腦電圖在克雅氏病中特征及診斷價值_Journal of Epilepsy

Authors：

康慧聰 ¹ ^* , 李存 ¹ ^* , 習新聰 ² ,  朱遂強 ¹

1. ;
2. ;

Corresponding?author：

朱遂強, Email: zhusuiqiang@163.com

Keywords：

克雅氏病; 腦電圖; 周期性尖慢復合波; 臨床診斷標志物

DOI：

10.7507/2096-0247.20180068

Video：

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腦電圖（EEG）是克雅氏病 (Creutzfeldt-Jakob disease，CJD) 臨床診斷的重要輔助檢查，反應了不同疾病階段非特異性腦功能改變及特征性異常。CJD 的 EEG 改變隨病程階段而變化，早期主要表現為非特異性彌漫性慢波及額部節律性 δ 活動，中期則出現特征性的周期性尖慢復合波（Periodic sharp wave complexes，PSWC），至晚期則再次呈現非典型特征表現為類似昏迷狀態的慢波及低電壓活動。因此具有診斷價值的 PSWC 與檢測時間密切相關，其敏感性一般但特異性較高，特別是對散發性 CJD（Sporadic CJD，sCJD），與朊蛋白基因 129 密碼子的多態性相關，都見于 MM 及 MV 型患者。遺傳性 CJD 中 PSWC 僅見于 10% 患者，醫源性 CJD 中 PSWC 與接觸部位明確相關，變異性 CJD 中無 PSWC 報道。總體而言 EEG 是 CJD 臨床診斷的重要標準，且可從一定程度上提示疾病的臨床分期，對疑診 CJD 的患者定期復查監測至關重要。

Citation： 康慧聰, 李存, 習新聰, 朱遂強. 腦電圖在克雅氏病中特征及診斷價值. 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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