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Acil Serviste Zor Bir Tanı: Creutzfeldt-Jakob Hastalığı

Year 2015, Volume: 6 Issue: 3, 49 - 51, 01.07.2015

Sertaç Güler Halit Aytar Sinan Genç Hayri Ramadan

Abstract

Giriş: İnsan beyin dokusunu etkileyerek karakteristik süngerimsi
değişikliklere neden olan bir grup hastalık prion hastalıkları olarak
bilinir. Creutzfeldt-Jakob hastalığı (CJH), bu grup hastalıkların en
sık görülen tipidir. Özellikle acil servis (AS) şartlarında bu hastalığın
tanısını koymak çok zordur ve tanı, temel olarak devam eden
bilinç durum değişikliğinin diğer nedenlerinin ekartasyonuna
dayanır.
Olgu Sunumu: AS’de sporadik CJH ön tanısı ile değerlendirilen
ve konsülte edilen 77 yaşında kadın bir hastayı sunuyoruz.
Hasta AS’ye yataktan kalkamama ve idrar ve gayta inkontinansı
şikayetleri ile getirildi. Hastanın fizik ve nörolojik muayenesinde
öne çıkan bulgular arasında; kötü genel durum, sözel
yanıtsızlık, oromandibular distoni, ağrılı uyaran verilmesi ile
üst ekstremitelerde fleksör yanıt, ağrılı uyaranla göz açma ve
koreatetotik hareketler mevcuttu. Hastanın devam eden nörolojik
semptomları, beyin manyetik rezonans görüntüleme (MRG),
beyin omurilik sıvısı (BOS) analizi ve elektroensefalografi (EEG)
sonuçları birlikte değerlendirildiğinde, hastaya CJH tanısı kondu.
Sonuç: Her ne kadar AS hekimi CJH tanısını hastayı ilk
değerlendirmesinde koyamayacak olsa da, özellikle devam eden
mental durum değişikliği, miyoklonik hareketler ve demansı olan
hastalarda ek görüntüleme (beyin MRG, BOS analizi, EEG gibi)
seçeneklerini kullanma ve konsültasyon eşiğini düşük tutmalıdır.

Keywords

Creutzfeldt-Jakob hastalığı prion hastalıkları demans acil tıp (MeSH Database)

References

  • Puoti G, Bizzi A, Forloni G, Safar JG, Tagliavini F, Gambetti P. Sporadic human prion diseases: molecular insights and diagnosis. Lancet Neurol 2012; 11: 618-28. [CrossRef]
  • Ladogana A, Puopolo M, Croes EA, Budka H, Jarius C, Collins S, et al. Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada. Neurology 2005; 64: 1586-91. [CrossRef]
  • Masters CL, Harris JO, Gajdusek DC, Gibbs CJ Jr, Bernoulli C, Asher DM. CreutzfeldtJakob disease: patterns of worldwide occurrence and the significance of familial and sporadic clustering. Ann Neurol 1979; 5: 177-88. [CrossRef]
  • Haywood AM. Transmissible spongiform encephalopathies. N Engl J Med 1997; 337: 1821-8. [CrossRef]
  • Heinemann U, Krasnianski A, Meissner B, Varges D, Kallenberg K, SchulzSchaeffer WJ, et al. Creutzfeldt-Jakob disease in Germany: a prospective 12-year surveillance. Brain 2007; 130: 1350-9. [CrossRef]
  • Pocchiari M, Puopolo M, Croes EA, Budka H, Gelpi E, Collins S, et al. Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies. Brain 2004; 127: 2348-59. [CrossRef]
  • Clendenin J, Lall M. Creutzfeldt-Jacob Disease: An Emergency Department Presentation. American Journal of Emergency Medicine (2014). [CrossRef]
  • Wientjens DP, Davanipour Z, Hofman A, Kondo K, Matthews WB, Will RG. Risk factors for Creutzfeldt-Jakob disease: a reanalysis of case-control studies. Neurology 1996; 46: 1287-91. [CrossRef]
  • Collins S, Law MG, Fletcher A, Boyd A, Kaldor J, Masters CL. Surgical treatment and risk of sporadic Creutzfeldt-Jakob disease: a case-control study. Lancet 1999; 353: 693-7. [CrossRef]
  • CDC's Diagnostic Criteria for Creutzfeldt-Jakob Disease (CJD), 2010 http://www.cdc.gov/ncidod/dvrd/cjd/diagnostic_criteria.html (Accessed on October 27, 2014).

A Difficult Diagnosis in Emergency Department: Creutzfeldt–Jakob Disease

Year 2015, Volume: 6 Issue: 3, 49 - 51, 01.07.2015

Sertaç Güler Halit Aytar Sinan Genç Hayri Ramadan

Abstract

Introduction: Prion diseases are a group of diseases that affect the human brain tissue and cause characteristic spongiform changes. Creutzfeldt-Jakob Disease (CJD) is the most common type of this group of diseases. The diagnosis of CJD is very difficult particularly in emergency department (ED) settings and is mainly based on the exclusion of the other causes of ongoing mental status changes.Case Report: We present the case of a 77-year-old female as a sporadic CJD patient who was consulted with the preliminary diagnosis of CJD in ED. The patient was brought to ED with complaints of being unable to get out of bed and urinary and fecal incontinence. Remarkable physical findings included poor general condition, the lack of verbal response, oromandibular dystonia, eye opening and flexor responses of upper extremities to painful stimuli, and choreoathetosis. All findings including progressive neurological symptoms, brain magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) analysis, and electroencephalography (EEG) were considered together, and the patient was diagnosed with CJD.Conclusion: Even if an ED physician could not diagnose CJD in first hand, he/she should keep the level of additional imaging (MRI, CSF analysis, and EEG) and consultation threshold low, particularly in patients with ongoing mental status changes, jerking movements, and dementia

Keywords

Creutzfeldt-Jakob Disease prion diseases dementia emergency medicine

References

  • Puoti G, Bizzi A, Forloni G, Safar JG, Tagliavini F, Gambetti P. Sporadic human prion diseases: molecular insights and diagnosis. Lancet Neurol 2012; 11: 618-28. [CrossRef]
  • Ladogana A, Puopolo M, Croes EA, Budka H, Jarius C, Collins S, et al. Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada. Neurology 2005; 64: 1586-91. [CrossRef]
  • Masters CL, Harris JO, Gajdusek DC, Gibbs CJ Jr, Bernoulli C, Asher DM. CreutzfeldtJakob disease: patterns of worldwide occurrence and the significance of familial and sporadic clustering. Ann Neurol 1979; 5: 177-88. [CrossRef]
  • Haywood AM. Transmissible spongiform encephalopathies. N Engl J Med 1997; 337: 1821-8. [CrossRef]
  • Heinemann U, Krasnianski A, Meissner B, Varges D, Kallenberg K, SchulzSchaeffer WJ, et al. Creutzfeldt-Jakob disease in Germany: a prospective 12-year surveillance. Brain 2007; 130: 1350-9. [CrossRef]
  • Pocchiari M, Puopolo M, Croes EA, Budka H, Gelpi E, Collins S, et al. Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies. Brain 2004; 127: 2348-59. [CrossRef]
  • Clendenin J, Lall M. Creutzfeldt-Jacob Disease: An Emergency Department Presentation. American Journal of Emergency Medicine (2014). [CrossRef]
  • Wientjens DP, Davanipour Z, Hofman A, Kondo K, Matthews WB, Will RG. Risk factors for Creutzfeldt-Jakob disease: a reanalysis of case-control studies. Neurology 1996; 46: 1287-91. [CrossRef]
  • Collins S, Law MG, Fletcher A, Boyd A, Kaldor J, Masters CL. Surgical treatment and risk of sporadic Creutzfeldt-Jakob disease: a case-control study. Lancet 1999; 353: 693-7. [CrossRef]
  • CDC's Diagnostic Criteria for Creutzfeldt-Jakob Disease (CJD), 2010 http://www.cdc.gov/ncidod/dvrd/cjd/diagnostic_criteria.html (Accessed on October 27, 2014).
There are 10 citations in total.

Details

Other ID JA52EY46ZC
Journal Section Case Report
Authors

Sertaç Güler

Halit Aytar This is me

Sinan Genç This is me

Hayri Ramadan This is me

Publication Date July 1, 2015
Submission Date July 1, 2015
Published in Issue Year 2015 Volume: 6 Issue: 3

Cite

APA Güler, S., Aytar, H., Genç, S., Ramadan, H. (2015). A Difficult Diagnosis in Emergency Department: Creutzfeldt–Jakob Disease. Journal of Emergency Medicine Case Reports, 6(3), 49-51.
AMA Güler S, Aytar H, Genç S, Ramadan H. A Difficult Diagnosis in Emergency Department: Creutzfeldt–Jakob Disease. Journal of Emergency Medicine Case Reports. July 2015;6(3):49-51.
Chicago Güler, Sertaç, Halit Aytar, Sinan Genç, and Hayri Ramadan. “A Difficult Diagnosis in Emergency Department: Creutzfeldt–Jakob Disease”. Journal of Emergency Medicine Case Reports 6, no. 3 (July 2015): 49-51.
EndNote Güler S, Aytar H, Genç S, Ramadan H (July 1, 2015) A Difficult Diagnosis in Emergency Department: Creutzfeldt–Jakob Disease. Journal of Emergency Medicine Case Reports 6 3 49–51.
IEEE S. Güler, H. Aytar, S. Genç, and H. Ramadan, “A Difficult Diagnosis in Emergency Department: Creutzfeldt–Jakob Disease”, Journal of Emergency Medicine Case Reports, vol. 6, no. 3, pp. 49–51, 2015.
ISNAD Güler, Sertaç et al. “A Difficult Diagnosis in Emergency Department: Creutzfeldt–Jakob Disease”. Journal of Emergency Medicine Case Reports 6/3 (July 2015), 49-51.
JAMA Güler S, Aytar H, Genç S, Ramadan H. A Difficult Diagnosis in Emergency Department: Creutzfeldt–Jakob Disease. Journal of Emergency Medicine Case Reports. 2015;6:49–51.
MLA Güler, Sertaç et al. “A Difficult Diagnosis in Emergency Department: Creutzfeldt–Jakob Disease”. Journal of Emergency Medicine Case Reports, vol. 6, no. 3, 2015, pp. 49-51.
Vancouver Güler S, Aytar H, Genç S, Ramadan H. A Difficult Diagnosis in Emergency Department: Creutzfeldt–Jakob Disease. Journal of Emergency Medicine Case Reports. 2015;6(3):49-51.