Epilepsy and prion diseases: A narrative review

  • Adan G, Mitchell JW, Ziso B, Larner AJ. Diagnosis and management of seizures in neurodegenerative diseases. Curr Treat Opt Neurol. 2020;22 (In press).

  • Epileptic seizures in neurodegenerative dementia syndromes.

    J Neurol Neurosci. 2010; 1: 3

  • Vercueil L. Epilepsy and neurodegenerative diseases in adults: a clinical review. Epileptic Disord. 2006;8:S44-S54. PubMed PMID: WOS:000239015100006.

  • Biology and genetics of prions causing neurodegeneration.

    Annu Rev Genet. 2013; 47 (): 601-623https://doi.org/10.1146/annurev-genet-110711-155524

    • Nakatani E.
    • Kanatani Y.
    • Kaneda H.
    • Nagai Y.
    • Teramukai S.
    • Nishimura T.
    • et al.

    Specific clinical signs and symptoms are predictive of clinical course in sporadic Creutzfeldt-Jakob disease.

    Eur J Neurol. 2016; 23 (): 1455-1462https://doi.org/10.1111/ene.13057

    • Baiardi S.
    • Capellari S.
    • Stella A.B.
    • Parchi P.

    Unusual clinical presentations challenging the early clinical diagnosis of Creutzfeldt-Jakob disease.

    J Alzheimers Dis. 2018; 64 (): 1051-1065https://doi.org/10.3233/Jad-180123

    • Zerr I.
    • Kallenberg K.
    • Summers D.M.
    • Romero C.
    • Taratuto A.
    • Heinemann U.
    • et al.

    Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease.

    Brain. 2009; 132 (): 2659-2668https://doi.org/10.1093/brain/awp191

  • Zerr I, Kallenberg K, Summers DM, Romero C, Taratuto A, Heinemann U, et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease (vol 132, pg 2659, 2009). Brain 2012;135:1335-. doi: 10.1093/brain/awr029. PubMed PMID: WOS:000302948700034.

  • Brown P, Cathala F, Castaigne P, Gajdusek DC. Creutzfeldt-Jakob disease – clinical analysis of a consecutive series of 230 neuropathologically verified cases. Ann Neurol. 1986;20(5):597-602. doi: DOI 10.1002/ana.410200507. PubMed PMID: WOS:A1986E726200006.

    • Brown P.
    • Goldfarb L.G.
    • Gibbs C.J.
    • Gajdusek D.C.

    The phenotypic-expression of different mutations in transmissible familial Creutzfeldt-Jakob disease.

    Eur J Epidemiol. 1991; 7 (): 469-476https://doi.org/10.1007/Bf00143124

    • Poser S.
    • Mollenhauer B.
    • Krauss A.
    • Zerr I.
    • Steinhoff B.J.
    • Schroeter A.
    • et al.

    How to improve the clinical diagnosis of Creutzfeldt-Jakob disease.

    Brain. 1999; 122 (): 2345-2351https://doi.org/10.1093/brain/122.12.2345

    • Wieser H.G.
    • Schindler K.
    • Zumsteg D.

    EEG in Creutzfeldt-Jakob disease.

    Clin Neurophysiol. 2006; 117 (): 935-951https://doi.org/10.1016/j.clinph.2005.12.007

    • Appel S.
    • Chapman J.
    • Cohen O.S.
    • Rosenmann H.
    • Nitsan Z.
    • Blatt I.

    Seizures in E200K familial and sporadic Creutzfeldt-Jakob disease.

    Acta Neurol Scand. 2015; 131 (): 152-157https://doi.org/10.1111/ane.12304

    • Parchi P.
    • Giese A.
    • Capellari S.
    • Brown P.
    • Schulz-Schaeffer W.
    • Windl O.
    • et al.

    Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects.

    Ann Neurol. 1999; 46 (): 224-233https://doi.org/10.1002/1531-8249(199908)46:2<224::Aid-Ana12>3.0.Co;2-W

  • Gambetti P, Kong QZ, Zou WQ, Parchi P, Chen SG. Sporadic and familial CJD: classification and characterisation. Brit Med Bull. 2003;66:213-+. doi: 10.1093/bmb/66.1.213. PubMed PMID: WOS:000185486900017.

    • Krasnianski A.
    • Meissner B.
    • Schulz-Schaeffer W.
    • Kallenberg K.
    • Bartl M.
    • Heinemann U.
    • et al.

    Clinical features and diagnosis of the MM2 cortical subtype of sporadic Creutzfeldt-Jakob disease.

    Arch Neurol-Chicago. 2006; 63 (): 876-880https://doi.org/10.1001/archneur.63.6.876

  • Zerr I. Clinical and therapeutic aspects of prion disease. In: Duyckaerts C, Litvan. I., editors. Handbook of clinical neurology Dementias. Dementias. 89 Edinburgh: Elsevier; 2008. p. 737-64.

    • Bagyinszky E.
    • Giau V.V.
    • Youn Y.C.
    • An S.S.A.
    • Kim S.

    Characterization of mutations in PRNP (prion) gene and their possible roles in neurodegenerative diseases.

    Neuropsych Dis Treat. 2018; 14 (): 2067-2085https://doi.org/10.2147/Ndt.S165445

    • Zilber N.
    • Kahana E.
    • Abraham M.

    The Libyan Creutzfeldt-Jakob disease focus in Israel – an epidemiologic evaluation.

    Neurology. 1991; 41 (): 1385-1389https://doi.org/10.1212/Wnl.41.9.1385

    • Chapman J.
    • Brown P.
    • Goldfarb L.G.
    • Arlazoroff A.
    • Gajdusek D.C.
    • Korczyn A.D.

    Clinical heterogeneity and unusual presentations of Creutzfeldt-Jakob-disease in Jewish Patients with the Prnp codon 200 mutation.

    J Neurol Neurosurg Psychiatry. 1993; 56 (): 1109-1112https://doi.org/10.1136/jnnp.56.10.1109

    • Meiner Z.
    • Gabizon R.
    • Prusiner S.B.

    Familial Creutzfeldt-Jakob disease – Codon 200 prion disease in Libyan Jews.

    Medicine. 1997; 76 (): 227-237https://doi.org/10.1097/00005792-199707000-00001

    • Nitsan Z.
    • Cohen O.S.
    • Chapman J.
    • Kahana E.
    • Korczyn A.D.
    • Appel S.
    • et al.

    Familial Creutzfeldt-Jakob disease homozygous to the E200K mutation: clinical characteristics and disease course.

    J Neurol. 2020; 267 (): 2455-2458https://doi.org/10.1007/s00415-020-09826-z

    • Mead S.
    • Gandhi S.
    • Beck J.
    • Caine D.
    • Gallujipali D.
    • Carswell C.
    • et al.

    A novel prion disease associated with diarrhea and autonomic neuropathy.

    N Engl J Med. 2013; 369 (): 1904-1914https://doi.org/10.1056/NEJMoa1214747

    • Colucci M.
    • Moleres F.J.
    • Xie Z.L.
    • Ray-Chaudhury A.
    • Gutti S.
    • Butefisch C.M.
    • et al.

    Gerstmann-Straussler-Scheinker: A new phenotype with ‘curly’ PrP deposits.

    J Neuropath Exp Neur. 2006; 65 (): 642-651https://doi.org/10.1097/01.jnen.0000228198.81797.4d

    • Jansen C.
    • Parchi P.
    • Capellari S.
    • Vermeij A.J.
    • Corrado P.
    • Baas F.
    • et al.

    Prion protein amyloidosis with divergent phenotype associated with two novel nonsense mutations in PRNP.

    Acta Neuropathol. 2010; 119 (): 189-197https://doi.org/10.1007/s00401-009-0609-x

  • Kim MO, Cali I, Oehler A, Fong JC, Wong K, See T, et al. Genetic CJD with a novel E200G mutation in the prion protein gene and comparison with E200K mutation cases. Acta Neuropathol Com. 2013;1. doi: Artn 80 10.1186/2051-5960-1-80. PubMed PMID: WOS:000422387200070.

    • Webb T.E.F.
    • Poulter M.
    • Beck J.
    • Uphill J.
    • Adamson G.
    • Campbell T.
    • et al.

    Phenotypic heterogeneity and genetic modification of P102L inherited prion disease in an international series.

    Brain. 2008; 131 (): 2632-2646https://doi.org/10.1093/brain/awn202

    • Smid J.
    • Neto A.S.
    • Landemberger M.C.
    • Machado C.F.
    • Nobrega P.R.
    • Canedo N.H.S.
    • et al.

    High phenotypic variability in Gerstmann-Straussler-Scheinker disease.

    Arq Neuro-Psiquiat. 2017; 75 (): 331-338https://doi.org/10.1590/0004-282×20170049

  • Montagna P, Cortelli P, Avoni P, Tinuper P, Plazzi G, Gallassi R, et al. Clinical features of fatal familial insomnia: Phenotypic variability in relation to a polymorphism at codon 129 of the prion protein gene. Brain Pathol. 1998;8(3):515-20. PubMed PMID: WOS:000074476100008.

  • Brown P, Brandel JP, Sato T, Nakamura Y, MacKenzie J, Will RG, et al. Iatrogenic Creutzfeldt-Jakob disease, final assessment. Emerg Infect Dis. 2012;18(6):901-7. Epub 2012/05/23. doi: 10.3201/eid1806.120116. PubMed PMID: 22607808; PubMed Central PMCID: PMCPMC3358170.

  • Rudge P, Jaunmuktane Z, Adlard P, Bjurstrom N, Caine D, Lowe J, et al. Iatrogenic CJD due to pituitary-derived growth hormone with genetically determined incubation times of up to 40 years. Brain. 2015;138. doi: 10.1093/brain/awv235. PubMed PMID: WOS:000365135700031.

  • Meissner B, Kallenberg K, Sanchez-Juan P, Ramljak S, Krasnianski A, Heinemann U, et al. MRI and clinical syndrome in dura mater-related Creutzfeldt-Jakob disease. J Neurol. 2009;256(3):355-63. Epub 2009/01/23. doi: 10.1007/s00415-009-0026-z. PubMed PMID: 19159063.

    • Hamaguchi T.
    • Sakai K.
    • Noguchi-Shinohara M.
    • Nozaki I.
    • Takumi I.
    • Sanjo N.
    • et al.

    Insight into the frequent occurrence of dura mater graft-associated Creutzfeldt-Jakob disease in Japan.

    J Neurol Neurosurg Psychiatry. 2013; 84 (): 1171-1175https://doi.org/10.1136/jnnp-2012-304850

    • Van Iseghem V.
    • Sieben A.
    • Verhaeghe A.
    • De Vil B.
    • Meurs A.

    Dura mater graft-associated Creutzfeldt-Jakob disease with an incubation period of 30 years, mimicking non-convulsive status epilepticus.

    Acta Neurol Belg. 2019; 119 (): 497-499https://doi.org/10.1007/s13760-019-01145-z

  • Kuru: clinical study of a new syndrome resembling paralysis agitans in natives of the Eastern Highlands of Australian New Guinea.

    Med J Aust. 1957; 44 (): 745-754

  • Silvredale M, Leach JP, Chadwick DW. New variant Creutzfeldt-Jakob disease presenting as localization-related epilepsy. Neurology 2000;54(11):2188-. doi: 10.1212/Wnl.54.11.2188. PubMed PMID: WOS:000087526200037.

    • Trinka E.
    • Cock H.
    • Hesdorffer D.
    • Rossetti A.O.
    • Scheffer I.E.
    • Shinnar S.
    • et al.

    A definition and classification of status epilepticus – Report of the ILAE Task Force on Classification of Status Epilepticus.

    Epilepsia. 2015; 56 (): 1515-1523https://doi.org/10.1111/epi.13121

    • Fisher R.S.
    • Cross J.H.
    • French J.A.
    • Higurashi N.
    • Hirsch E.
    • Jansen F.E.
    • et al.

    Operational classification of seizure types by the International League Against Epilepsy: Position Paper of the ILAE Commission for Classification and Terminology.

    Epilepsia. 2017; 58 (): 522-530https://doi.org/10.1111/epi.13670

  • Aronyk K, Petito F, Solomon GE. Partial elementary motor seizures as the first symptom of Creutzfeldt-Jakob disease. Ann Neurol. 1984;15(2):210-1. Epub 1984/02/01. doi: 10.1002/ana.410150220. PubMed PMID: 6367622.

    • Yamanouchi H.
    • Budka H.
    • Vass K.

    Unilateral Creutzfeldt-Jakob disease.

    Neurology. 1986; 36 (): 1517-1520https://doi.org/10.1212/Wnl.36.11.1517

  • Cokgor I, Rozear M, Morgenlander JC. Seizures and Creutzfeldt-Jakob disease. A case report and series review. N C Med J. 1999;60(2):108-9. Epub 1999/05/27. PubMed PMID: 10344137.

  • Hsiao PS, Lee YM, Chu FS, Lee CL, Liu FC, Tsai PH. Probable sporadic Creutzfeldt-Jakob disease mimicking focal epilepsy. Epilepsy Behav Case Rep. 2019;11:77-80. Epub 2019/02/23. doi: 10.1016/j.ebcr.2019.01.006. PubMed PMID: 30788213; PubMed Central PMCID: PMCPMC6369248.

  • Lee K, Haight E, Olejniczak P. Epilepsia partialis continua in Creutzfeldt-Jakob disease. Acta Neurol Scand. 2000;102(6):398-402. Epub 2000/12/28. doi: 10.1034/j.1600-0404.2000.102006398.x. PubMed PMID: 11125757.

    • Parry J.
    • Tuch P.
    • Knezevic W.
    • Fabian V.

    Creutzfeldt-Jakob syndrome presenting as epilepsia partialis continua.

    J Clin Neurosci. 2001; 8 (): 266-268https://doi.org/10.1054/jocn.2000.0845

    • Donmez B.
    • Cakmur R.
    • Men S.
    • Oztura I.
    • Kitis A.

    Coexistence of movement disorders and epilepsia partialis continua as the initial signs in probable Creutzfeldt-Jakob disease.

    Movement Disord. 2005; 20 (): 1220-1223https://doi.org/10.1002/mds.20502

    • Lowden M.R.
    • Scott K.
    • Kothari M.J.

    Familial Creutzfeldt-Jakob disease presenting as epilepsia partialis continua.

    Epileptic Disord. 2008; 10 (): 271-275https://doi.org/10.1684/epd.2008.0216

    • Taskiran A.
    • Tezer F.I.
    • Saygi S.

    Epilepsia partialis continua as the presenting symptom in probable sporadic Creutzfeldt-Jakob disease.

    Epileptic Disord. 2011; 13 (): 82-87https://doi.org/10.1684/epd.2011.0400

  • Mader EC, Jr., El-Abassi R, Villemarette-Pittman NR, Santana-Gould L, Olejniczak PW, England JD. Sporadic Creutzfeldt-Jakob disease with focal findings: caveats to current diagnostic criteria. Neurol Int. 2013;5(1):e1. Epub 2013/05/30. doi: 10.4081/ni.2013.e1. PubMed PMID: 23717780; PubMed Central PMCID: PMCPMC3661986.

    • Yang S.H.
    • Yeh P.S.
    • Chen T.Y.

    Epilepsia partialis continua as the first presenting symptom in probable sporadic Creutzfeldt-Jacob disease: A case report and literature review.

    Case Rep Neurol. 2018; 10 (): 193-198https://doi.org/10.1159/000490909

    • Ogawa K.
    • Yoshihashi H.
    • Suzuki Y.
    • Oishi M.
    • Kamei S.

    Case of probable Creutzfeldt-Jakob disease presenting with complex partial seizure following sleeplessness and cognitive impairment.

    Geriatr Gerontol Int. 2011; 11 (): 229-232https://doi.org/10.1111/j.1447-0594.2010.00653.x

    • Rees J.H.
    • Smith S.J.
    • Kullmann D.M.
    • Hirsch N.P.
    • Howard R.S.

    Creutzfeldt-Jakob disease presenting as complex partial status epilepticus: a report of two cases.

    J Neurol Neurosurg Psychiatry. 1999; 66 (): 406-407https://doi.org/10.1136/jnnp.66.3.406

    • Cohen D.
    • Kutluay E.
    • Edwards J.
    • Peltier A.
    • Beydoun A.

    Sporadic Creutzfeldt-Jakob disease presenting with nonconvulsive status epilepticus.

    Epilepsy Behav. 2004; 5 (): 792-796https://doi.org/10.1016/j.yebeh.2004.06.019

    • Fernandez-Torre J.L.
    • Solar D.M.
    • Astudillo A.
    • Cereceda R.
    • Acebes A.
    • Calatayud M.T.

    Creutzfeldt-Jakob disease and non-convulsive status epilepticus: a clinical and electroencephalographic follow-up study.

    Clin Neurophysiol. 2004; 115 (): 316-319https://doi.org/10.1016/j.clinph.2003.10.012

  • Shapiro JM, Shujaat A, Wang J, Chen X. Creutzfeldt-Jakob disease presenting as refractory nonconvulsive status epilepticus. J Intensive Care Med. 2004;19(6):345-8. Epub 2004/11/04. doi: 10.1177/0885066604269771. PubMed PMID: 15523120.

  • Vaz J, Sierazdan K, Kane N. Non convulsive status epilepticus in Creutzfeldt-Jakob disease – A short report. J Neurol Neurosurg Psychiatry. 2005;76(9):1318-. PubMed PMID: WOS:000231538100071.

  • Creutzfeldt-Jakob disease: Evolution from nonconvulsive status epilepticus, through SIRPIDs, to generalized periodic discharges.

    Clin Neurophysiol. 2007; 118 (): 2533-2536https://doi.org/10.1016/j.clinph.2007.08.002

    • Aiguabella M.
    • Falip M.
    • Veciana M.
    • Bruna J.
    • Palasi A.
    • Corral L.
    • et al.

    Refractory nonconvulsive status epilepticus in Creutzfeldt-Jakob disease.

    Epileptic Disord. 2010; 12 (): 239-242https://doi.org/10.1684/epd.2010.0318

    • Espinosa P.S.
    • Bensalem-Owen M.K.
    • Fee D.B.

    Sporadic Creutzfeldt-Jakob disease presenting as nonconvulsive status epilepticus case report and review of the literature.

    Clin Neurol Neurosurg. 2010; 112 (): 537-540https://doi.org/10.1016/j.clineuro.2010.03.025

    • Lapergue B.
    • Demeret S.
    • Denys V.
    • Laplanche J.L.
    • Galanaud D.
    • Verny M.
    • et al.

    Sporadic Creutzfeldt-Jakob disease mimicking nonconvulsive status epilepticus.

    Neurology. 2010; 74 (): 1995-1999https://doi.org/10.1212/WNL.0b013e3181e39703

  • Coric L, Vargek-Solter V, Supanc V, Miskov S, Drnasin S, Sabolek K, et al. Sporadic Creutzfeldt-Jakob disease in a patient with episodes of nonconvulsive status epilepticus: case report. Acta Clin Croat. 2012;51(1):89-92. PubMed PMID: WOS:000307151300014.

  • van der Horn HJ, Egbers PH, Kuiper MA, Schuiling WJ. Diagnosing sporadic creutzfeldt-jakob disease in a patient with a suspected status epilepticus in the intensive care unit. Case Rep Neurol Med. 2013;2013:630141. Epub 2013/05/09. doi: 10.1155/2013/630141. PubMed PMID: 23653871; PubMed Central PMCID: PMCPMC3638556.

  • Rakitin A, Vibo R, Veikat V, Ounapuu A, Liigant A, Haldre S. Creutzfeldt-Jakob disease presenting as nonconvulsive status epilepticus. Case Rep Neurol Med. 2018. doi: Artn 3092018 10.1155/2018/3092018. PubMed PMID: WOS:000450785600001.

  • Mahboob HB, Kaokaf KH, Gonda JM. Creutzfeldt-Jakob disease presenting as expressive aphasia and nonconvulsive status epilepticus. Case Rep Crit Care. 2018;2018:5053175. Epub 2018/04/19. doi: 10.1155/2018/5053175. PubMed PMID: 29666711; PubMed Central PMCID: PMCPMC5832162.

    • Katsikaki G.
    • Dagklis I.E.
    • Angelopoulos P.
    • Ntantos D.
    • Prevezianou A.
    • Bostantjopoulou S.

    Atypical and early symptoms of sporadic Creutzfeldt – Jakob disease: case series and review of the literature.

    Int J Neurosci. 2020; ()https://doi.org/10.1080/00207454.2020.1759594

    • Neufeld M.Y.
    • Talianski-Aronov A.
    • Soffer D.
    • Korczyn A.D.

    Generalized convulsive status epilepticus in Creutzfeldt-Jakob disease.

    Seizure-Eur J Epilep. 2003; 12 (): 403-405https://doi.org/10.1016/S1059-1311(02)00378-3

    • Karatas H.
    • Dericioglu N.
    • Kursun O.
    • Saygi S.

    Creutzfeldt-Jakob disease presenting as hyperparathyroidism and generalized tonic status epilepticus.

    Clin Eeg Neurosci. 2007; 38 (): 203-206https://doi.org/10.1177/155005940703800404

  • Gomez Roldos A, Esteban de Antonio E, Perez-Chirinos Rodriguez M, Perez Sanchez JR. Refractory status epilepticus in genetic Creutzfeldt-Jakob disease with E200K mutation. Neurologia. 2020. Epub 2020/05/22. doi: 10.1016/j.nrl.2020.04.013. PubMed PMID: 32434752.

  • Krasnianski A, Kaune J, Jung K, Kretzschmar HA, Zerr I. First symptom and initial diagnosis in sporadic CJD patients in Germany. J Neurol. 2014;261(9):1811-7. Epub 2014/07/16. doi: 10.1007/s00415-014-7410-z. PubMed PMID: 25022936.

  • Appel SA, Chapman J, Prohovnik I, Hoffman C, Cohen OS, Blatt I. The EEG in E200K familial CJD: relation to MRI patterns. J Neurol. 2012;259(3):491-6. Epub 2011/08/13. doi: 10.1007/s00415-011-6208-5. PubMed PMID: 21833705.

  • Prodi E, Rossi S, Bertaina I, Pravata E, Sacco L. Report of a Case of Creutzfeldt-Jakob Disease With an Unusual Clinical Presentation. Front Behav Neurosci. 2020;14:55. Epub 2020/04/25. doi: 10.3389/fnbeh.2020.00055. PubMed PMID: 32327983; PubMed Central PMCID: PMCPMC7161217.

    • Steinhoff B.J.
    • Zerr I.
    • Glatting M.
    • Schulz-Schaeffer W.
    • Poser S.
    • Kretzschmar H.A.

    Diagnostic value of periodic complexes in Creutzfeldt-Jakob disease.

    Ann Neurol. 2004; 56 (): 702-708https://doi.org/10.1002/ana.20261

    • Au W.J.
    • Gabor A.J.
    • Vijayan N.
    • Markand O.N.

    Periodic lateralized epileptiform complexes (Pleds) in Creutzfeldt-Jakob Disease.

    Neurology. 1980; 30 (): 611-617https://doi.org/10.1212/Wnl.30.6.611

    • Kalamangalam G.P.
    • Slater J.D.

    Periodic lateralized epileptiform discharges and afterdischarges: common dynamic mechanisms.

    J Clin Neurophysiol. 2015; 32 (): 331-340https://doi.org/10.1097/Wnp.0000000000000173

  • Mead S, Rudge P. CJD mimics and chameleons. Pract Neurol. 2017;17(2):113-21. Epub 2017/02/06. doi: 10.1136/practneurol-2016-001571. PubMed PMID: 28153848; PubMed Central PMCID: PMCPMC5520355.

  • Ng MC, Westover MB, Cole AJ. Treating seizures in Creutzfeldt-Jakob disease. Epilepsy Behav Case Rep. 2014;2:75-9. Epub 2015/02/11. doi: 10.1016/j.ebcr.2014.01.004. PubMed PMID: 25667875; PubMed Central PMCID: PMCPMC4308028.

  • Nozaki I, Hamaguchi T, Sanjo N, Noguchi-Shinohara M, Sakai K, Nakamura Y, et al. Prospective 10-year surveillance of human prion diseases in Japan. Brain. 2010;133(10):3043-57. Epub 2010/09/22. doi: 10.1093/brain/awq216. PubMed PMID: 20855418.

  • Cobb WA, Hornabrook RW, Sanders S. The EEG of kuru. Electroencephalogr Clin Neurophysiol. 1973;34(4):419-27. Epub 1973/04/01. doi: 10.1016/0013-4694(73)90093-x. PubMed PMID: 4120316.

    • Will R.G.
    • Zeidler M.
    • Stewart G.E.
    • Macleod M.A.
    • Ironside J.W.
    • Cousens S.N.
    • et al.

    Diagnosis of new variant Creutzfeldt-Jakob disease.

    Ann Neurol. 2000; 47 (): 575-582

  • Tschampa HJ, Herms JW, Schulz-Schaeffer WJ, Maruschak B, Windl O, Jastrow U, et al. Clinical findings in sporadic Creutzfeldt-Jakob disease correlate with thalamic pathology. Brain. 2002;125(Pt 11):2558-66. Epub 2002/10/23. doi: 10.1093/brain/awf253. PubMed PMID: 12390980.

  • Mameniskiene R, Wolf P. Epilepsia partialis continua: A review. Seizure. 2017;44:74-Epub 2016/12/29. doi: 10.1016/j.seizure.2016.10.010. PubMed PMID: 28029552.

  • Albanese M, Placidi F, Romigi A, Schirinzi T, Liguori C, Marchi A, et al. Symptomatic nonconvulsive status epilepticus erroneously suggestive of sporadic Creutzfeldt-Jakob disease. J Neurol Sci. 2015;348(1-2):274-6. Epub 2014/12/01. doi: 10.1016/j.jns.2014.11.012. PubMed PMID: 25433451.

  • Labate A, Manna I, Gambardella A, Le Piane E, La Russa A, Condino F, et al. Association between the M129V variant allele of PRNP gene and mild temporal lobe epilepsy in women. Neurosci Lett. 2007;421(1):1-4. Epub 2006/11/10. doi: 10.1016/j.neulet.2006.10.020. PubMed PMID: 17092648.

  • Wang X, Sun W, Zhu X, Wu X, Li L, Zhu S, et al. M129V polymorphism in the prion protein gene is not associated with mesial temporal lobe epilepsy in a Han Chinese population. Eur J Neurol. 2008;15(8):827-30. Epub 2008/06/14. doi: 10.1111/j.1468-1331.2008.02191.x. PubMed PMID: 18549399.

  • Collinge J, Gorham M, Hudson F, Kennedy A, Keogh G, Pal S, et al. Safety and efficacy of quinacrine in human prion disease (PRION-1 study): a patient-preference trial. Lancet Neurol. 2009;8(4):334-44. Epub 2009/03/13. doi: 10.1016/S1474-4422(09)70049-3. PubMed PMID: 19278902; PubMed Central PMCID: PMCPMC2660392.

  • Vallabh SM, Minikel EV, Schreiber SL, Lander ES. Towards a treatment for genetic prion disease: trials and biomarkers. Lancet Neurol. 2020;19(4):361-8. Epub 2020/03/22. doi: 10.1016/S1474-4422(19)30403-X. PubMed PMID: 32199098.

  • Sen A, Capelli V, Husain M. Cognition and dementia in older patients with epilepsy. Brain. 2018;141(6):1592-608. Epub 2018/03/06. doi: 10.1093/brain/awy022. PubMed PMID: 29506031; PubMed Central PMCID: PMCPMC5972564.

    • Powell G.
    • Ziso B.
    • Larner A.J.

    The overlap between epilepsy and Alzheimer’s disease and the consequences for treatment.

    Expert Rev Neurother. 2019; 19 (): 653-661https://doi.org/10.1080/14737175.2019.1629289

  • Wulf MA, Senatore A, Aguzzi A. The biological function of the cellular prion protein: an update. Bmc Biol. 2017;15. doi: ARTN 34 10.1186/s12915-017-0375-5. PubMed PMID: WOS:000401489200001.

    • Collinge J.
    • Whittington M.A.
    • Sidle K.C.L.
    • Smith C.J.
    • Palmer M.S.
    • Clarke A.R.
    • et al.

    Prion protein is necessary for normal synaptic function.

    Nature. 1994; 370 (): 295-297https://doi.org/10.1038/370295a0

  • Walz R, Castro RMRPS, Velasco TR, Carlotti CG, Sakamoto EC, Brentani RR, et al. Cellular prion protein: Implications in seizures and epilepsy. Cell Mol Neurobiol. 2002;22(3):249-57. doi: Doi 10.1023/A:1020711700048. PubMed PMID: WOS:000178670100004.

  • Linden R. The Biological Function of the Prion Protein: A Cell surface scaffold of signaling modules. Front Mol Neurosci. 2017;10. doi: ARTN 77 10.3389/fnmol.2017.00077. PubMed PMID: WOS:000396743500001.

    • Colling S.B.
    • Khana M.
    • Collinge J.
    • Jefferys J.G.R.

    Mossy fibre reorganization in the hippocampus of prion protein null mice.

    Brain Res. 1997; 755 (): 28-35https://doi.org/10.1016/S0006-8993(97)00087-5

  • Chen S, Yadav SP, Surewicz WK. Interaction between human prion protein and amyloid-beta (Abeta) oligomers: role of N-terminal residues. J Biol Chem. 2010;285(34):26377-83. Epub 2010/06/26. doi: 10.1074/jbc.M110.145516. PubMed PMID: 20576610; PubMed Central PMCID: PMCPMC2924066.

  • Miyake K, Hara T, Oshima E, Kawada K, Ishizu H, Yamauchi Y, et al. Creutzfeldt-Jakob disease with Alzheimer pathology, presenting with status epilepticus following repeated partial seizures: a case report and literature review. BMC Neurol. 2018;18(1):54. Epub 2018/04/28. doi: 10.1186/s12883-018-1055-y. PubMed PMID: 29699515; PubMed Central PMCID: PMCPMC5921414.

  • Beagle AJ, Darwish SM, Ranasinghe KG, La AL, Karageorgiou E, Vossel KA. Relative Incidence of Seizures and Myoclonus in Alzheimer’s Disease, Dementia with Lewy Bodies, and Frontotemporal Dementia. J Alzheimers Dis. 2017;60(1):211-23. Epub 2017/08/23. doi: 10.3233/JAD-170031. PubMed PMID: 28826176; PubMed Central PMCID: PMCPMC5608587.

  • Peng C, Trojanowski JQ, Lee VM. Protein transmission in neurodegenerative disease. Nat Rev Neurol. 2020;16(4):199-212. Epub 2020/03/24. doi: 10.1038/s41582-020-0333-7. PubMed PMID: 32203399.

  • Stopschinski BE, Diamond MI. The prion model for progression and diversity of neurodegenerative diseases. Lancet Neurol. 2017;16(4):323-32. Epub 2017/02/28. doi: 10.1016/S1474-4422(17)30037-6. PubMed PMID: 28238712.

  • Ayers JI, Prusiner SB. Prion protein – mediator of toxicity in multiple proteinopathies. Nat Rev Neurol. 2020;16(4):187-8. Epub 2020/03/04. doi: 10.1038/s41582-020-0332-8. PubMed PMID: 32123368.

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