脑电图在克雅氏病中特征及诊断价值_Journal of Epilepsy

Authors：

康慧聪 ¹ ^* , 李存 ¹ ^* , 习新聪 ² ,  朱遂强 ¹

1. ;
2. ;

Corresponding author：

朱遂强, Email: zhusuiqiang@163.com

Keywords：

克雅氏病; 脑电图; 周期性尖慢复合波; 临床诊断标志物

DOI：

10.7507/2096-0247.20180068

Video：

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脑电图（EEG）是克雅氏病 (Creutzfeldt-Jakob disease，CJD) 临床诊断的重要辅助检查，反应了不同疾病阶段非特异性脑功能改变及特征性异常。CJD 的 EEG 改变随病程阶段而变化，早期主要表现为非特异性弥漫性慢波及额部节律性 δ 活动，中期则出现特征性的周期性尖慢复合波（Periodic sharp wave complexes，PSWC），至晚期则再次呈现非典型特征表现为类似昏迷状态的慢波及低电压活动。因此具有诊断价值的 PSWC 与检测时间密切相关，其敏感性一般但特异性较高，特别是对散发性 CJD（Sporadic CJD，sCJD），与朊蛋白基因 129 密码子的多态性相关，都见于 MM 及 MV 型患者。遗传性 CJD 中 PSWC 仅见于 10% 患者，医源性 CJD 中 PSWC 与接触部位明确相关，变异性 CJD 中无 PSWC 报道。总体而言 EEG 是 CJD 临床诊断的重要标准，且可从一定程度上提示疾病的临床分期，对疑诊 CJD 的患者定期复查监测至关重要。

Citation： 康慧聪, 李存, 习新聪, 朱遂强. 脑电图在克雅氏病中特征及诊断价值. Journal of Epilepsy, 2018, 4(5): 411-414. doi: 10.7507/2096-0247.20180068 Copy

1.	Geschwind MD. Prion disease. Continuum(Minneap Minn), 2005, 21(6): 1612-1638.
2.	Zerr I, Poser S. Clinical diagnosis and differential diagnosis of CJD and vCJD with special emphasis on laboratory tests. APMIS, 2002, 110(1): 88-98.
3.	Steinhoff BJ, Zerr I, Glatting M, et al. Diagnostic value of periodic complexes in Creutzfeldt-Jakob disease. Ann Neurol, 2004, 56(5): 702-708.
4.	World Health Organization. Global Surveillance, diagnosis, and therapy of human transmissible spongiform encephalopathies: Report of a WHO consultation. 1998, Geneva, Switzerland.
5.	Gloor P. EEG characteristics in Creutzfeldt-Jakob disease. Ann Neurol, 1980, 8(3): 341.
6.	Wieser HG, Schwarz U, Blattler T, et al. Serial EEG findings in sporadic and iatrogenic Creutzfeldt-Jakob disease. Clin Neurophysiol, 2004, 115(11): 2467-2478.
7.	Terzano MG, Parrino L, Pietrini V, et al. Precocious loss of physiological sleep in a case of Creutzfeldt-Jakob disease: A serial polygraphic study. Sleep, 1995, 18(10): 849-858.
8.	Sujith A, Udaya S. Electroencephalographic changes in sporadic Creutzfeldt-Jakob disease and correlation with clinical stages: A retrospective analysis. J Clin Neurophysiol, 2014, 31(6): 586-593.
9.	Heinz GW, Urs S, Thomas B, et al. Serial EEG findings in sporadic and iatrogenic Creutzfeldt-Jakob disease. Clinical Neurophysiol, 2004, 115(11): 2467-2478.
10.	Hill AF. Molecular classification of sporadic Creutzfeldt-Jakob disease. Brain, 2003, 126(Pt 6): 1333-1346.
11.	Snodgrass SM, Tsuburaya K, Ajmone-Marsan C. Clinical significance of periodic lateralized epileptiform discharges: Relationship with status epilepticus. J Clin Neurophysiol, 1989, 6(2): 159-72.
12.	Hansen HC, Zschocke S, Sturenburg HJ, et al. Clinical changes and EEG patterns preceding the onset of periodic sharp wave complexes in Creutzfeldt-Jakob disease. Acta Neurol Scand, 1998, 97(2): 99-106.
13.	Schlenska GK, Walter GF. Temporal evolution of electroencephalographic abnormalities in Creutzfeldt-Jakob disease. J Neurol, 1989, 236(8): 456-460.
14.	Lee RG, Blair RD. Evolution of EEG and visual evoked response changes in Jakob-Creutzfeldt disease. Electroencephalogr Clin Neurophysiol, 1973, 35(2): 133-142.
15.	Aguglia U, Gambardella A, Le Piane E, et al. Disappearance of periodic sharp wave complexes in Creutzfeldt-Jakob disease. Neurophysiol Clin, 1997, 27(4): 277-282.
16.	Asai Y, Shimoda M, Sasaki K, et al. Alpha-like activity in terminal stage of Creutzfeldt-Jakob disease. Acta Neurol Scand, 2001, 104(2): 118-122.
17.	Johnson RT, Gibbs CJ. Creutzfeldt-Jakob disease and related transmissible spongiform encephalopathies. N Engl J Med, 1998, 339(27): 1994-2004.
18.	Roos R, Gajdusek DC, Gibbs Jr CJ. The clinical characteristics of transmissible Creutzfeldt-Jakob disease. Brain, 1973, 96(1): 1-20.
19.	Aronyk K, Petito F, Solomon GE. Partial elementary motor seizures as the first symptom of Creutzfeldt-Jakob disease. Ann Neurol, 1984, 15(2): 210-211.
20.	Cohen D, Kutluay E, Edwards J, et al. Sporadic Creutzfeldt-Jakob disease presenting with non-convulsive status epilepticus. Epilepsy Behav, 2004, 5(5): 792-796.
21.	Fernandez-Torre JL, Solar DM, Astudillo A, et al. Creutzfeldt-Jakob disease and non-convulsive status epilepticus: a clinical and electroencephalographic follow-up study. Clin Neurophysiol, 2004, 115(2): 316-319.
22.	Shapiro JM, Shujaat A, Wang J, et al. Creutzfeldt-Jakob disease presenting as refractory nonconvulsive status epilepticus. J Intensive Care Med, 2004, 19(6): 345-348.
23.	Justus M, Susanne K, Adam S, et al. Periodic EEG patterns in sporadic Creutzfeld-Jakob disease can be benzodiazepine responsive and be difficult to distinguish from non-convulsive status epilepticus. Seizure, 2017, 53: 47-50.
24.	Fushimi M, Sato K, Shimizu T, et al. PLEDs in Creutzfeldt-Jakob disease following a cadaveric dural graft. Clin Neurophysiol, 2002, 113(7): 1030-1035.
25.	Bernoulli C, Siegfried J, Baumgartner G, et al. Danger of accidental person-to-person transmission of Creutzfeldt-Jakob disease by surgery. Lancet, 1977, 26(8009): 478-479.
26.	Kovacs GG, Puopolo M, Ladogana A, et al. Genetic prion disease: The EUROCJD experience. Hum Genet, 2005, 118(2): 166-174.
27.	Tietjen GE, Drury I. Familial Creutzfeldt-Jakob disease without periodic EEG activity. Ann Neurol, 1990, 28(4): 585-588.
28.	Neufeld MY, Talianski-Aronov A, Soffer D, et al. Generalized convulsive status epilepticus in Creutzfeldt-Jakob disease. Seizure, 2003, 12(6): 403-405.

1. Geschwind MD. Prion disease. Continuum(Minneap Minn), 2005, 21(6): 1612-1638.
2. Zerr I, Poser S. Clinical diagnosis and differential diagnosis of CJD and vCJD with special emphasis on laboratory tests. APMIS, 2002, 110(1): 88-98.
3. Steinhoff BJ, Zerr I, Glatting M, et al. Diagnostic value of periodic complexes in Creutzfeldt-Jakob disease. Ann Neurol, 2004, 56(5): 702-708.
4. World Health Organization. Global Surveillance, diagnosis, and therapy of human transmissible spongiform encephalopathies: Report of a WHO consultation. 1998, Geneva, Switzerland.
5. Gloor P. EEG characteristics in Creutzfeldt-Jakob disease. Ann Neurol, 1980, 8(3): 341.
6. Wieser HG, Schwarz U, Blattler T, et al. Serial EEG findings in sporadic and iatrogenic Creutzfeldt-Jakob disease. Clin Neurophysiol, 2004, 115(11): 2467-2478.
7. Terzano MG, Parrino L, Pietrini V, et al. Precocious loss of physiological sleep in a case of Creutzfeldt-Jakob disease: A serial polygraphic study. Sleep, 1995, 18(10): 849-858.
8. Sujith A, Udaya S. Electroencephalographic changes in sporadic Creutzfeldt-Jakob disease and correlation with clinical stages: A retrospective analysis. J Clin Neurophysiol, 2014, 31(6): 586-593.
9. Heinz GW, Urs S, Thomas B, et al. Serial EEG findings in sporadic and iatrogenic Creutzfeldt-Jakob disease. Clinical Neurophysiol, 2004, 115(11): 2467-2478.
10. Hill AF. Molecular classification of sporadic Creutzfeldt-Jakob disease. Brain, 2003, 126(Pt 6): 1333-1346.
11. Snodgrass SM, Tsuburaya K, Ajmone-Marsan C. Clinical significance of periodic lateralized epileptiform discharges: Relationship with status epilepticus. J Clin Neurophysiol, 1989, 6(2): 159-72.
12. Hansen HC, Zschocke S, Sturenburg HJ, et al. Clinical changes and EEG patterns preceding the onset of periodic sharp wave complexes in Creutzfeldt-Jakob disease. Acta Neurol Scand, 1998, 97(2): 99-106.
13. Schlenska GK, Walter GF. Temporal evolution of electroencephalographic abnormalities in Creutzfeldt-Jakob disease. J Neurol, 1989, 236(8): 456-460.
14. Lee RG, Blair RD. Evolution of EEG and visual evoked response changes in Jakob-Creutzfeldt disease. Electroencephalogr Clin Neurophysiol, 1973, 35(2): 133-142.
15. Aguglia U, Gambardella A, Le Piane E, et al. Disappearance of periodic sharp wave complexes in Creutzfeldt-Jakob disease. Neurophysiol Clin, 1997, 27(4): 277-282.
16. Asai Y, Shimoda M, Sasaki K, et al. Alpha-like activity in terminal stage of Creutzfeldt-Jakob disease. Acta Neurol Scand, 2001, 104(2): 118-122.
17. Johnson RT, Gibbs CJ. Creutzfeldt-Jakob disease and related transmissible spongiform encephalopathies. N Engl J Med, 1998, 339(27): 1994-2004.
18. Roos R, Gajdusek DC, Gibbs Jr CJ. The clinical characteristics of transmissible Creutzfeldt-Jakob disease. Brain, 1973, 96(1): 1-20.
19. Aronyk K, Petito F, Solomon GE. Partial elementary motor seizures as the first symptom of Creutzfeldt-Jakob disease. Ann Neurol, 1984, 15(2): 210-211.
20. Cohen D, Kutluay E, Edwards J, et al. Sporadic Creutzfeldt-Jakob disease presenting with non-convulsive status epilepticus. Epilepsy Behav, 2004, 5(5): 792-796.
21. Fernandez-Torre JL, Solar DM, Astudillo A, et al. Creutzfeldt-Jakob disease and non-convulsive status epilepticus: a clinical and electroencephalographic follow-up study. Clin Neurophysiol, 2004, 115(2): 316-319.
22. Shapiro JM, Shujaat A, Wang J, et al. Creutzfeldt-Jakob disease presenting as refractory nonconvulsive status epilepticus. J Intensive Care Med, 2004, 19(6): 345-348.
23. Justus M, Susanne K, Adam S, et al. Periodic EEG patterns in sporadic Creutzfeld-Jakob disease can be benzodiazepine responsive and be difficult to distinguish from non-convulsive status epilepticus. Seizure, 2017, 53: 47-50.
24. Fushimi M, Sato K, Shimizu T, et al. PLEDs in Creutzfeldt-Jakob disease following a cadaveric dural graft. Clin Neurophysiol, 2002, 113(7): 1030-1035.
25. Bernoulli C, Siegfried J, Baumgartner G, et al. Danger of accidental person-to-person transmission of Creutzfeldt-Jakob disease by surgery. Lancet, 1977, 26(8009): 478-479.
26. Kovacs GG, Puopolo M, Ladogana A, et al. Genetic prion disease: The EUROCJD experience. Hum Genet, 2005, 118(2): 166-174.
27. Tietjen GE, Drury I. Familial Creutzfeldt-Jakob disease without periodic EEG activity. Ann Neurol, 1990, 28(4): 585-588.
28. Neufeld MY, Talianski-Aronov A, Soffer D, et al. Generalized convulsive status epilepticus in Creutzfeldt-Jakob disease. Seizure, 2003, 12(6): 403-405.

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