hepato-vascular disease, diffuse calcification of liver ,pancreas and spleen ,and cirrhosis. The main clinical manifestation included mental disturbance,cognitive abilities decline, Parkinson-like syndrome,dysarthria,ataxia,and sleep disorder. Hepato-enzymology revealed mild or moderate elevation,and all cases had high level of blood ammonia. EEG showed diffuse slow electrical activity of brain,4 of which had triphasic wave. Magnetic resonance imaging of the brain revealed symmetrical high T1signals in the bilateral globus pallidus ,cerebral peduncle,cerebellum and subcortex and T2highsignal intensities in the bilateral basal ganglia,midbrain,pons,and medulla olivary nucleus. Conclusion AHD is a neurologic syndrome associated with hepatocellular degeneration and hepato-vascular diseases. Hepato-enzymologic changes ,elevated level of blood ammonia ,EEG consistent with metabolic disorder ,combined with typical manifestation of neuroimaging,have pivotal significance in the diagnosis of AHD.
出处 《中风与神经疾病杂志》 CAS CSCD 北大核心 2007年第1期61-63,共3页 Journal of Apoplexy and Nervous Diseases
关键词 获得性肝脑变性 临床表现 影像学 Acquired hepatocerebral degeneration Clinical manifestation Neuroimaging
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参考文献6

  • 1Adams RD,Victor M.Acquired (non-Wilsonian) hcpatocerebral degeneration[A].In:Barry BK,Schwartz M,Scott EJ,cds,Principles of Neurology[M].3rd ed.New York,NY:McGraw-Hill,1985.800 802.
  • 2Lee J,Lacomis D,Comu S,et al.Acquired hepatocercbral degen eration:MR and pathologic findings[J].Am J Neuroradiol,1998,19:485-487.
  • 3Jog MS,Lang AE.Chronic acquired hepatocerebral degenera tion:caste repors and new insights[J].Mov Disord,1995,10:714 722.
  • 4Inoue E,Hori S,Narumi Y,et al.Portal systemic encephalopathy:presence of basal ganglia lesions with high signal intensity on MR images[J].Radiology,1991,179:551-555
  • 5Hitoshi S,Terao Y,Mizuno T,et al.A case of portal-systemic encephalopathy presenting characteristic MR images in globus pallidus,hypothalamus,corpus callosum,pontine base,and middle cerebellar peduncle[J].Clin Neurol,1992,32:217-219
  • 6Hauser RA,Zesiewicz TA,Rosemurgy AS,et al.Manganese intoxication and chronic liver failure[J].Ann Neurol,1994,36:871-875.

同被引文献9

  • 1Ferrara J, Jankovic J. Acquired hepatocerebral degeneration [J]. J Neurol,2009,256:20-332.
  • 2Cordoba J, Sanpedro F, Alonso J, et al. ^1 H magnetic resonance in the study of hepatic encephaiopathy in human [J]. Metab Brain Dis,2002,17 (4) :415-429.
  • 3Krieger D, Krieger S, Jansen O, et al. Manganese and chronic hepatic encephalopathy[J]. Lancet, 1995,346:270-274.
  • 4Papapetropoulos S, Tzakis A, SengunC, et al. Case of pediatric acquired chronic hepatocerebral degeneration[J]. Pediatr Neurol,2008,38:67 -70.
  • 5Stracciari A, Mattarozzi K, D' Alessandro R, et al. Cognitive functioning in chronic acquired hepatoeerebral degeneration [J]. Metab Brain Dis,2008,23 : 155-160.
  • 6杨晓萍,马强华,叶建军,陈纲,许斐,周怀阳,朱晓华.获得性肝性脑部变性的MRI表现[J].实用放射学杂志,2007,23(10):1305-1307. 被引量:3
  • 7宋胜斌,王玮,周俊英.获得性肝脑变性的研究新进展[J].国际内科学杂志,2008,35(12):727-729. 被引量:2
  • 8马玲玲,李华.获得性肝性脑部变性的临床与影像学特点[J].中国实用神经疾病杂志,2011,14(16):49-50. 被引量:2
  • 9金延方,岳云龙,贺聪,尹南,杨磊.获得性肝性脑部变性的MRI表现[J].中华放射学杂志,2000,34(12):841-843. 被引量:19

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