Journal of Neurology

, Volume 259, Issue 3, pp 491–496

The EEG in E200K familial CJD: relation to MRI patterns

  • Shmuel A. Appel
  • Joab Chapman
  • Isak Prohovnik
  • Chen Hoffman
  • Oren S. Cohen
  • Ilan Blatt
Original Communication


The aim of the study was to examine the relationship between EEG abnormalities and the pattern of MRI changes in familial Creutzfeldt–Jakob Disease (fCJD) patients with E200K mutation. As part of a controlled, prospective study, 13 E200K fCJD patients underwent comprehensive evaluations, with EEG and an extensive MRI protocol that included one of the most prion-disease sensitive sequences, diffusion-weighted imaging (DWI). The relationship between EEG abnormalities and the pattern of DWI hyperintensities was examined. EEG demonstrated the classical CJD finding of PSWC (periodic sharp wave complexes) in five patients (38%) while in eight patients (62%) the EEG showed only slow activity. Six patients showed the typical cortical changes on MRI, and in five of them (83%) concordance between the MRI and the EEG was found. Five patients had isolated basal ganglia involvement per MRI, and in two of them (40%) concordance between the MRI and the EEG laterality was found. In the remaining two patients MRI did not show any changes suggesting CJD and EEG showed focal slow activity. The EEG of our E200K fCJD patients appears similar to that of the largest prion disease patient group, sporadic CJD (sCJD). EEG abnormalities in E200K fCJD appear to correlate mainly with cortical pathology, as revealed by DWI, rather than basal ganglia pathology. The observation that PSWC abnormalities reflect cortical rather than basal ganglia pathology is significant with respect to theories of the origins of EEG abnormalities in prion disease.


Creutzfeldt–Jakob Disease MRI EEG 


  1. 1.
    Eggenberger E (2007) Prion disease. Neurol Clin 25:833–842PubMedCrossRefGoogle Scholar
  2. 2.
    Knight RS, Will RG (2004) Prion diseases. J Neurol Neurosurg Psychiatry 75(Suppl 1):36–42CrossRefGoogle Scholar
  3. 3.
  4. 4.
    Otto M, Wiltfang J, Cepek L et al (2002) Tau protein and 14-3-3 protein in the differential diagnosis of Creutzfeldt–Jakob disease. Neurology 58:192–197PubMedGoogle Scholar
  5. 5.
    Wang GR, Gao C, Shi Q et al (2010) Elevated levels of tau protein in cerebrospinal fluid of patients with probable Creutzfeldt–Jakob disease. Am J Med Sci 340:291–295PubMedCrossRefGoogle Scholar
  6. 6.
    Hansen HC, Zschocke S, Sturenburg HJ, Kunze K (1998) Clinical changes and EEG patterns preceding the onset of periodic sharp wave complexes in Creutzfeldt–Jakob disease. Acta Neurol Scand 97:99–106PubMedCrossRefGoogle Scholar
  7. 7.
    Wieser HG, Schindler K, Zumsteg D (2006) EEG in Creutzfeldt–Jakob disease. Clin Neurophysiol 117:935–951PubMedCrossRefGoogle Scholar
  8. 8.
    Levy SR, Chiappa KH, Burke CJ, Young RR (1986) Early evolution and incidence of electroencephalographic abnormalities in Creutzfeldt–Jakob disease. J Clin Neurophysiol 3:1–21PubMedGoogle Scholar
  9. 9.
    Steinhoff BJ, Zerr I, Glatting M, Schulz-Schaeffer W, Poser S, Kretzschmar HA (2004) Diagnostic value of periodic complexes in Creutzfeldt–Jakob disease. Ann Neurol 56:702–708PubMedCrossRefGoogle Scholar
  10. 10.
    Gertz HJ, Henkes H, Cervos-Navarro J (1988) Creutzfeldt–Jakob disease: correlation of MRI and neuropathologic findings. Neurology 38:1481–1482PubMedGoogle Scholar
  11. 11.
    Tian HJ, Zhang JT, Lang SY, Wang XQ (2010) MRI sequence findings in sporadic Creutzfeldt–Jakob disease. J Clin Neurosci 17:1378–1380PubMedCrossRefGoogle Scholar
  12. 12.
    Shiga Y, Miyazawa K, Sato S et al (2004) Diffusion-weighted MRI abnormalities as an early diagnostic marker for Creutzfeldt–Jakob disease. Neurology 63:443–449PubMedGoogle Scholar
  13. 13.
    Young GS, Geschwind MD, Fischbein NJ et al (2005) Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt–Jakob disease: high sensitivity and specificity for diagnosis. Am J Neuroradiol 26:1551–1562PubMedGoogle Scholar
  14. 14.
    Zerr I, Poser S (2002) Clinical diagnosis and differential diagnosis of CJD and vCJD. With special emphasis on laboratory tests. APMIS 110:88–98PubMedCrossRefGoogle Scholar
  15. 15.
    Fulbright RK, Hoffmann C, Lee H, Pozamantir A, Chapman J, Prohovnik I (2008) MR imaging of familial Creutzfeldt–Jakob disease: a blinded and controlled study. Am J Neuroradiol 29:1638–1643PubMedCrossRefGoogle Scholar
  16. 16.
    Meissner B, Kallenberg K, Sanchez-Juan P et al (2009) MRI lesion profiles in sporadic Creutzfeldt–Jakob disease. Neurology 72:1994–2001PubMedCrossRefGoogle Scholar
  17. 17.
    Vitali P, Maccagnano E, Caverzasi E et al (2011) Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias. Neurology 76(20):1711–1719PubMedCrossRefGoogle Scholar
  18. 18.
    Zeidler M, Stewart GE, Barraclough CR et al (1997) New variant Creutzfeldt–Jakob disease: neurological features and diagnostic tests. Lancet 350:903–907PubMedCrossRefGoogle Scholar
  19. 19.
    Na DL, Suh CK, Choi SH et al (1999) Diffusion-weighted magnetic resonance imaging in probable Creutzfeldt–Jakob disease: a clinical-anatomic correlation. Arch Neurol 56:951–957PubMedCrossRefGoogle Scholar
  20. 20.
    Cambier DM, Kantarci K, Worrell GA, Westmoreland BF, Aksamit AJ (2003) Lateralized and focal clinical, EEG, and FLAIR MRI abnormalities in Creutzfeldt–Jakob disease. Clin Neurophysiol 114:1724–1728PubMedCrossRefGoogle Scholar
  21. 21.
    Kandiah N, Tan K, Pan AB et al (2008) Creutzfeldt–Jakob disease: which diffusion-weighted imaging abnormality is associated with periodic EEG complexes? J Neurol 255:1411–1414PubMedCrossRefGoogle Scholar
  22. 22.
    Mizobuchi M, Tanaka C, Sako K, Nihira A, Abe T, Shirasawa A (2008) Correlation between periodic sharp wave complexes and diffusion-weighted magnetic resonance images in early stage of Creutzfeldt–Jakob disease: a report of two cases. Seizure 17:717–722PubMedCrossRefGoogle Scholar
  23. 23.
    Kovacs GG, Seguin J, Quadrio I (2011) Genetic Creutzfeldt–Jakob disease associated with the E200K mutation: characterization of a complex proteinopathy. Acta Neuropathol 121:39–57PubMedCrossRefGoogle Scholar
  24. 24.
    Kovacs GG, Puopolo M, Ladogana A et al (2005) Genetic prion disease: the EUROCJD experience. Hum Genet 118:166–174PubMedCrossRefGoogle Scholar
  25. 25.
    Korczyn AD (1991) Creutzfeldt–Jakob disease among Libyan Jews. Eur J Epidemiol 7:490–493PubMedCrossRefGoogle Scholar
  26. 26.
    Wu HM, Lu CS, Huang CC et al (2010) Asymmetric involvement in sporadic Creutzfeldt–Jakob disease: clinical, brain imaging, and electroencephalographic studies. Eur Neurol 64:74–79PubMedCrossRefGoogle Scholar
  27. 27.
    Neufeld MY, Korczyn AD (1992) Topographic distribution of the periodic discharges in Creutzfeldt–Jakob disease (CJD). Brain Topogr 4:201–206PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  • Shmuel A. Appel
    • 1
  • Joab Chapman
    • 1
  • Isak Prohovnik
    • 2
  • Chen Hoffman
    • 3
  • Oren S. Cohen
    • 1
  • Ilan Blatt
    • 1
  1. 1.Department of Neurology, The Sagol Neuroscience Center, and Chaim Sheba Medical Center affiliated to the Sackler Faculty of MedicineTel-Aviv UniversityTel-HashomerIsrael
  2. 2.Departments of Psychiatry and RadiologyMount Sinai School of MedicineNew YorkUSA
  3. 3.Department of Diagnostic Imaging, Chaim Sheba Medical Center affiliated to the Sackler Faculty of MedicineTel-Aviv UniversityTel-HashomerIsrael

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